A 39-year-old man of Caucasian descent with a positive family history for NF1 disease had been diagnosed with NF1 at the age of 10 years. Two years later he underwent resection of spinal and radicular neurofibromas with stabilization of the vertebral column. He presented at our out-patient service 27 years later with a recurrent soft tissue mass involving the left gluteal region along with paravertebral extension to Th6 through L4. The paravertebral and gluteal finding on the left side was a soft tissue tumor measuring 27×15cm, whereas the paravertebral tumor on the right side measured 10×13cm. The gluteal mass entailed ptosis and bulging (Figure A). Within the paravertebral moiety, a cyst-like fluctuation of 8×8cm was palpable. No pain, swelling or eczema was noted but enlargement of the paravertebral soft tissue tumor could be elicited by pressing. Furthermore, subcutaneous papulonodular indurations were palpable. The patient was heavily impaired by the tumor while sitting or performing physical activity, and reported on being distressed by the cosmetic deformity. Pressure on the tumor provoked sharp pain. After a history of multiple resections of several smaller neurofibromas, the tumor now showed a quick progression during one month. In addition, he developed multiple "café au lait" spots on his back, and bilateral gluteal freckling as well as multiple small neurofibromas on his trunk and both thighs. On his left forehead, a neurofibroma of 3×1.5cm was present. Lisch nodules without retinopathy were observed in both eyes.
An abdominal-pelvic computed tomography scan revealed an extensive mass lesion displacing the soft tissue of the left gluteal region and a fluid-filled vertebral cystic cavity of 8.3×20.3cm (Figures and ). Although no subcutaneous connection between the paravertebral and gluteal findings was demonstrated, the consulting neurosurgeon advised against the resection of what was interpreted to represent a meningocele.
The patient underwent wide partial excision (20×12cm) of the neurofibroma under general anesthesia. Because of the presence of irregular frail and plexus-like veins, subtle hemostasis was mandatory throughout the procedure. Direct wound closure could be achieved by advancement and rotation of the adjacent skin. Seven weeks following surgery, there was significant reduction of the left paravertebral and gluteal bulging, and an improved symmetry of the lumbar and gluteal regions (Figure B). The patient also reported a reduction in the localized pain he had experienced prior to resection and remained satisfied at 1 year follow-up, with no recurrence of the neurofibromatous mass.
Following overnight fixation in 10% buffered formalin, the resection specimen was routinely processed to paraffin, and 3μm-thick slides were stained with hematoxylin and eosin for microscopic study. Immunohistochemistry was performed with the following panel of antibodies: vimentin (clone Vim 3B4; Dako, Glostrup, Denmark), CD34 (clone QBend/10; Dako), S100 protein (polyclonal; Dako), smooth muscle actin (clone 1A4; Sigma, St. Louis, MO, United States of America), epithelial membrane antigen (EMA; clone E29; Dako), glucose transporter 1 (GLUT-1; polyclonal; Abcam, Cambridge, United Kingdom), according to established protocols in our laboratory. Slides were developed with polymer-bound horseradish peroxidase (Envision+; Dako) and 3,3'-diaminobenzidine as chromogen.
Microscopic examination of the surgical specimen showed a plaque-like proliferation of spindle cells with wavy bipolar cytoplasm and mostly cigar-shaped nuclei, diffusely involving the entire breadth of the dermis (Figure A), and focally encroaching upon the subcutaneous fat (Figure B). Although there was neither significant nuclear atypia nor any diagnostically relevant mitotic activity, the tumor was conspicuous for including several aggregates of multinucleated giant cells with star-shaped cytoplasmatic projections corresponding to floret-like cells (Figure C, D). Furthermore, some areas of Meissner-like differentiation were seen (Figure 4D). Although an occasional peripheral nerve fascicle was felt to contain neurofibroma cells within the endoneurium (Figure E), no bona fide plexiform component was detected. The immunophenotype of the FMGCs included positivity for vimentin and CD34 (Figure A, B); whereas no staining was observed for S100 protein, SMA, EMA, and GLUT-1, respectively.


