A 66-year-old man was referred to our hospital for the treatment of an epithelial lesion with severe dysplasia, which was histologically confirmed at a local primary care clinic. Five years previously, he had undergone esophagogastroduodenoscopy (EGD) during a health checkup, and an esophageal subepithelial tumor (SET) was diagnosed during the examination. However, the patient did not return for follow-up of the lesion. The EGD showed a protruding mass that measured 1×1 cm in diameter and was located 25 cm from the upper incisors. The surface mucosa of the lesion was slightly irregular and did not stain with Lugol's solution. Endoscopic ultrasonography (EUS) demonstrated a hypoechoic and homogeneous lesion that originated from the muscularis mucosa (MM) below the epithelial layer. Endoscopic biopsy confirmed that the esophageal lesion was an SCC. EUS and chest computed tomography showed no evidence of lymph node metastasis in the mediastinum. The patient was diagnosed with SCC overlying a leiomyoma from the MM. En bloc resection using ESD was chosen as a curative treatment (). Histopathological examination of the resected lesion revealed an esophageal leiomyoma that underlay the SCC in situ and was 20×11 mm in size, and the leiomyoma consisted of bland spindle cells with cigar-shaped nuclei. The atypical squamous cells were confined to the mucosa, and both the lateral and vertical resection margins were tumor-free. Immunohistochemical staining showed Ki-67-positive atypical squamous cells in the entire layer. The leiomyoma demonstrated diffuse positive staining for smooth muscle actin (). The patient was followed up with annual EGD after the ESD. There was no local recurrence over the following 46 months.


A 74-year-old man was admitted for treatment of a histologically proven esophageal SCC. EGD revealed a 1 cm-sized elevated lesion with a smooth surface that was located in the proximal third of the esophagus. After the lesion was sprayed with Lugol's solution, ill-demarcated and unstained areas could be observed overlying the lesion. EUS demonstrated a hypoechoic tumor that was 5.6×2.9 mm in size and that originated from the MM. The patient was diagnosed with SCC overlying a leiomyoma from the MM. ESD was performed under general anesthesia. En bloc resection of the lesion was performed. There was no lymphovascular invasion or involvement of the deep resection margin, but there was involvement of the lateral resection margin in one block section. Histopathological examination of the resected lesion revealed high-grade dysplasia that overlay a leiomyoma (). The leiomyoma was positive for smooth muscle actin and desmin and its greatest dimension was 1.3 cm (). Follow-up endoscopy was selected rather than additional surgical resection because the apparent involvement of the lateral resection margin could have been a false positive due to a cautery effect. The patient underwent endoscopic examination after 8 months. Local recurrence was not observed; therefore, no biopsy was performed. The patient was also followed up with annual EGD and to date, the patient has remained disease-free for 28 months.


A 78-year-old man underwent EGD for screening because of anemia. EGD revealed an SET in the lower esophagus. Endoscopic biopsy showed high-grade dysplasia. EUS demonstrated a homogeneous and hypoechoic tumor that was 7.4×4.4 mm in size and located in the MM. It was concluded that the lesion had high-grade dysplasia and was a leiomyoma. Therefore, ESD was performed and the resected specimen was 30×23 mm in size (). Histopathological examination of the resected lesion revealed an esophageal leiomyoma that was 14×7×3 mm in size and covered with high-grade dysplasia. The resection margins were clear. After the treatment, the patient was followed up for 38 months with annual endoscopic examination. Local recurrence was not observed.


A 26-year-old Hindu male educated up to class 8th without any past and family history of psychiatric disorders, well-adjusted pre-morbidly, presented to the hospital with the history of being treated by cardiothoracic surgeon for multiple self-inserted nails in pericardium. History revealed that the patient had continuous illness of 4 years characterized by symptoms of muttering and smiling to self, being suspicious towards family members, decreased sleep, aimless wandering behavior, aggressive and assaultive behavior against family members, which was mostly unprovoked, poor self-care, and impaired biological functions. Mental status examination revealed blunted effect and prominent auditory hallucination in the form of people discussing among themselves about him and his actions. For the above symptoms, he was seen by a psychiatrist two years back, but the patient did not show much improvement as he remained poorly compliant to prescribed medications. In the mean time, the patient started having self-injurious behavior in the form of piercing nails and safety pins in his chest wall just adjacent to his left nipple. This behavior was not evident to his parents as he would do this mostly when he would be alone. His injury was noted by his uncle when he had fever and was taken to a doctor who noticed an infective swelling over left chest wall on the side of nipple. When inquired about any chest injury, the patient admitted about his self-mutilating behavior of piercing his chest wall with nails and safety pins. Radiological examination of the chest showed multiple foreign body in the chest wall and pericardial cavity. He was referred to a cardiothoracic surgeon for treatment. After being treated for foreign body in chest and pericardial cavity, patient's guardian came to the hospital for treatment of psychiatric symptoms. On asking the reason for the self-mutilating behavior, patient told that he did it to relieve his feeling of burning sensation in left side of chest and palpitation. The patient did not attribute his self-injury behavior as a response to auditory hallucination. He also said that he did not have much perception of pain during this behavior. Physical examination revealed multiple scar mark over left chest wall and postoperative scar marks over sternum []. Patient was prescribed with tablet trifluperazine 10 mg and trihexyphenidyl 2 mg, and was asked to follow up after 1 month. After 1 month of treatment, patient showed good improvement in psychopathology, and there were no reports of self-injurious behavior during this period.


A 19-year-old girl presented with a history of recurrent hemoptysis for 3 years. The hemoptysis was insidious in onset with significant blood loss necessitating multiple blood transfusions on different occasions. There was no history to suggest tuberculosis (TB). Clinical examination of the respiratory and cardiovascular systems did not reveal any abnormality.
A contrast-enhanced computed tomography (Philips Brilliance CT 6- slice scanner, CECT) revealed normally aerated lungs with normal bronchial anatomy and absent basal segmental pulmonary arterial branches to the left lower lobe. There was a large systemic artery arising from the left lateral aspect of the descending thoracic aorta coursing through the pulmonary ligament and supplying the basal segments of the left lower lobe []. Left inferior pulmonary vein drained normally into the left atrium. These findings were consistent with ISSNL, also known as type B isolated arterial pulmonary malinosculation. Since the lung was otherwise normal, endovascular treatment was planned instead of surgical resection.
A digital subtraction angiogram (Siemens ARTIS ZEE Biplane, Ehrlang, Germany and Siemens Multistar DSA machine) was performed using a percutaneous approach through the right common femoral artery, which confirmed the findings of the CT scan []. A 100 cm, 8F long vascular sheath was placed with its tip in the descending thoracic aorta. The aberrant artery was selectively cannulated and a 16 mm × 12 mm Amplatzer Vascular Plug II (9-AVP2-016; St. Jude Medical, Plymouth, Minnesota, USA) was deployed within the distal portion of the aberrant artery. Since there was persistent blood flow in the artery even after 25 min, two embolization coils (NESTER 0.035″ pushable coils; COOK, Bloomington, IN47404, USA) measuring 15 mm × 15 cm and 10 mm × 15 cm were deployed proximal to the Amplatzer plug, followed by injection of a small amount of 80% n-butyl cyanoacrylate (NBCA) (Histacryl; B Braun, Tuttlingen, Germany), after which there was complete occlusion of the anomalous artery []. Post-procedural period was uneventful. The patient was discharged on a course of antibiotics and thrice-daily steroids for 10 days. A follow-up telephonic interview 5 months later revealed the patient to be symptom-free.


A 32-year-old lady presented with history of intermittent hemoptysis for the past 20 years. She did not require hospital admissions or blood transfusions. She did not have any clinical or laboratory investigation evidence to suggest diabetes mellitus, hypertension, or TB. Her bronchial lavage and transbronchial lung biopsy as part of the TB workup were normal. CECT thorax revealed features consistent with [] ISSNL or type B isolated arterial pulmonary malinosculation.
A digital subtraction angiogram confirmed the findings []. A 60 cm, 7F vascular sheath was placed with its tip in the descending thoracic aorta. The aberrant artery was selectively cannulated with a glide SIM-1 catheter and tip of cathter was placed in the distal portion of the aberrant vessel. Two 12-mm detachable balloons (Goldbalt-5) were deployed []. Post-deployment angiogram revealed a small stump with opacification of a segmental branch of the basal lung. There was distal occlusion of the artery. The post-procedural period was uneventful and the patient was discharged on a course of antibiotics. She was symptom-free for 6 months but later presented with recurrent hemoptysis. CECT revealed compete recanalization of the aberrant artery []. A repeat endovascular management with Amplatzer plug II was planned but the patient did not return for follow-up.


A 27-year-old man presented with recurrent episodes of hemoptysis for 2 years. CECT revealed features suggestive of [] ISSNL or type B isolated arterial pulmonary malinosculation.
A digital subtraction angiogram was performed. A 7F-long sheath (Arrow, Bernville road, PA, USA) was placed into the aberrant artery [], through which a vertebral glide catheter was passed and placed in the distal aspect. Three embolization coils (NESTER 0.035″ pushable coils; COOK, Bloomington, IN47404, USA) measuring 10 mm × 14 cm were deployed proximally [] and a check angiogram was performed which showed complete occlusion of the aberrant artery. Post-procedural period was uneventful. The patient was discharged and antibiotics were prescribed for a week. A follow-up telephonic interview conducted 1 year later revealed that the patient was symptom-free for 10 months after which he had one episode of minimal hemoptysis not requiring intervention. Repeat CT has not yet been performed.


A 15-year-old boy presented with recurrent hemoptysis for 3 months. He did not require any hospital admission or blood transfusion. Clinical examination of his respiratory and cardiovascular systems was normal. CECT revealed [] ISSNL or type B isolated arterial pulmonary malinosculation.
A digital subtraction angiogram was performed []. A 6F Ansel sheath was placed into the aberrant artery from the descending thoracic aorta and a 12 mm × 9 mm Amplatzer Vascular Plug II (9-AVP2-016) was deployed into the distal portion of the aberrant artery. A check angiogram, 25 minutes later, showed complete occlusion of the aberrant artery []. Post-procedural period was uneventful. The patient was discharged and antibiotics were prescribed for a week. A follow-up CECT showed areas of collapse in the anterior and lateral basal segments of the left lower lobe with non-opacification of the aberrant artery. Patient was symptom-free at 6 months follow-up. summarizes the management of our patients and the various types of pulmonary malinosculations with proposed standard strategies.


A 55-year-old female patient was transferred from another hospital because of chest pain radiating to her back and thrombocytopenia. She had been diagnosed as having essential hypertension approximately 3 years earlier, but was not medicated. The chest pain developed upon exertion and radiated to her back the day prior to admission to a local hospital; it persisted and became more severe after admission. On admission, her temperature was 36.8°C, her pulse rate was 106, her respiration was 20, and her blood pressure was 190/100 mmHg. On physical examination, the patient appeared acutely ill, but her heart sounds were normal. A neurologic examination revealed no abnormalities. Laboratory test results are shown in . Urinalysis revealed many red blood cells and 0–5 white blood cells per high power field. Chest radiographs revealed mediastinal widening, and a computer tomography (CT) scan of the thorax and abdomen revealed a dissecting aneurysm of the descending thoracic aorta, which descended inferiorly to the proximal abdominal aorta (). Medical treatment started immediately with the infusion of nitroprusside and esmolol. On the 2nd day of admission, the patient reported an improvement in her chest pain, and she had a platelet count of 63,000/mm3. On the 7th day, her platelet count was 377,000/mm3, and her prothrombin and partial-thromboplastin times were normal. We followed up the aortic dissecting aneurysm by a thorax CT on the same day and disease progression was not observed.
At 3 weeks after admission, her blood pressure and pulse rate had normalized and her chest pain had fully subsided. The DIC profiles, including platelet count, prothrombin and partial-thromboplastin times, D-dimer, FDP, antithrombin III, and fibrinogen, had normalized (). Outpatient follow-up by laboratory testing, thorax CT, and echocardiography have been performed at regular intervals over a period of 9 months, and the patient is doing well.


A 70-year-old woman was admitted with severe abdominal pain, which began a few hours before admission. She had neither a specific past medical history nor any underlying illness. The abdominal pain was continuous and diffuse without localization. The initial blood pressure was 120/80 mmHg, the pulse rate was 84 beats per minute, the temperature was 36.4°C, and the respiratory rate was 32 breaths per minute. On physical examination, the abdomen was soft but moderately tender throughout without rebound tenderness. The laboratory values were as follows: white blood cell count, 11,900/mm3; AST, 26 IU/L; ALT, 11 IU/L; amylase, 210 IU/L; lipase, 316 IU/L. An ultrasonographic examination showed that the gallbladder had no abnormal findings but the small intestine showed diffuse dilatation and wall thickening (, ). A computed tomographic examination of the abdomen and pelvis performed with the intravenous administration of contrast media showed severe thickening of the small intestinal wall and small quantity of ascites without any evidence of bowel perforation or acute appendicitis (). During the next three hours, the patient’s blood pressure fell to 80/50 mmHg and the temperature rose to 38°C. Her abdominal pain worsened with newly developed rebound tenderness and rigidity on abdominal palpation. After deliberating on the patient’s age, clinical manifestation and radiological findings, abdominal angiography was performed to rule out the possibility of acute intestinal ischemia. Abdominal angiography showed multiple stenoses at the superior and inferior mesenteric artery and extensive spasms at the branches of the superior mesenteric artery (). An exploratory laparotomy was performed on with the impression of small bowel necrosis due to acute intestinal ischemia. On exploration, there were neither intestinal necrosis nor intestinal perforation. Only bile-colored ascites was noted. Although the gallbladder appeared normal, bile leaked on a localized focus of the gallbladder with gentle squeezing. Therefore, the exploration was stopped after performing the cholecystectomy. The excised gallbladder appeared grossly normal except for the focal necrotic area without a frank perforation or stone (). On a pathologic examination, the gallbladder showed a focal area of inflammation and necrosis confined to the microscopically perforated site at the body (). The results of the bile culture were negative. After surgery she recovered and was discharged 16 days later.


A 54-year-old man presented with a several-day history of erythematous rashes on his extremities. He also had a history of ingesting raw freshwater fish, but not of bronchial asthma or other medical side effects. He had visited a community clinic where a chest radiograph showed a solitary pulmonary nodule in the right upper lobe (). This nodule was not present on a chest radiograph taken 4 years before. The rashes subsided following treatment with an antihistamine and a non-steroidal anti-infilammatory agent. The patient’s physical examination was unremarkable. On admission, his leukocyte count was 11,250 cells/μL with 38% neutrophils, 23% lymphocytes, 4% monocytes, and 35% eosinophils. Other hematologic parameters were 13.5 g/dL hemoglobin, 7 mm/hr ESR, 12.5 sec (control=11.5 sec) PT, and 28 sec (INR=1.0) PTT. Urinalysis, blood chemistry, pulmonary function test, and electrocardiography were normal. Anti-Hepatitis B surface antigen was negative, but anti-Hepatitis B surface antibody was positive. All stains and cultures of sputum for bacteria and mycobacteria were negative. There were no malignant cells in sputum cytology, but many eosinophils were present. Stool examination for helminths revealed many Clonorchis sinensis ova; stool ova count was 200 eggs per gram. A skin test for Paragonimus westermani was negative, but one for Clonorchis sinensis was strongly positive. Total serum IgE was 1020 IU/mL and Clonorchis-specific IgG antibody by micro-ELISA was 0.27 (<0.25).
Serial chest radiography (, ) and high resolution computed tomography (, , ) showed that the pulmonary nodules and patchy densities migrated to other sites and changed in shape and size daily. On the second day of admission, the patient underwent fiberoptic bronchoscopy that revealed no endobronchial lesion. The bronchoalveolar lavage (BAL) fluid had cell proportions of 45% macrophage 15% lymphocytes, 15% neutrophils, and 25% eosinophils (). The BAL fluid was negative for bacterial, acid-fast bacilli, and fungi. A transbronchial lung biopsy was performed at the right middle lobe. Microscopy of the lung tissue showed eosinophilic infiltration (). Abdominal ultrasound revealed no specific abnormalities except mild splenomegaly. On the ninth day of admission, migrating nodular densities on chest radiographs decreased and then disappeared dramatically following oral praziquantel therapy (). Blood eosinophilia consequently decreased and normalized. There was no evidence of recurrence after one year of follow-up.


A 64-year-old woman was presented to us with a two-year history of poly arthritis, most marked in the knees and both hand proximal interphalangial joints (PIP) and associated with left ankle pain, in October 1998. Past medical history revealed a history of hypertension and intracerebral hemorrhage. Rheumatoid arthritis was diagnosed according to 1987 ACR revised criteria, based on a symmetric polyarthritis involving the PIP, shoulders, left ankle and knees, morning stiffness and the presence of rheumatoid factor.
She was treated with hydroxychloroquine and methotrexate, but an exacerbation occurred involving the right knee in January 1999. Also, she had numbness of the right sole. At radiologic evaluation of the knees, narrowing of the medial joint space and joint effusion were noted, but no erosion was seen. Treatment with local steroid injection was resumed with increments of methotrexate doses.
Within a few weeks of local steroid injection in the knee joint, however, right knee pain worsened and numbness of the sole continued. She gave no history of prior injury or other paraesthesia.
On physical examination, she revealed pain and increased sensation along the plantar aspect of the right foot and the mass was palpable on the posterior aspect of the right knee. Also, she showed weak flexion of the big toe and decreased right ankle jerk.
A lobulated 4×4×3 cm3 cystic lesion with inhomogeneous signal intensity was seen on ultrasound. The needle aspiration of the right knee joint was unproductive, and magnetic resonance imaging of the right knee and popliteal fossa was obtained and revealed a Baker’s cyst(). The cyst extended towards the popliteal tendon and lateral collateral ligament and there was no evidence of deep vein thrombosis.
On electromyography, 3+ and 2+fibrillation and positive sharp waves potentials were observed in the abductor hallucis and tibialis posterior showing abnormal spontaneous response. During contraction, polyphasic motor unit was observed in the abductor hallucis and tibialis posterior.
On nerve conduction studies, proximal latency and amplitude in the right tibial nerve was 15.4ms and 0.1mV, showing low amplitude and delayed proximal latency of right tibial compound muscle action potential.
Repeated needle aspiration was unproductive, and open surgical procedure was performed. At operation, a lobulated cystic lesion displacing neurovascular structures was released, but neurovascular invasion by a cyst was not found(). The cavity contained many folds and measured 4×4×3 cm3, and extended anteriorly toward the posterior cortex of the tibia. The communication between the synovial compartment and the cavity was present near the popliteal hiatus. The biopsy finding was compatible with Baker’s cyst due to rheumatoid arthritis(). Her pain resolved following surgical removal of Baker’s cyst, but mild degree of numbness remained.


A 39-year-old woman was admitted to the hospital because of left groin pain. She had a past medical history of meningitis. Four years previously, she was found to have MCTD on the clinical diagnostic criteria of Sharp et al. At that time, she experienced arthritis, Raynaud’s phenomenon, swollen hands and biopsy-proven myositis. Laboratory examinations revealed the following results: leukocyte count 3600/mm3, antinuclear antibody 1:640, speckled pattern, positive anti-nRNP & Ro antibody, LDH 208 U, CK 23 U. The patient was treated with hydroxychlorquine, prednisolone (10 to 20 mg/day, sometimes on 5 mg/day), piroxicam and angiotensin-converting enzyme inhibitor or calcium channel blocker. Thereafter, she was intermittently admitted to the hospital due to drug-induced hepatitis, severe dysplasia of the uterine cervix, fever with chills and painful lymphadenopathy. At the presentation, she visited the hospital due to back and left groin pain during walking down stairs about 15 days ago. She denied a significant trauma history. Pelvis X-ray previously checked was unremarkable except for an osteoporotic change (). Physical examination on admission showed positive a Patric test on the left hip joint. We suspected her to have the avascular necrosis of the left femoral head. However, MRI of the left femoral head did not confirm our suspicions but instead, revealed a synovitis with effusion. Simple pelvis radiograph was done. A plain radiograph of the pelvis revealed insufficiency fractures of parasymphyseal and left pubic rami (). We recommended bed rest, tapering of prednisolone and management of steroid-induced osteoporosis. After 5 months, a follow-up pelvis X-ray revealed the healing of parasymphyseal fracture ().