A six-month-old baby girl was admitted in the department of paediatric surgery of Hassan II university hospital in Morocco, with the complaints of abdominal distension and pain since one month. On examination her weight was 5.5 kgs and her abdomen was hugely distended. On palpation, a firm mass occupying all of the left lumb of the abdomen was palpable, which was tender, moving with respiration and the margins were irregular. Both the renal and liver functions as well as the findings of hematological studies were within the normal limits. A chest x-ray showed no abnormality. Abdominal ultrasonography demonstrated a pelvic left kidney measuring 18 cms in diameter with an important expansion of the excretory cavities and internal cystic and solid changes. Computed tomography showed a mass containing low-density areas of the left kidney. The right kidney was normal. The patient did not found any more tumors in the other organs. Because the most frequent tumor of the kidney occurring in newborns is congenital mesoblastic nephroma and patients with ectopic kidneys have a high risk for Wilms’ tumor, a diagnosis of either mesoblastic nephroma or Wilms’ tumor arising from ectopic kidney was tentatively made, and she was thus considered to be indicated for surgery.
Patient was operated upon; laparotomy was done through a median underumblical incision. A large partly cystic and partly solid mass with extensive areas of haemorrhage in the cystic areas was present in the left pelvic region. It was roughly spherical in shape. Postoperative recovery of the patient was uneventful and she was discharged on 10th postoperative day. The specimen was sent for histopathological examination.
Grossly a large mass was received measuring 18×12×8 cms in maximum dimensions. It weighed 200 Gms, linked to an ureter of 3×0,2 cms. On sectioning a multilocular circumscribed lesion was identified measuring 2,5 at 10 cms. It was attached to the cystic wall, which focally showed thickened brownish areas in the wall (Figure ). The entire kidney was replaced by the lesion, only a thin rim of renal parenchyma was identified at the periphery. Multiple sections were taken from the cyst wall and from the nodular lesion and vessels.
Microscopically small cystic spaces lined by keratinizing stratified squamous epithelium with skin adnexae were identified (Figure ). The solid areas showed large foci of cartilage, mucinous columnar epithelium (Figure ) and bone formation (Figure ). Melanin containing cells and neuroglial cells with occasional foci of immature neuroectodermal tissue were also identified (Figures and ). The cyst wall was thick fibromuscular without any lining. Sections from solid areas in the cyst wall revealed immature renal tissue. The diagnosis of immature teratoma was retained.


A 72-year-old female patient had been hospitalized for three days with pain in the right upper quadrant of the abdominal region. History reveals that the patient had experienced spontaneous pain, which increased after eating. The patient did not feel abdominal distension, nausea, vomiting and fever. Physical examination revealed body temperature (TPR) was 36.5 to 37.3C°; the heart rate (HR) of the patient was 78 beats/min and blood pressure (BP) was 120/90 mmHg. In addition, skin and tunica dura of the patient were not stained yellow; superficial lymphoid node was not swollen; abdomen was flat; GI form and peristaltic wave(−) subcutaneous varicose vein of abdominal wall(−) and abdominal muscle was soft. The patient felt light tenderness in the right upper quadrant without rebound tenderness and muscle tonus. Tumescent gallbladder could be palpable and was about 6×6 cm with Murphy sign(+). Liver and spleen were not touched below costal margin. Bowel sound (BS) was 2 to 3 min.
Blood counts and liver function were normal. Carbohydrate antigen (CA)19-9, carcinoembryonic antigen (CEA) and α-fetoprotein (AFP) were normal. Ultrasonography B (Ultra-B) showed increased volume of gallbladder, thickening of wall (0.6 cm) and a 6.4×4.9 cm of tumor (6.4×4.9 cm) could be viewed (Figure ). Plain computed tomography (CT) scan of the liver, gallbladder and pancreas showed increased volume of gallbladder (Figure ). Soft tissue tumor could be seen and manifestation intensified after enhancement (CT value 72 Hu). The bile duct of the left hepatic lobe was found to be slightly expanded. The size and shape were normal. The ratio of liver lobe and segment was normal. The density of liver parenchyma was well-distributed with specific mass (Figure ).
The liver of the patient showed chronic liver disease alteration but no metabasis nodus. The size of the gallbladder was 12×10×6 cm. The color was gray-red. The capsule wall of the gallbladder was thickened without being touched by the tumor on the plasma membrane. The tumor extended up to the visceral surface of the bottom of the gallbladder. The size was about 8×6 cm with hard texture and less movement. There were no metabasis nodus and swelling of lymphoid nodes on ligament hepatoduodenale, stomach, small intestine, pelvic cavity, abdominal membrane and root of mesentery. The surgical intervention included resection of the gallbladder, partial resection of the fifth segment of the liver and skeletonized dissection of the ligament hepatoduodenale.
A gray-red tumor 7 × 4.5 cm in size was removed from the gallbladder. Microscopic examination revealed that neoplastic cells were makr-fusiform shape, few short-fusiform shapes and similar-round shape. The karyotin of the cells was well-distributed, contained few nucleoli, and had light-red cytoplasm with slender cellosilk. The cells were arranged with a fasciculated and weaving appearance. The nucleo-heterology of the cells was quiet, but neoplastic cells were abundant and intensive. The nuclear fission could be found 1 per 2 to 3 high power field (Figure ). The lymphoid nodes of 12a (hepatic artery nearby), 12b (bile duct nearby), 12c (portal vein nearby), 8 group (hepatic common artery nearby), 13 group (after head of pancreas nearby) showed no neoplastic cells.
Vimentin (++), S-100 (+), CD34 (+), Neuron specific enolase (+), CK (−), CD68 (−), CD163 (−), CD31 (−), SMA (−), Masson dyed:blue (Figures , and )