A 25 year-old female presented herself to a local clinic because of exposure to phogene. She had been in excellent health until September 8, 1995, when she was exposed to phosgene. She complained of coughing, chest tightness and dyspnea which were not relieved despite therapy with oxygen. She was transferred to CNUH about six hours after exposure.
Admitting vital signs were blood pressure 100/60 mmHg. pulse 110/min, respiration 20/min, temperature 36.4°C. Examination of the chest revealed bilateral inspiratory and expiratory crackles. The chest X-ray showed diffuse increased haziness on both lung field (). The WBC were 12.7 × 103/μl, the LDH 409 unit, initial arterial blood gases determinations on room air were, PaO2, 68.9 mmHg, PaCO2, 34.7 mmHg, pH, 7.392.
The patient was treated with a face mask with 8 L/min oxygen. On the second day of admission, she was transferred to the ICU with respiratory insufficiency. She had progressively increasing respiratory symptoms with the respiratory rate rising to 44/min, pulse rate to 114/min and blood pressure decreasing 70/40 mmHg. The WBC were 25.2 × 103/μl with 95.6% neutrophils and LDH was 665 unit. The arterial blood gases were PaO2, 41.2 mmHg, PaCO2 34.9 mmHg, pH, 7.364. The patient was intubated and ventilated with controlled mode and 8.0 cmH2O of positive-end expiratory pressure (PEEP) was applied. Two hours after ventilation, the patient became cyanotic and oxygen saturation decreased to 60% and a chest X-ray showed pneumothorax on the right thoracic cavity. Chest tube was inserted with a water seal bottle. Five hours after controlled ventilation, arterial blood gases on FiO2 0.6 were PaO2, 60.4 mmHg, PaCO2, 31.3 mmHg, pH, 7.520. Ventilation was controlled with diazepam and vecuronium to reduce anxiety and fighting.
On the third day of ventilation, she was weaned from the ventilator and extubated. The patient received methylprednisolone (500 mg) for 7 days, 2 days of furosemide (120 mg), aminophylline, dopamine and antibiotics from the day of admission.
On the fifteenth day following exposure, pulmonary function tests showed a restrictive pattern and the diffusion capacity was within the normal limit (). A follow-up chest X-ray was cleared to normal ().


This 37 year-old male was admitted to CNUH about ten hours after phosgene exposure. Physical findings on admission were blood pressure 110/70 mmHg, pulse 154/min, respiration 30/min, temperature 36.4°C. Examination of the chest X-ray revealed bilateral diffuse pulmonary edema (). Initial blood gas determinations on room air were, PaO2, 61.8 mmHg, PaCO2, 37.6 mmHg, pH, 7.356.
Ten minutes later, he developed tachycardia and tachypnea despite therapy with oxygen and aminophylline. The arterial blood gases on 10 L/min oxygen were PaO2, 51.9 mmHg, PaCO2, 35.9 mmHg, pH, 7.317. The patient was immediately intubated and ventilated with controlled mode. Arterial blood gases were PaO2, 45.7 mmHg, PaCO2, 40.4 mmHg, pH, 7.301. The blood pressure was 80/50 mmHg. The patient was given fresh frozen plasma, as well as crystalloid solutions and dopamine to stabilize the circulation. After fluid administration, the arterial blood gases were PaO2, 35.2 mmHg, PaCO2, 61.5 mmHg, pH, 7.144. Metabolic acidosis was treated with NaHCO3, as required. However the patient had a progressive metabolic acidosis despite controlled ventilation and fluid administration. The patient died of uncontrolled hypoxemia.


The patient was a 30 year-old male who was exposed to phosgene twelve hours prior to admission to a local clinic complaining of dyspnea, coughing and sputum. Auscultation of the chest revealed coarse breath sound with crackles all over the lung fields. A chest X-ray showed bilateral diffuse interstitial infiltration. The WBC were 12.3 × 103/μl. Arterial blood gases were PaO2, 50.2 mmHg, PaCO2, 37.2 mmHg, pH, 7.401. The patient was transferred to CNUH with worsening dyspnea. The vital signs on admission were blood pressure 130/70 mmHg, pulse 89/min, respiration 28/min and temperature 36.5°C. The WBC were 18.0 × 103/μl with 98% neutrophils. The LDH was 350 unit. Initial blood gas determinations on room air were, PaO2, 55.3 mmHg, PaCO2, 26.7 mmHg. pH, 7.477. Twelve hours after admission, the patient began to have progressively increasing respiratory symptoms with the respiratory rate rising to 55/min. The patient was intubated and ventilated with controlled mode. Ventilation was maintained with diazepam and vecuronium and treated with methylprednisolone and aminophylline. Three hours after ventilation, the fraction of inspired oxygen could be reduced to 0.6. After 24 hours of ventilation with 6 cmH2O PEEP, he developed subcutaneous emphysema extending into the neck and anterior chest. The PEEP could be reduced and subcutaneous emphysema and arterial oxygenation did not get-worse. On the third day, the patient could be weaned from the ventilator and extubated. The patient was treated with methylprednisolone, aminophylline and antibiotics. Pulmonary function tests were performed on the fifth day following exposure and showed a restrictive pattern and a normal diffusion capacity ().
The three other patients exposed to phosgene were transferred to CNUH and all were in their early forties. Their complaints were also shortness of breath, chest tightness without derangements of vital signs and arterial blood gases. The pertinent findings in the physical examination were bilateral crackles over the lung fields and diffuse bilateral interstitial infiltrates on chest X-ray.
They were treated with supplemental oxygen by external devices, methylprednisolone, aminophylline and antibiotics. Their symptoms were gradually improved in 3 to 5 days of supportive care.


A 61-year-old man, with a history of NIDDM ten years previously, was admitted due to epigastric pain, indigestion, and malaise. On admission, he had a regular pulse rate, was normotensive and relatively well-looking. There was no icterus, spider angioma, palmar erythema or cardiac murmur. Liver and spleen were not palpable.
Laboratory examinations showed serum trans aminase(SGOT:89 IU/L. SGPT:33 IU/L, and GGT 105 IU/L). Blood count was normal. Abdominal ultrasonogram favored a hepatoma of the right lobe with a right hepatic vein thrombus(). Liver biopsy showed a poorly differentiated hepatocellular carcinoma(). The electrocardiogram was normal. Two-dimensional echocardiographic examination and transesophageal echocardiogram were performed, showing a large right atrial mass(). The IVC had a tumor, which appeared to be continuous with the right atrial mass.
Hence, chemotherapy with 5-fluorouracil, adriamycin and mitomycin was started. The serial echocardiogram showed no significant change in the right atrial mass.
The patient expired 6 months later due to hepatic failure.


The patient was a 34-year-old woman. One year prior to admission, she had began suffer-ing from an abdominal fullness and weakness. Physical findings revealed a hard non-tender mass in the right upper quadrant.
Laboratory examinations manifested serum ALT of 391IU/L, serum AST of 341IU/L, alkaline phosphatase of 279IU/L, gamma glutamyl transferase of 298IU/L, alpha-fetoprotein of more than 7000ng/ml and alpha 1-antitypsin of 410mg/dl. Blood count was normal.
Abdominal computed tomography revealed an ill-definded large inhomogeneous mass filling the entire right lobe of the liver with underlying liver cirrhosis and tumor thrombus in the upper portion of the IVC and the right atrium (). The electrocardiogram was normal. Two-dimensional echocardiogram manifested thrombus in the right atrium(). She expired 4 months later.


A 35-year-old man was admitted to the hospital because of hematemesis one month prior to admission. He had been treated with NPH insulin, 30 units daily, due to known diabetes mellitus for the past 2 years.
On admission, hemoglobin was 11.1 g/dl and alkaline phosphatase was 100 IU/l, but other liver function tests were within normal ranges. Serum HBs Ag was negative and alpha-fetoprotein was below 5 ng/ml. Bleeding time, prothrombin time, activated partial thromboplastin time and platlet counts were all normal.
Upper gastrointestinal fiberscopy revealed slightly bluish, beaded esophageal varices, located in the lower esophagus, with no sign of redness (). No evidence of recent bleeding was found. Sclerotherapy was started with 99.9% ethanol intravariceally. After discharge, 2 more sessions of sclerotherapy were done with intravariceal injections of 5% ethanolamine oleate in one week intervals. Two days after the third session, severe swallowing difficulty and retrosternal pain developed. On endoscopy, a huge submucosal bulge in the lumen of the esophagus 25 cm distal to the upper incisors was found with dark bluish mucosal discoloration (). The esophageal lumen was nearly occluded. Barium esophagogram revealed a large smooth filling defect in the lower two thirds of the esophagus with near total occlusion (). Because of the submucosal nature of the mass, an intramural hematoma was presumed as the cause. Supportive care with parenteral nutrition was given and liquids could be swallowed with mild discomfort on the seventh day after sclerotherapy. Two weeks after the third sclerotherapy, repeat endoscopy revealed that the hematoma had completely disappeared and was replaced with a longitudinal mucosal defect ().


A 50-year-old female patient presented with an indolent cutaneous nodule growing at the frontoparietal region of anterior scalp since last 6 years. It was asymptomatic to begin with, but started producing intense itch since last 4 years, later associated with intermittent localized pain and scanty purulent discharge from an erosion spontaneously formed on the nodule since last 1 year. There was no history of any local trauma or bleeding from the growth. The personal and family medical history were insignificant especially pertaining to any malignancies, immunosuppression or related symptoms. A thorough systemic and general physical examination did not reveal any significant clinical abnormality or any local or distant lymphadenopathy. The local examination displayed a dull erythematous, woody firm, non-tender, non-fluctuant, multinodular, cauliflower-shaped growth of size 7 × 7 cm which was fixed to the deeper subcutaneous tissues at mid frontoparietal region of scalp. The overlying skin was tense, smooth and eroded at the center with superficial crust deposition. Overall, it appeared like a small turban tumor clinically []. X-ray skull and CT scan showed a lobular supratentorial lesion within the scalp in the midline showing nodular foci of calcification and necrotic component. There was outer table bony erosion but no intracranial extension. The clinical differentials included cylindroma (the archetypal turban tumor), dermatofibrosarcoma protuberans, cutaneous metastases (from occult primary) and deep fungal infection. An initial incisional biopsy suggested a diagnosis of an epithelial neoplasm, made up of numerous rounded tumor islands composed of small and dark poroid cells and large pink cuticular cells. Both types of cells were somewhat monomorphous cells with rounded nuclei although occasional mitotic figures were also present. A few ducts and occasional necrotic areas could also be seen within the tumor islands. The stroma showed abundant fibroplasia, clefts and dilated capillaries. Overall features were consistent with a benign tumor, but with atypical features. The morphology of cells and necrosis pointed towards poroma, however, the architecture and dermal localization of the neoplasm were features in favor of hidradenoma [Figure and ]. The closest acceptable fit was a mix/overlap diagnosis of atypical poroid hidradenoma. We planned a wide local excision, which was performed under local anesthesia. The outer table of skull had to be chiseled out [] and the resultant defect had to be covered with flap advancement and a split-thickness skin graft, which healed slowly in about 6 weeks. A biopsy of the excised tumor exhibited a fairly well circumscribed epithelial neoplasm with no connection with overlying epidermis. It consisted of interconnected nodules as well as differentiated ducts, the neoplastic cells showing pleomorphism of nuclei with occasional normal and abnormal mitotic figures and abundant pale cytoplasm. Foci of en masse necrosis and clefts separating the tumor cells from the surrounding sclerotic stroma were also present. These features were consistent with a well circumscribed and slowly growing hidradenocarcinoma [Figure -]. It is probable that malignancy originated within the mass of benign hidradenoma that had been present since many years. Surgical margins and base were free of tumor. The patient was following in the outpatient clinic with apparent complete healing of the surgical wound after 3 months. However, at the last visit (6 months post-operative) the patient presented with a local recurrence in form of large multinodular tumor at the same site.


In November 2007, a 14 year old female patient presented to our dental office. She had an open bite and had experienced a traumatic dental injury at nine years of age. Tooth 21 was avulsed and had been replanted and tooth 11 was intruded. Both teeth had ankylosed, were infraoccluded, and demonstrated a stone percussion sound. Her complaints were the aesthetic appearance of these teeth and the fact that she had used an orthodontic appliance for a long time (since nine years of age). Clinical and radiographic findings presented a face with a convex profile, open bite, and the presence of an orthodontic appliance (). On radiographic evaluation, tooth 11 showed pulpal canal obliteration and tooth 21 had received root canal treatment. Inflammatory root resorption and replacement root resorption were present in both teeth, and tooth 12 demonstrated apical root resorption ().
After evaluation, orthodontic treatment was recommended, with extraction of the four first premolars and autotransplantation of the mandibular premolars to the site of the upper central incisors. The treatment plan consisted of removing the orthodontic appliance, study for orthodontic treatment, extraction of teeth 14 and 24, extraction of teeth 11 and 21 and preparation of the sockets, extraction of teeth 34 and 44 and transplantation to the sockets of teeth 11 and 21, reshaping the transplanted teeth, placement of a full fixed appliance, removal of the orthodontic appliance, and the placement of an upper Hawley retainer and lower flexible lingual bonded retainer on the six anterior teeth. The patient's parents accepted the proposed treatment plan.
Articaine hydrochloride anhydrous was used as a local anesthetic, with 1:200,000 adrenaline tartrate (Meganest 1:200,000, Clarben, S.A., Madrid, Spain). Teeth 11 and 21 were extracted after an intrasulcular incision to allow for better gingival fiber reinsertion. The recipient sites were evaluated clinically and on the radiographs (). The donor teeth were measured with periapical radiography and acrylic model teeth were made with the same shape and dimensions of the donor teeth (). These models were used in the preparation of the alveolar recipient sites.
The recipient bed was prepared for immediate transplantation to the site of tooth 11, whereas transplantation was deferred by ten days for tooth 21 (). The teeth chosen for transplantation were cleaned with a polishing brush and toothpaste, and disinfection of the oral cavity was carried out with a solution of 0.1% chlorhexidine digluconate and 0.1% clorbutanol hemihydrate (Eludril, Pierre Fabre Dermo-Cosmetique, Soual, France). The teeth chosen for transplantation were atraumatically extracted and gently inserted into the prepared alveolar socket. The extraalveolar time of the transplanted teeth was minimal (less than five minutes), in order to maintain the vitality of periodontal ligament cells.
After transplantation, the teeth were splinted during a period of ten days using 3-0 silk sutures crossing the occlusal face of the tooth, and then were fixed with composite (). The occlusion was checked and controlled with blue/red 60 µm interarticular paper (D-50769, Bausch KG, Koln, Germany). An antibiotic was administered, starting 24 hours before the intervention (one gram amoxicillin tablets, one tablet every 12 hours), and continuing for six days after the procedure.
Instructions were given for analgesic treatment (paracetamol, 500 mg tablets as necessary, no more than 4 tablets in 24 hours). An ice pack was applied to the face, protected with fabric or paper. A soft diet was advised for 48 hours after the transplantation, and chewing with the transplanted teeth was prohibited for a period of seven days. Careful oral hygiene was also recommended, followed by a mouthwash with 1 mg/mL chlorhexidine digluconate (Eludril), diluted in 5 mL of water. The sutures were removed 10 days after the operation. The day after the transplantation, the patient was observed and questioned regarding the occurrence of pain, swelling, and bleeding. Follow-up examinations were performed 10 days, one month, three months, six months, and annually after the transplantation. In each follow-up examination, a radiographic examination and sensitivity testing to cold and percussion were performed, as well as observations of the gums, the marginal groove, and dental mobility.
Orthodontic treatment began three months after the transplantation, because orthodontic movement after periodontal healing could prevent the ankylosis of autotransplanted teeth. After a temporary resin-based composite build-up on the natural premolar crowns, they were shaped into upper central incisors (). Orthodontic treatment was performed and transplanted teeth were clinically evaluated through percussion, mobility tests, and radiography. Due to the young age of the patient, a period of time was allowed to pass with monthly vitality checks, in the hope that revascularization would occur. However, revascularization did not take place. The transplanted teeth developed pulp necrosis, and root canal treatment was performed (). The periodontal ligament healed and healthy periradicular tissues were maintained.
A satisfactory aesthetic result and functional occlusion were obtained. The 7 year follow-up showed maintenance of aesthetic improvements, adequate function of the transplanted and reshaped teeth, and radiographic evidence of periradicular tissue health ().


A 65-year-old white male patient had had an asymptomatic, smooth-surfaced, erythematous
nodular lesion of 1.2 cm in diameter, with telangiectasia and progressive growth on the
left nasal ala for eight months (). The
initial diagnostic hypothesis was basal cell carcinoma. Histopathological examination
revealed a preserved dermis and epidermis with formation of lymphoid follicles.
Lymphocytic cells were identified both in the periphery and center of the follicle
(). Immunohistochemistry was positive
for CD20, CD10 and Bcl-6 in cells of the germinal center and negative for Bcl-2, thus
confirming the diagnosis primary cutaneous follicle center lymphoma (). Laboratory tests showed normal blood count
and normal Lactate Dehydrogenase (LDH) levels, negative VDRL test, and negative anti-HIV
and anti-HTLV 1 antibodies. CT scans of the chest/abdomen and pelvis showed no signs of
malignancy and the bone marrow biopsy/aspirate showed no abnormalities. The patient
underwent surgical excision of the lesion ().


A 19 year-old, otherwise healthy male presented with a 2-year history of an asymptomatic
frontal lesion. A firm, erythematous, non-tender, frontal papule was found (). Definitive diagnosis was uncertain with
physical examination and the differential diagnosis included a dermal nevus, a Spitz
nevus, an adnexal tumor and a spontaneous keloid. Complete excision and microscopic
examination was performed, showing a non-capsulated, dermal, fusocellular-spindle cell
tumor with eosinophilic cytoplasm, including mild, cellular pleomorphism and moderately
dense fibrous stroma without myxoid matrix (). No atypical cells or mitotic figures were seen. IHQ was positive for CD10
and negative for S100 and Claudin-1 ().
This was compatible with cellular NT. At the 6-month follow-up, the patient was
asymptomatic without recurrence signs and excellent healing process.


Male patient, 53 years old, presented vegetating plaque on the back of the tongue and
telangiectasias on the lips, gums, palate, oral and nasal mucosa as well as on fingers
for 10 years (). He complains also of
recurrent epistaxis of varying intensity ever since his childhood. Routine laboratory
tests showed mild anemia and iron deficiency (hemoglobin 11.9 g/dL; serum iron levels 56
mcg/dL), since there was no major bleeding in the past months.
Mother (who died at age 45 due to stroke), sister and daughter have similar lesions.
Histology of nasal injury point to vascular dilation and deposition of hemosiderin
().
Bronchoscopy, esophagogastroduodenoscopy and colonoscopy show diffuse telangiectasias
without signs of recent bleeding ( and
). As the most serious complaint of the
patient was nose bleeding, he was referred to otolaryngology and is being monitored to
exclude other possible foci of AVMs.


A 52-year-old male patient arrived in the accident and emergency department after suddenly collapsing during his daily work. His mental functions were normal; he was conscious and cooperative. He had abdominal pain of 1 month’s duration and had extreme pruritus in his body for 4 months. The history of the patient was insignificant. Physical examination revealed hepatomegaly and upper-quadrant repletion. There was widespread abdominal tenderness. His blood pressure was 80/50 mmHg and his heart rate was 128 bpm. Laboratory parameters were as follows: hemoglobin: 8 g/dL (normal range: 12.2–18.1), leukocytes 14.4 10^3/uL (4.3–10.3), alanine aminotransferase 87 U/L (0–40), aspartate aminotransferase 221 U/L (0–41), direct bilirubin 0.30 mg/dL (0.0–0.20). The other parameters were in the normal range.
After these findings were determined, the patient went to the emergency radiology department. Abdominal US revealed multiple liver masses with a maximum axis diameter of 13 cm. Several of the lesions constituted markedly irregular liver contour lobulations. There were also copious free fluids in the abdomen. The peritoneal aspiration material was bloody. The patient was directed to abdominal dynamic CT.
CT revealed multiple nodular liver lesions with a long axis of a maximum of 133 mm. The lesions were heterogeneous and had cystic areas. Varied masses had contact with the liver contour and several of these lesions resulted in being unable to select contour integrity. The liver lesions had heterogeneous contrast enhancement in the arterial phase. Contrast material output leakage in the arterial delayed phase images was demonstrated outside of the liver in the neighborhood of these areas. Delayed phase images revealed focal contrast material accumulation in these lesions and several of the lesions demonstrated contrast material washout ( and ). We diagnosed the patient with hemoperitoneum secondary to liver lesion rupture. The contrast enhancement and washout characteristics of the liver lesions were compatible with multifocal HCC (noninvasive tissue characterization).
The patient underwent surgery and the pathological result was concordant with HCC in a non-cirrhotic liver. Cellular differentiation of HCC was G1. The serology findings were negative for hepatitis B virus (HBV) and hepatitis C virus (HCV).