During a 3-year period, a previously healthy 82-year-old male repeatedly consulted a general practitioner as well as a rheumatologist because of joint pains. The patient was eventually diagnosed with seronegative rheumatoid arthritis. During this 3-year period, the patient was treated with increased doses of PO prednisolone to 20 mg daily and, eventually, the addition of methotrexate gradually increased to 15 mg weekly. Two series of intra-articular injections of methylprednisolone were administered to several metacarpophalangeal joints of the right hand as well as the right shoulder.
The patient was later admitted to the emergency department at the University Hospital, Uppsala, Sweden, 1 week after the last injections, with complaints of fever, chills, myalgia as well as an open wound on the right hand but denied any respiratory symptoms. The wound had appeared approximately at the time of the latest methylprednisolone injections and was open and purulent, with swelling of the dorsal side of the right hand. The left elbow and the dorsal side of the left hand were also swollen. The patient was circulatory stabile and afebrile. Auscultation of the heart and lungs revealed no abnormalities. Plasma CRP-levels (301 mg/l) and total leucocytes (15 × 10(9)/l) were elevated.
Blood and tissue samples were taken for routine cultures before treatment with cefotaxime 1 g IV q8h was started. Surgical exploration and debridement of the wound was performed upon arrival, revealing damage to extensor tendons of the forearm/hand. The swollen left elbow was also punctured and clear fluid was extracted and these particular symptoms were therefore suspected to be due to a non-infectious process, e.g. reactive arthritis. Several surgical revisions were performed during the first 10 days, due to the emergence of novel abscesses on the hands and forearms.
All cultures acquired prior to antibiotic therapy resulted negative. Due to non-satisfactory effect 11 days after admission, treatment was replaced with a combined therapy of imipenem + cilastatin (500 mg/500 mg IV q8h) and clindamycin (600 mg IV q8h). Supplementary cultures were taken, as well as biopsies subsequently analyzed for non-tuberculous mycobacteria (NTM) (culture, microscopy, and PCR), fungus (microscopy), Nocardia and Actinomyces (cultures). Samples were taken from wound material for bacterial 16S-RNA PCR. TBC-specific IGRA (QuantiFERON, Cellestis) turned out negative.
All further wound cultures as well as direct microscopy for acid-fast rods (mycobacteria ssp.) resulted negative. The 16S-RNA PCR did though indicated the presence of L. bozemanii in wound material. No reports of L. bozemanii causing soft tissue infections were previously reported and, therefore, NTM was still considered a possible cause and the therapy was now altered to target mycobacteria. A combination of moxifloxacin (400 mg PO q24h), amikacin (500 mg IV q24h), and clarithromycin (500 mg IV q12h) was introduced. Additional tissue samples were taken from the right upper arm and again 16S-RNA PCR was positive for L. bozemanii, and a Legionella-specific PCR was performed which also detected L. bozemanii DNA. Serological analysis was undertaken and compared to blood samples acquired on admission, which revealed a fivefold increase of anti-body titers of L. bozemanii and L. longbeachae serogroup 1 and 2 during a 12-day period. Treatment was now continued with moxifloxacin and clarithromycin.
Due to inadequate improvement of the patient's general condition a chest X-ray was performed on day 16. This revealed widespread perihilar consolidations, interpreted as alveolar edema. During the next 2 weeks, repeated X-rays showed only minor improvement, and a CT scan was therefore performed, revealing bilateral alveolar consolidations as well as consolidations in the left lung. These abnormalities were interpreted by radiologists to be due to a previous infection. During this period, several novel abscesses appeared on both upper limbs, requiring additional surgical evacuation.
However, the patient eventually did show clear general improvement as well as reduction of skin lesions and could be discharged from the hospital, after approximately 3 months. Treatment with moxifloxacin and clarithromycin was continued for a further 2 months. Follow-up examination was performed after a further 4 months. No new abscesses had developed and the previous lesions had completely healed.