A female patient aged 17 years reported to Faculty of Dentistry, SEGI University for a routine dental check-up. The patient was of Chinese ethnicity and was accompanied by her mother. Patient's medical history revealed underlying Williams syndrome. Detailed history, extra-oral examination, intra-oral examination, and radiographic evaluations were performed. Extra-oral examination revealed the long face, broad forehead, wide cheeks, underdeveloped mandible, pouting lips, and widely spaced eyes. The characteristic “elfin facies” seen in Williams syndrome was noticed []. Intra-oral examination revealed Angle's class III malocclusion []. The mandibular right lateral incisor was screwdriver shaped [Figure and ]. Plaque and calculus deposits, especially in the mandibular anterior region, deep pits and fissures and dental caries, was noticed in maxillary left and right second molars []. Intra-oral periapical radiographs were taken which showed generalized widening of the periodontal ligament space [Figure –]. Pulp vitality tests revealed that the teeth were vital. No history of trauma was reported. A treatment plan involving oral prophylaxis, pit and fissure sealants, and restorations for carious teeth were planned for the patient. Due to the various systemic complications associated with Williams syndrome, the patient was referred to the physician for medical opinion and consent for the above mentioned dental treatment.
Patient's physician provided the medical consent for dental treatment, along with the medical history of the patient. Williams syndrome was diagnosed by FISH test 7q11.2 deletion. The patient had a history of epilepsy for which she was taking anticonvulsants. However, anti-convulsants were stopped since 2 years in the patient. Ultrasound examination of the kidney and the neural artery Doppler studies were also normal in the patient. The patient had a history of transient hypertension, but her blood pressure was normal without any medication. The patient had some learning difficulties but was otherwise very independent, cheerful and friendly with good self-esteem.
The treatment needed for the patient was explained, and informed consent was obtained from the patient. Oral prophylaxis, composite restorations in maxillary right and left second molars, and pit and fissure sealant in mandibular right and left first molars were performed in the patient. The patient was advised for regular follow-up and an orthodontic consultation for malocclusion.


A 27-year-old male patient presented to us with painless hard mass in superonasal quadrant (SNQ) of left orbit for 3 months which had progressively increased in size during 1-month associated with drooping of upper lid of left eye for 20 days []. The patient has no significant past, personal, systemic and family history.
On examination, best corrected visual acuity was 20/20 in both eyes. Intraocular pressure in both eyes was OD-13 and OS-14 by Perkins applanation tonometer. Anterior segment and posterior segment of both eyes were normal except for approximately 2 mm downward and 2 mm outward dystopia of left eyeball. Full movements of both eyeballs were noted. Exophthalmometry of both eyeballs measured by Hertel's exophthalmometer were OD-14 mm and OS-15 mm and interpuppilary distance was 62 mm.
Computerized tomography scan report showed approximately 13 mm × 14 mm sized well defined minimally enhancing soft tissue density lesion in SNQ of left orbit. The lesion was found in extraconal compartment causing downward and outward displacement of the left eyeball. Scalloping and erosion of the lateral wall of left frontal sinus were noticed [].
Patient was operated for left-sided anteromedial orbitotomy through lid crease approach under general anesthesia. Though mass was found to be adherent to the bone, it was separated in toto by blunt dissection and sent for histopathological examination. Intraoperative and postoperative course were uneventful.
On gross examination, mass was a well-circumscribed nodule measuring 1.5 cm × 1.8 cm, having glistening white multilayered appearance []. Microscopical examination revealed a multilobulated lesion composed of cytologically benign appearing spindle cells. The tissue was composed of round to ovoid nuclei in lacunar spaces suggesting cartilaginous differentiation with little pleomorphism and no mitotic figures []. Immunohistochemical studies were positive for S-100 protein consistent with a cartilaginous tumor and for vimentin, consistent with a mesenchymal lesion, suggesting benign cartilaginous tumor - chondroma. Patient was symptomatically better 1 moth post operatively [].
Patient was advised to follow-up after 6 months or in case of recurrence of symptoms.


A 31-year-old gravida 2 para 1 with a gestational age of 37 weeks 6 days presented with the diagnosis of placenta percreta, which had invaded the bladder. Her past obstetric history included a previous caesarean section for placenta praevia. In order to avoid obstetric hysterectomy, which would have led to sacrificing a part of the bladder, a staged procedure that involved bilateral internal artery balloon catheterisation, caesarean section delivery, and embolisation was planned.
An informed written consent was obtained after explaining the risks and benefits involved in the procedure. Adequate blood was reserved, and the blood bank was informed about the possibility of massive blood transfusion. Keeping the possibility of conducting a caesarean section in the interventional radiology suite, arrangements were made with the required anaesthesia equipment to deal with the airway, operating instruments, neonatal resuscitation, and portable lights for such an emergency.
In the interventional radiology suite monitoring along with invasive arterial blood pressure was established. Two wide bore 18 gauge cannulae were secured. Under local anaesthesia, bilateral internal iliac artery balloons were placed through right femoral artery []. Injection heparin 3000 units was given intravenous (I.V.) Accompanied by the anaesthesiologist and trained staff, the patient was transported to the operation theatre with adequate monitoring. During the procedure, and during the transportation, a wedge under the patient's right hip to facilitate left uterine displacement and also protect the femoral artery sheath. In the operation theatre, general anaesthesia was instituted. Post-delivery oxytocics were avoided to prevent placental separation. The balloons in the internal iliac arteries were inflated intra-operatively to control the haemorrhage. The total blood loss during surgery was 800 ml. A healthy baby of 3.2 kg was delivered. Crystalloids were used during the procedure.
In order to embolise the internal iliac artery [], the patient was once again transported to the interventional radiology suite with monitoring and ventilation. At the end of the procedure in the radiology suite, patient was reversed and extubated. The placenta was left in situ, and she received 4 doses of methotrexate 1 mg/kg I.V. along with leucovorin rescue 0.1 mg/kg in the post-operative period. Serial ultrasonography and beta human chorionic gonadotropin showed a downwards trend. The patient was discharged on 7th post-operative day without any complications. Complete resorption of the placenta with the absence in the Doppler flow took 14 weeks.


A 30-year-old adult male presented with complaints of tingling and numbness in left lower extremity since last 6 months which progressed further within next 2 months to involve the right lower extremity. 2 months later, the patient started experiencing difficulty in walking and standing from sitting position, associated with shooting pain in the lower back region. No bowel and bladder involvement was present. There was no history of any congenital spinal deformity, any spinal surgery or any other spinal procedures like a lumbar puncture.
On physical examinations, there was no motor or sensory deficit in either extremity. No cutaneous abnormalities or dermal sinus tracts were found. No congenital spinal deformity was seen. Neurological examination showed normal superficial and deep tendon reflexes. The laboratory parameters were unremarkable.
The magnetic resonance imaging (MRI) scan revealed a well-defined intradural extramedullary mass lesion measuring 2.7 cm cranio-caudally × 1.6 cm transversely × 1.1 cm anteroposteriorly, located opposite L4 vertebrae level predominantly on right side leading to compression and displacement of cauda equina nerve roots toward left side. The mass was hypointense to cord on T1-weighted images and hyperintense on T2-weighted images and showed minimal enhancement on postgadolinium images [Figure –]. There was no lateral extension of the mass into lateral recess. The rest of spinal cord, cord termination, and conus medullaris were normal in signal thus ruling out spinal cord edema or myelomalacia. Intervertebral discs were found to be normal. No prevertebral and paravertebral collection was present. Surgical intervention was planned, and the patient underwent total resection of the tumor along with L3 partial and L4 complete laminectomy performed in the prone position. On incising the duramater via midline incision, a yellow, oval-shaped, fatty cyst was observed []. Following the incision into the tumor tissue, whitish fluid containing grayish soft tumor tissue was found []. Separation of the capsule and total removal of the tumor were possible. The histopathology showed ciliated pseudostratified columnar epithelium, keratin debris, adipose tissue, and glandular tissue []. Histopathology examination suggested that the overall features were consistent with mature cystic teratoma [Figures –]. The dura mater was closed in a watertight manner. Postoperative period was uneventful, and the patient was discharged on day 7 without any neurological deficit or signs of meningitis. No further neurological deterioration was observed during the 3-month follow-up period.


A 29-year-old man had received an L4–L5 laminectomy and discectomy surgery 3 years prior to his admission to our ward complaining of low back pain radiating to the right lower leg for 3 months. He underwent an L45S1 laminectomy + L4/5 and L5/S1 discectomy operation. Twelve hours later, he developed right lower quadrant pain, with suspicion of acute appendicitis. The diagnostic laparoscopy showed a retroperitoneal hematoma and no acute appendicitis []. An emergency abdominal computed tomography showed the rupture of a pseudoaneurysm from the right internal iliac artery []. His hemodynamic status became unstable, so we performed a transarterial embolization with multiple metallic coils in the aneurysm cavity and connected the proximal and distal right internal iliac artery after angiography diagnosis [Figures and ]. After the embolization and a massive fluid and blood transfusion, his hemodynamic status stabilized. Finally, he was discharged smoothly and reported no new neurological problems 10 days after admission.


A 32-year-old woman reported to Department of Periodontology, TPCT's Terna Dental College, Nerul, Navi Mumbai with the chief complaint of swelling in upper left back region of the mouth since 3 months. Patient's history revealed that a small swelling appeared 3 months back on left buccal aspect of the jaw, which gradually extended palatally and increased to attain present size. The patient reported the lesion to be asymptomatic, but bleeding tendency was observed if accidentally bitten on during mastication. Patient also gave the history of mishri use twice daily since 10 years. Extraoral examination did not reveal any pathological findings and facial asymmetry. Medical history of the patient revealed no systemic diseases; examination of lymphonodes in the head and neck region revealed no lymphadenopathy. Patient was systemically healthy and was not taking any medications.
Intra-oral examination revealed a nodular growth on the maxillary buccal interdental gingiva in relation to the second premolar and first molar which was extending to palatal mucosa. Swelling was exophytic, sessile, bluish-red in color, firm in consistency which measured 1 cm × 1 cm on the buccal aspect []. Palatal swelling was pedunculated, irregular and multilobulated which measured 2.5 cm × 2.5 cm []. Other oral findings included an over-retained root piece of deciduous second molar distal to the growth and overall simplified oral hygiene index for the patient was 3.66 indicating poor oral hygiene.
Her complete blood count revealed normal results. Superficial erosion of the alveolar crest in relation to the growth was seen in periapical radiograph [], such superficial erosions of alveolar bone are usually observed in cases of PGCG.[] In occlusal radiographs, no significant bone loss was found []. Orthopantomogram of patient showed interdental bone loss with second premolar and first molar []. Cone beam computed tomography revealed soft tissue growth originating from left maxilla measuring 23.28 mm × 16.57 mm in greatest dimensions [Figure and ].
After phase I periodontal treatment, incisional biopsies were performed for both the buccal and palatal lesions. Biopsy specimens were embedded in 10% formalin and sent to Department of Oral Pathology. Routine histological examination with hematoxylin and eosin (H and E) stains were performed. After confirmation of the diagnosis of the lesion complete surgical excision was performed []. The H and E section showed hyperplastic parakeratinized stratified squamous epithelium exhibiting acanthosis and ulceration. The underlying connective tissue stroma showed high cellularity. Nodular proliferation of multinucleated giant cells along with mesenchymal cells was observed, with few areas of hemorrhage interspersed with zones of bone formation and chronic inflammatory cells [Figure and ]. The microscopic features of the lesion were consistent with PGCG.
Due to likelihood of recurrence, a wide excision of the lesion was performed to minimize the chance of further recurrence. Subgingival band of calculus was observed with second premolar region after excision of the lesion []. High power diode laser of wavelength 810 nm with a treated tip in continuous mode at 0.6–1 W was used to cauterize the bleeding tissue. To extend the periphery of excision treated tip in continuous mode at 1.2–1.5 W was used [Figure and ]. The excised specimen was embedded in 10% formalin and sent to the Department of Oral Pathology. The histology of the excised lesion re-confirmed the diagnosis of PGCG.
Healing was uneventful till 4 months postoperative [Figure and ]. Patient is still under follow-up.


A 10-year-old female child seropositive for HIV (acquired perinatally) presented to our dermatology outpatient department with a swelling on left cheek, which had gradually increased in size over a period of 6 months. In addition, she also complained of multiple skin coloured to white lesions over face, neck, lower abdomen, both hands and lower extremities since 6 months. Patient didn’t have any other systemic complaints. There was no history of tuberculosis, herpes zoster, diarrhoea, or any other opportunistic infections. One-year back, patient had been started on anti-retroviral therapy (ART) (zidovudine, lamivudine, nevirapine [ZLN]) in a government ART centre on account of her low CD4 count (222 cells/mm3 [normal: 448–1611 cells/mm3]). However she had poor compliance and her CD4 count was consistently low (ranging between 172 and 224 cells/mm3) during the year.
On examination there was a single, well defined, lobulated, tumoral swelling 5 cm × 6 cm × 7 cm in size on the left cheek []. Skin overlying the swelling was thinned out with surface showing evidence of ulceration, haemorrhagic crust with slough. Swelling was freely mobile over underlying structures. Multiple, discrete, pearly white, umbilicated papules were present over cheek, forehead, upper eyelids, nose, lower extremities and buttocks. There was no lymphadenopathy. Systemic examination including central nervous system examination was normal. A differential diagnosis of giant molluscum contagiosum, keratoacanthoma, squamous cell carcinoma and cryptococcosis was considered.
On investigations, she had anemia (Haemoglobin: 7.6 g/dl [Normal: 9–11 g/dl]) and CD4 count was 251 cells/mm3. Other investigations including total leukocyte count, liver function tests and renal function tests were normal. Viral load estimation could not be done as the patient's parent refused to do the same due to financial constraints at that time. A punch biopsy of the tumoral swelling on left cheek revealed acanthosis with keratinocytes showing intracytoplasmic eosinophilic inclusion bodies, which were becoming basophilic in the granular layer. Thus a diagnosis of giant molluscum contagiosum was made.
After correction of anemia, complete excision of the giant molluscum contagiosum was done followed by split thickness skin grafting. The cut surface of the excised lesion showed multiple gyri like corrugations corresponding to the marked acanthosis seen on histopathology. At the base of the excised lesion, multiple pearly white papules could be seen []. On histopathological examination [], there was marked acanthosis, keratinocytes showing intracytoplasmic eosinophilic to basophilic inclusions which were extruding out with keratinous debris. The epidermal ridges extended deep into the dermis surrounded by a dense lymphohistiocytic infiltrate with multiple giant cells and few plasma cells/neutrophils. Within the infiltrate, occasional degenerated molluscum bodies were seen. The infiltrate also extended into the subcutaneous fat resulting in septal panniculitis with extensive fibrosis. One-month later, there was complete healing at the site of skin grafting with minimal scarring []. Rest of the lesions were treated with topical 10% trichloroacetic acid (TCA) application resulting in gradual resolution. The patient was continued on ART (ZLN) with thorough counselling regarding compliance with medication. Her CD4 count improved to 676 cells/cmm after 10 months and had undetectable viral load. One-year later, on follow up, there has been no recurrence of lesions.


A 61-year-old male underwent a left upper lobectomy in January 2014. The lobar bronchus was cut and closed with a suturing device. A pedicled intercostal muscle flap or pericardial fat tissue was not used to cover the bronchial stump because there were no risk factors for broncho-pleural fistula (BPF) such as diabetes. The patient's postsurgical course was not particularly eventful. He was dismissed 8 days after surgery but was re-admitted a week later due to excessive retention of pleural fluid on the operated side. The computed tomography (CT) scan images showed nothing remarkable other than a few tiny air spaces around the bronchial stump (Fig. ). The patient complained of an obstinate dry cough that had not improved since his operation. He was discharged 11 days after his second admission, after successful control of pleural fluid. Neither microorganisms nor cancer cells were present in the fluid.
Throughout this period, minor inflammation with at most 3.2 mg/ml of C-reactive protein, but with no fever, was observed. On the fourth day after leaving our hospital, he had a sudden massive hemoptysis and was taken to the emergency unit of a nearby hospital. His blood leukocyte count was 8200/μl, and his serum C-reactive protein level was 2.8 mg/ml; this was almost the same as during his last examination before leaving our hospital. The patient's wife explained that he had had fresh hemoptysis several times in a couple of hours before the event. The patient died 3 h after the initial massive hemoptysis and did not undergo an emergency thoracotomy. The postmortem exam revealed that a BPAF had formed between the upper lobe stump and the left pulmonary artery trunk (Fig. ). The precise location of the ruptured pulmonary artery was approximately one-third of an inch away from the bifurcation of the centermost pulmonary artery branch. After the left upper lobectomy, the pulmonary hilum became extremely stiff due to the underlying chronic inflammation.


A 52-year-old Caucasian woman was admitted to our hospital from the nursing home due to refusal of percutaneous endoscopic gastrostomy tube feeding and altered mental status, which was attributed to hypoglycemia and urinary tract infection. Her medical history was significant for long-standing severe anorexia nervosa, depression and recent percutaneous endoscopic gastrostomy tube placement for nutritional support at another hospital. She was allergic to ciprofloxacin and penicillin and her nursing home medications included multivitamin, megestrol, ferrous sulfate, calcium carbonate, ranitidine, thiamine, folic acid, and olanzapine.
Upon admission, her weight was 21 kg and her BMI was 7.9, which amounted to approximately 40%percnt; of her ideal body weight. The vital signs were normal. The patient was bed bound, alert and well oriented. The physical exam revealed dry skin, excoriations all over the body, brittle nails, pallor and profound emaciation with bitemporal wasting. Her abdomen was scaphoid without organomegaly, and she had a percutaneous endoscopic gastrostomy tube with granulation tissue surrounding the stoma. There were no stigmata of chronic liver disease. The remainder of her physical examination was unremarkable.
Laboratory investigations revealed hemoglobin of 8.4 g/dl, white cell count of 3.5 k/μl, platelet count of 230 k/μl, prothrombin time of 12 s and activated partial thromboplastin time of 29 s, normal serum electrolytes except for a blood sugar level of 56. Her liver function tests showed serum alanine aminotransferase of 32 units/l, serum aspartate aminotransferase of 32 units/l and serum total bilirubin of 0.6 mg/dl.
She continued to refuse oral and tube feeding and she had intermittent episodes of hypoglycemia. She was evaluated by psychiatrists and was deemed to lack decisional capacity. It was recommended that percutaneous endoscopic gastrostomy tube feeding be imposed for nutritional support. After consultation with the nutritional team, tube feeding was begun at 400 kcal/day and advanced gradually over the next 5 days to 900 kcal/day.
Five days after the initiation of feeding, she developed tachycardia and lab work revealed electrolyte abnormalities such as hypophosphatemia (phosphorus 0.8 mg/dl) and hypokalemia (potassium 2.9 mEq/l) (fig. ). An electrocardiogram was done, which showed sinus tachycardia with some premature ventricular complexes and prolonged QT interval (QTc 472). She was then evaluated by cardiologists and nutritional teams. A diagnosis of refeeding syndrome was made, and the patient was transferred to the telemetry unit for further management. Further laboratory tests revealed elevated transaminases (alanine aminotransferase 193 units/l, aspartate aminotransferase 173 units/l), alkaline phosphatase (392 IU/l) and bilirubin (1.4 mg/dl), which were normal upon admission (fig. ). A consultation with a gastroenterologist was called. Viral hepatitis was excluded by serological tests and autoimmune workup, including anti-smooth muscle antibody, anti-liver/kidney microsomal antibody, antinuclear antibody and antimitochondrial antibody, was negative. Serum ceruloplasmin level was normal. Other causes of hepatitis such as hepatotoxic drugs were excluded. The liver and pancreas appeared normal on a computed tomogram. An abdominal ultrasound revealed normal echotexture of the liver without intra- or extrahepatic biliary dilatation and normal Doppler evaluation of the hepatic and portal vein blood flow.
Infusions of potassium phosphate and magnesium sulfate were administered in addition to oral potassium phosphate supplements. Intravenous thiamine was given. Her enteral intake was reduced and daily electrolyte levels were obtained along with regular monitoring of the liver function tests. The liver enzymes started improving after reduction of the tube feeding rate. Electrolytes also normalized over the next few days and the patient was eventually discharged.


A 77-year-old Japanese female was diagnosed with chronic hepatitis C in 1990. A HCC (13 × 10 mm) was detected in segment 7/8 on a computed tomography (CT) scan and treated with percutaneous ethanol injection therapy in April 2007. Subsequently, recurrence of the HCC (23 × 13 mm) was observed in December 2010 in the same segment, and microwave coagulation therapy was performed. In April 2011, a chest X-ray image and CT scan revealed multiple lung metastases (fig. ). No brain or bone metastases could be detected.
Laboratory test results at the time of diagnosis were as follows: albumin 2.9 g/dl, aspartate aminotransferase 30 IU/l, alanine aminotransferase 15 IU/l, total bilirubin 0.5 mg/dl and prothrombin international normalized ratio 1.05. The patient had good reserve liver function with a Child-Pugh score of 6 (Child-Pugh class A) and did not have any ascites or findings of encephalopathy. Tumor markers such as α-fetoprotein (AFP; 76,170 ng/ml), the lens culinaris agglutinin-reactive fraction of AFP (7.5%percnt;) and des-γ-carboxyprothrombin (DCP; 63,400 mAU/ml) were elevated. Sorafenib administration was initiated at a reduced dose of 400 mg/day, because of the patient's advanced age and low body mass index (BMI) of 19.7.
After 4 months of sorafenib administration, the tumor markers decreased to normal levels (AFP 7.5 ng/ml, DCP 30 mAU/ml), and the metastatic lung lesions had disappeared (fig. ). We considered that the patient had achieved a CR according to the RECIST evaluation. The patient continues to be treated with sorafenib at a reduced dose of 400 mg/day without adverse effects and is still maintaining a CR.


A 20-year-old male was found dead in a function hall with history of electrocution. We received a specimen of the heart, two pieces of lungs, one half of each kidney, skin piece over electric contact mark, and control skin piece. On autopsy, all organs showed minimal congestion. Petechial hemorrhages were present in the heart, the lungs, and the brain.
The heart was grossed by the inflow and outflow method along the course of blood flow. The external surface showed soldier's plaques over the right lateral border. The weight and measurement values of the heart are depicted in . The pulmonary arterial trunk and the valves were normal. However, on dissecting the aorta, right coronary ostia were two in number [] and left coronary ostia was only one in number. The ostia and course of the two coronary arteries were traced. Anterior RCA arising from one of the ostia coursed anteriorly. Anterior RCA coursed anteriorly descending downward, directed toward the apex. Posterior RCA from the other ostia coursed toward the junction between the right atrium and the right ventricle. The aorta also showed numerous foci of atheromatous plaque up to 0.5 cm from the root of the aorta. All the arteries appeared patent. Microscopy showed foci of neutrophil aggregates in the anterior wall of the left ventricle. The aorta showed features of atherosclerosis. However, sections from both the RCAs, left coronary artery, left anterior descending artery, and left circumflex artery revealed no significant pathology.
Sections from the lungs showed evidence of areas of patchy consolidation, areas of congestion, edema, and hemorrhage. Sections from the kidneys showed areas of congestion, edema, and hemorrhage. Sections from the skin tissue labeled as skin showed features of electric current injury. The control skin showed no significant pathology.


A 6-year-old female had history of electric contact and expired after 1 h. We received the heart, four pieces of the lungs, and one half of each kidney. The heart and the lungs appeared congested on autopsy.
The weight and measurement values of the heart are depicted in . The pulmonary arterial trunk and the semilunar valves were normal. The aorta showed two right coronary ostia[ ]. The courses of the arteries from two right coronary ostia were similar to that of the first case. The posterior RCA that coursed toward the junction between the right atrium and the right ventricle showed partial obliteration. Other coronary arteries and their branches were patent. Microscopy of the posterior RCA revealed obliterative arteritis with neutrophilic infiltration in the intima and media []. Sections from the rest of the myocardium and other arteries showed no significant pathology. Sections from the lungs and the kidneys showed areas of congestion, edema, and hemorrhage.


Mr. A is a 16-year-old boy with congenital hydrocephalus who presented with left sided chest pain of 2 months duration. The chest pain was insidious in onset, progressive, and pressure-like. Initially, he had a ventriculoperitoneal (VP) shunt placed which drained into his peritoneal cavity. This shunt ruptured and was replaced with a ventriculopleural shunt that drained into his left pleural cavity [Figure and ]. The patient got a chest X-ray done, which showed a left-sided pleural effusion and left basilar atelectasis []. An ultrasound of his chest showed a large left pleural effusion without evidence of loculations []. Computed tomography (CT) scan of the chest confirmed the shunt in the left lower posterior pleural space with the pleural effusion [Figure –]. A nuclear medicine scan was performed with technetium 99m MAA, which showed radiotracer uptake in the reservoir of the shunt and the left pleural space []. The patient was treated surgically with thoracentesis and continued to do well.


Mr. B is 29-year-old male with a history of obstructive hydrocephalus, and a ventriculoperitonal shunt placement [] came in with complaints of abdominal pain of 6 days duration with a 3 day history of nausea and vomiting. He had an abdominal X-ray which was inconclusive []. An ultrasound of his abdomen showed a large loculated, hypoechoic fluid collection extending from his xiphoid process to just below the umbilicus with a portion of the VP shunt inside [Figure and ]. He had a CT scan of his abdomen and pelvis which showed a large fluid collection containing a portion of the VP shunt inside [Figure and ]. He got a nuclear medicine scan with technetium diethylene-triamine-pentaacetate which showed ventriculomegaly and free spill of contrast into the peritoneal cavity []. The patient was treated surgically and got his VP shunt replaced with a ventriculoatrial shunt.


A 59 year-old man admitted to our emergency center complaining of pain in the right upper quadrant area which had abruptly started 1 day before. During the assessment, he felt dizziness and had mild fever. He had been treated for hypertension for 5 years. There was no past history of liver or biliary diseases. On physical examination, mild tenderness in right upper abdomen was felt. The laboratory results were as follows: hemoglobin, 13.1 g/dl (reference range: 13-18 g/dl), white blood cell, 12.44×103/µl (reference range: 4-10×103/µl) and platelet, 281×103/µl (reference range: 150-450×103/µl). The results of serum chemistry and electrolyte were as follows: AST, 205 IU/L (reference range: 5-35 IU/L) and ALT 325 IU/L (reference range: 5-40 IU/L). Other results were within the normal limits. Plain chest and abdomen X-rays showed no abnormal findings. Serum carbohydrate antigen (CA) 19-9 was 34.9 IU/ml (reference range: 0-33 IU/ml) and serum alpha-fetoprotein (AFP) and chorioembryonic antigen (CEA) were 2.58 IU/ml (reference range: 0.-5.5 IU/ml) and 7.32 IU/ml (reference range 0-4.6 IU/ml), respectively.
An abdominal computed tomography (CT) showed a homogenous low density lesion in the anterior section of the liver and a heterogeneous enhanced mass lesion in the delayed phase with the enhanced peripheral bile duct in the posterior section (). In magnetic resonance imaging (MRI) study, a large tumor of the right posterior section in the liver showed heterogeneous lower signal intensity in T1-weighted image and heterogeneous high signal intensity with necrosis and hemorrhage on mass in T2-weighted image. It was associated with focal dilation around the bile duct and hepatic capsular retraction of the posterior section in the liver. The large hematoma of the right anterior section was connected with the heterogenous tumor of the posterior section by high signal intensity in the right perihepatic space that was suspected as hemorrhage (). Intrahepatic cholangiocarcinoma was highly suspected through MRI and CT findings. We concluded that the large subcapsular hematoma was caused by ruptured cholangiocarcinoma.
After 1 week of conservative treatment, a follow-up CT scan showed no internal bleeding but increase in hematoma size. The patient was discharged from the hospital for personal reasons and re-admitted after 1 month. The tumor was further increased in size compared with the previous CT scan findings.
Upon readmission, the patient underwent a laparotomy. The intraoperative examination revealed a small amount of ascites in the peritoneal cavity. The tumor had strongly adhered to the diaphragm and a 3 cm-sized metastatic nodule was found in the diaphragm adjacent to the live dome without peritoneal seeding or lympadenopathy. Right hemihepatectomy with cholecystectomy and radical lymph node dissection was performed. The tumor was measured 18×15×10.5 cm. On the cut section, the surface showed massive necrosis with hemorrhage and 6.5 cm of cystic change (). In histological examination, the tumor showed a malignant neoplasm with a carcinomatous component and a sarcomatous component. The former consisted of moderately differentiated adenocarcinoma, while the latter consisted of a poorly differentiated sarcoma (). Immunohistochemical examination of the carcinomatous component revealed positive staining for epithelial membrane antigen (EMA), pan-cytokeratin (CK) stain and CEA stain and negative staining for vimentin. This immunoprofile was in keeping with cholangiocarcinoma. The sarcomatous component was positive for vimentin but negative for pan-CK and S-100 proteins (). Based on the histological and immunohistochemical findings, a diagnosis of sarcomatoid cholangiocarcinoma was made and the patient was referred to the department of Hemato-oncology for consideration of adjuvant chemotherapy.
The patient was started on chemotherapy combined with 5-fluorouracil and cisplatin, which he tolerated well. A postoperative contrast CT at 3 months after adjuvant chemotherapy confirmed disease recurrence with 2 small nodular lesions that had grown in the right subphrenic space (1 cm) and the omentum (8 mm) that was suspicious of peritoneal seeded metastasis. Since the follow-up CT, he received gemcitabine (1,000 mg/m2) and cisplatin (30 mg/m2) chemotherapy in a 21-day cycle. The patient received a total of six cycles and a follow up CT scan showed a partial response. At present, he is doing well for 8 months post-initial presentation.


A 75-year-old male was admitted with a 15-hour history of fever and right upper quadrant abdominal pain. His pain was not controlled by a pain killer and he was irritable. He was on Warfarin 2 mg for atrial fibrillation for 5 years without complication. His past medical history included hypertension, smoking but no diabetes. A physical examination revealed direct tenderness and rebound tenderness in the right upper abdominal quadrant with rigidity. His white cell count was 14.1×103/ul (neutrophils 86.1%) and erythrocyte sedimentation rate (ESR) 17 mm/hr. The results of chemistry studies included a total bilirubin of 1.77 mg/dl with direct bilirubin 0.64 mg/dl, aspartate aminotransferase of 640 IU/L, alanine aminotransferase of 167 IU/L, gamma-glutamyltransferase of 375 IU/L. At presentation, his prothrombin time (PT) was elevated measuring 28.4 sec, INR was elevated measuring 2.4, but aPTT was not elevated. The CT scan of the abdomen with intravenous contrast showed few gallstones with mild gallbladder wall thickening, high density in gallbladder and bile duct. A CT scan suggested sludge, a stone or tumor obstructing the distal common bile duct combined with acute cholecystitis that was unlikely hemobilia or hemorrhagic cholecystitis ().
Based on the patient's symptoms, signs and CT findings, the patient underwent an urgent laparoscopic cholecystectomy. A distended edematous gallbladder was found and filled with dark blood clots (). The patient underwent an uneventful laparoscopic cholecystectomy and his pain was controlled. The next day, his bilirubin was elevated (total bilirubin of 7.14 mg/dl; direct bilirubin 2.64). The 3rd postoperative day endoscopic retrograde cholangiopancreatography (ERCP) was performed, which showed a dilated biliary tract with sludge and stones but there was no evidence of hemobilia. Sludge and small stones were removed by an endoscopic sphincterotomy (EST) and endoscopic naso-biliary drainage (ENBD) was performed then removed 2 days later.
A histologic examination of the gallbladder showed acute erosion and a focal gangrenous lesion however it was not clear whether it was from a transmural bleeding. His symptoms and laboratory findings had all improved and anticoagulation therapy was uneventfully reinitiated. He was discharged 9 days after admission. At the follow-up examination 3 months after surgery, he was asymptomatic and doing well.


A 35-year-old man complained with painless progressive asymmetric enlargement of the bilateral thyroid lobes for a year. In the recent 3-month, he was suffered from diuresis and polydipsia. No hereditary syndromes, family history of cancer, and personal history were disclosed. There was no additional past medical or surgical history. Blood tests revealed a normal full blood count, electrolytes, urea, creatinine, calcium, and phosphate levels. Thyroid function tests reported a thyroid stimulating hormone level of 0.97 (normal range 0.3–4.6 uIU/mL), free T4 of 21.78 (normal range 6.6–24.8 pmol/L), and free T3 of 5.80 (normal range 3.8–8.5 pmol/L), thyroglobulin antibodies of 53.20 (normal range <30 U/mL), thyroid peroxidase antibody of 64.00 (normal range 0.16–10 IU/mL). In addition, serum calcitonin was recorded at <1.0 (normal range <11.8 ng/L) and urine catecholamine levels were within normal range. Water pressure test revealed explicitly for central diabetes insipidus, and desmopressin acetate was given for 1-month. USS of the neck, conducted at the referring hospital, reported an enlarged (3.1 cm and 2.8 cm in diameter) bilateral thyroid lobes []. Subsequently, a needle biopsy from the thyroid mass revealed features of langerhans cell with the tumor cells staining positive for CD1a, S-100, negative for cytokeratin []. MRI sections of the head revealing pituitary stalk enlargement []. 18FDG PET/CT was performed for further evaluation of LCH in the whole body []. The maximum projection image of the PET revealed increased uptake in thyroid gland (standardized uptake value [SUVmax] = 9.4) [], pituitary stalk (SUVmax = 4.9) [], and maxillofacial skin lesion (SUVmax = 4.9) [], which corresponded well to bilateral thyroid nodule, the thicking of pituitary stalk, and skin lesion on the CT. However, CT showed the left lung nodule [] and the skull destruction [] without 18FDG uptake.


Patient 1 was a 53-year-old woman with a 6 cm-sized mass in segment VIII. She had undergone an open cholecystectomy 24 years earlier. Her preoperative liver function and tumor marker tests gave normal results. She was diagnosed with an IHCC with left portal vein invasion and of clinical stage IVA (T2N1M0). We performed a right hemihepatectomy including S4b, resection of the bile duct, left portal vein, and lymph node dissection. On gross examination, this patient was found to have a mass-forming type of IHCC, and on microscopic examination, she was diagnosed with an adenocarcinoma with moderate differentiation, vascular invasion, and perineural invasion, with tumor cell spread to the 12p lymph node; the final staging was IVA (T3N1M0). The surgery was uneventful, and she underwent radiotherapy for one month postoperatively. At 39 months after surgery, the patient was surviving with no evidence of tumor recurrence or metastasis.


Patient 2 was a 74-year-old man with chronic hepatitis C infection, hypertension and a history of tuberculosis pleurisy about 55 years earlier. Preoperatively, his PIVKA-II level was elevated highly (>2,400 mAU/ml) and his serum HCV RNA was 2.3×105 IU/ml. Imaging showed multiple, variable sized masses in the right hepatic lobe and segment IV, variable sized nodules in both lungs and an about 1 cm-sized nodule in the left adrenal gland. The largest liver mass was more than 10 cm in size. His preoperative liver function test was in normal range except ICG R15 of 15.6%. We performed a palliative right trisectionectomy. Pathologic examination showed an HCC of trabecular and hepatic types, scored as Edmondson-Steiner Grade 3/2. Microscopically, the tumor showed vascular invasion and multiple satellite nodules. After the surgery, he underwent transarterial chemoinfusion (TACI) twice, followed by regular treatment with sorafenib. At present, 38 months after surgery, the patient remains alive with the improvement of quality of life in spite of residual pulmonary metastases.


Patient 3 was a 50-year-old man with HBV-associated HCC before surgery. He had a huge mass replacing his right hemiliver with malignant thrombosis in the right posterior portal vein and invasion of the right and middle hepatic veins (). His serum AFP concentration was 298,000 ng/ml. He had started to take sorafenib at the time of diagnosis and underwent TACE before surgery. His preoperative levels of AST and ALT were mild elevated (89/57 IU/L) and prothrombin time was mildly prolonged (74.4%). We performed a right hemihepatectomy, diaphragm excision due to invasion, evacuation of tumor thrombus from the hepatic vein, venoplasty of the right hepatic vein and inferior vena cava (IVC) with total vascular exclusion. The tumor was an 11.5×10×6.8 cm-sized HCC with invasion of the diaphragm and portal vein thrombosis (). Despite bad results on a preoperative ICG R15 of 26.7%, he had good postoperative course, and underwent TACI after the surgery. For 12 months after surgery, imaging revealed no unusual postoperative findings and was maintained on sorafenib ().


Patient 4 was a 67-year-old man with HBV-associated HCC. His AST/ALT was mildly elevated preoperatively (140/87 IU/L), and his PIVKA-II level was very high (> 20,000 mAU/ml). Imaging showed two large (10 and 11 cm) nodular HCCs and three tiny intra-hepatic metastatic lesions in the left lobe, tumor thrombus in the right hepatic vein and thromboembolisms in both pulmonary arteries. He was staged clinically stage IVB. Before the operation, he received an IVC filter to prevent aggravation of the pulmonary thromboembolism, and after the operation he started anticoagulation therapy. We performed a right trisectionectomy with partial resection of the diaphragm and partial hepatectomy for a small lesion on the left hemiliver, and total vascular exclusion. Pathology showed 2 large tumors measuring 15 and 10.8 cm on the right hemiliver and one small (0.7 cm) HCC on segment 3. After the operation, the patient recovered well without complications. The patient has continued chemotherapy with sorafenib, along with anticoagulation therapy. At 6 months after surgery, he shows no evidence of recurrent HCC.


Patient 5 was a 45-year-old previous healthy woman with a 5 cm-sized IHCC in the caudate lobe and invasion of the IVC and main portal vein (). At the time of diagnosis, her CA19-9 level was 88.6 U/ml. After 1 month of preoperative chemo- and radio-therapy, she underwent a left trisectionectomy and resection of the IVC. During the operation, we resected the IVC which had been invaded by the tumor, followed by an anastomosis by interpositioning the graft with total vascular exclusion (). Pathologic examination showed that the tumor was a mass forming type of cholangiocarcinoma, measuring 5.8×5.0×4.2 cm with poor differentiation and invading the IVC (). Twenty months after surgery, this patient showed no evidence of recurrence or metastases despite an IVC thrombus with venous congestion. The patient is currently receiving anticoagulation therapy and was waiting for a thrombectomy.


Patient 6 was 64-year-old woman who underwent a right posterior sectionectomy in June 2003 for a mucinous type of IHCC measuring 5×2.5×2.4 cm in size. Six years later, this patient was found to have a recurrent IHCC on the right anterior section, for which she underwent a right hemihepatectomy, S1, and bile duct resection. At that time, the recurrent tumor was diagnosed pathologically as a mucinous, intraductal growth type of cholangiocarcinoma with moderate differentiation, measuring 4.5×3.2× 2.1 cm in size and accompanied by lymph node metastases. One year later, we observed enlargement of a para-aortic lymph node, which was dissected surgically, followed by adjuvant chemotherapy. At 20 months after last surgery, the patient remained alive despite the increase of size and number of recurrent tumors.


Patient 7 was a 49-year-old man who had previously undergone surgery for colon cancer with liver metastasis, consisting of palliative right hemihepatectomy and caudate lobectomy, diaphragm resection, and anterior resection of the colon. However, the metastatic mass in the liver recurred twice at 1-year intervals, for which the patient underwent repeated resections, followed by 34 doses of chemotherapy. At 17 months after last surgery, he remained free from cancer.


A 28-year-old asymptomatic obese female presented to the bariatric clinic for weight reduction surgery. During the routine bariatric preoperative evaluation, examination revealed decreased breath sounds bilaterally and dullness to percussion in the anterior chest. On X-ray, widening of the mediastinum with possible intrathoracic mass was identified and the patient was referred to thoracic surgery. Further laboratory investigation of common tumor markers, such as ABG, AFP, HCG, CA 19.9 and CEA, were all negative along with chemistry and hepatic panels. CT imaging was performed and revealed a large heterogeneous anterior mediastinal mass with fat and soft tissue. With possible differential including thymolipoma, liposarcoma and germ cell tumors, further imaging of the abdomen, brain and bones was performed and did not show any evidence of metastasis. CT-guided biopsy revealed a well-differentiated sclerosing variant liposarcoma with cellular-spindled areas. The cells were positive for CD34, desmin, S100 and MDM2. They were negative for SMA, CD117 and DOG1 (Figs –).
The patient was taken electively for resection of the mass. The surgical approached was via a median sternotomy and the mass was resected without any complications from the anterior mediastinum and with an intact capsule. The smooth lobulated yellow-red mass weighed 1840 g, and measured 28.0 × 19.0 × 8.0 cm. The patient was discharged without any complications and has continued to follow up in clinic without any complaints.


A 42-year-old woman presented with dyspnea and reduced exercise tolerance, which had begun a month before admission. Her medical history included hysterectomy and left salpingoophorectomy for menorrhagia and a left ovarian mass five years ago. Histopathologic examination demonstrated multiple leiomyomas and fibrothecomas of the ovary. Four months later, she complained of spotting. Transvaginal ultrasonography showed a heterogeneous solid mass, about 28 mm × 45 mm, in the cuff of the vagina. She underwent laparotomy and excision of the mass as well as right oophorectomy. Histopathologic examination revealed a LG-ESS. She underwent 28 courses of radiotherapy.
On examination, the patient had dyspnea at rest, tachycardia, and normal blood pressure. She had mild to moderate lower limb edema. Transthoracic echocardiography showed a large multilobulated mass (4.5 cm × 3.5 cm) in the right ventricle (). Computed tomographic (CT) angiography demonstrated tumoral process in the right atrium, coronary sinus, right ventricle, and pulmonary outlet tract as well as multiple metastases in both lung fields (). Findings of abdominopelvic and brain CT scan were normal. Color Doppler ultrasonography of the vessels of both lower limbs revealed normal findings.
Median sternotomy was performed and cardiopulmonary bypass instituted. Right atriotomy revealed a large mass, attached to the septal leaflet of the tricuspid valve, and a mass in the coronary sinus. The ventricular mass, which originated from the posterior ventricular wall, extended to the chordae tendineae. A large red mass was seen in the right ventricle’s outflow tract. The mas was completely resected ().
The patient was extubated in shorter than 10 hours of surgery. She recovered without complications and was discharged from hospital nine days later. Histopathologic examination revealed a metastatic ESS, which was confirmed by Immunohistochemistry (IHC). The estrogen receptor (ER), progesterone receptor (PR), and CD10 were positive in the tumor cells. She was referred to the oncologist for adjuvant therapy. After 28 months, follow-up examination revealed no abnormality. All patient information was kept confidential and for publication of the article, written informed consent was obtained.