An 18-year-old female was referred to the otolaryngology department with a swelling on the left side of the neck since 4 months. On examination, nonpulsatile mass near angle of the left mandible was seen. Cranial nerve examination was normal. Doppler ultrasonography revealed hypoechoic mass in the left carotid space showing mild peripheral vascularity (). Computed tomography of the neck showed a heterogeneously enhancing hypodense mass in the left carotid space causing splaying of carotid bifurcation (Figures and ). On MRI examination, the mass was predominantly hypointense on T1- and hyperintense on T2-weighted images (Figures and ). Also MR angiography confirmed splaying of external and internal carotid vessels on the left side (). The patient underwent surgery. At surgery encapsulated mass was found at the carotid bifurcation extending posteriorly which was separated by blunt dissection from carotid vessels. Vagal and glossopharyngeal nerves were separately seen from the mass. At surgery, the tumor was easily peeled off from carotid and jugular vessels without significant bleeding. Histopathology confirmed diagnosis of cervical sympathetic chain schwannoma with distinct Antoni A areas (). Postoperatively patient developed Horner's syndrome on the left side which recovered 3 months after surgery.


A 17-year-old female came with history of painless swelling on the left side of the neck and change in voice since 2 months. On examination, there was nontender swelling noted posterior to the angle of the left mandible. USG neck revealed hypoechoic mass on the left parapharyngeal space showing mild vascularity on the Doppler study (). On CT scan, large heterogeneous predominantly isodense to hypodense mass was seen in the left parapharyngeal space showing mild heterogeneous contrast enhancement (). Mass was seen insinuating between internal and external carotid arteries with resultant splaying of internal and external carotid arteries (). Digital subtraction angiography revealed hypovascular mass causing splaying of internal and external carotid arteries (). The patient underwent surgery. Intraoperatively, tumour was seen arising from the left vagus nerve and was separating internal and external carotid arteries. On histopathology, mass was proven to be vagal schwannoma ().


A 62-year-old man presented at the outpatient clinic for device control after receiving an ICD shock. A single-chamber ICD (Current VR, St Jude Medical) had been implanted two years earlier for primary prevention while the patient was having symptoms of NYHA class III heart failure due to a dilated cardiomyopathy with left ventricular ejection fraction of 23%. Three tachycardia zones were programmed: ventricular fibrillation (VF) at a heart rate (HR) ≥200 bpm (300 ms), VT (VT-2) at HR between 171 and 200 bpm (350-300 ms), and a slow VT zone (VT-1) as a monitoring zone between 120 and 171 bpm (500-350 ms). Tachycardia was diagnosed once 12 recorded intervals were within any of the tachycardia zones. Supraventricular tachycardia (SVT) discriminators were programmed "ON" in the monitor zone using the nominal settings: morphology criterion (5 of 8 complexes have ≥ 60% morphology match with the sinus complex), sudden onset (programmed to 100 ms), and stability criterion (variability < 80 ms).
The patient's baseline rhythm was atrial fibrillation (AF). A VF episode was diagnosed once 12 consecutive intervals were detected in the VF zone (interval of 270-289 ms), and capacitors charging began. Despite spontaneous termination of tachycardia during charging, a shock of 36J was delivered. Upon interrogation both device and lead malfunction were ruled out. The tracing of the device interrogation of the episode is depicted in .
During charging of the capacitors, the device is programmed by default to terminate therapy after 5 intervals of sinus rhythm (SR), meaning 5 intervals below the programmed tachycardia zones (in this specific case below the monitor zone of HR 120-171 bpm). The device does this through the process of "binning": an interval is classified on the basis of its duration (in this patient, a cut-off value of 500 ms) and the average of the 3 preceding ones.
As can be seen in the figure, the first 5 intervals during charging are properly classified as VF (marked as F). Thereafter the tachycardia terminates and AF continues. The irregular and rather fast ventricular response with intervals in the monitor zone explain why only 4 beats could be binned as SR. Three intervals were probably misclassified as VF (marked as ∞) because they met both binning criteria, but the morphology was unchanged (a criterion the device does not use in this zone). Intervals >500 ms can be detected, but were not classified (marked as -) because they did not meet the second criterion for binning (the average of the preceding beats). In the absence of 5 intervals that could be classified as SR, at the end of charging a synchronized shock was delivered.


A 40-year-old woman with arrhythmogenic right ventricular dysplasia received a single-chamber ICD (Current VR, St Jude Medical) for secondary prevention. Three tachycardia zones were programmed: VF at HR ≥214 bpm (<280 ms), VT-2 with HR between 184 and 214 bpm (326-280 ms), and a VT-1 as a monitoring zone between 120 and 183 bpm (500-326 ms). Nominal SVT discriminators were programmed "ON" and SVT criteria timeout and VT therapy timeout were programmed "OFF". The patient presented at the outpatient clinic after receiving 6 ICD shocks. Device and lead dysfunction were ruled out. The tracing of the device interrogation of the episode is depicted in .
The episode started with a SVT at 155 bpm, which was appropriately discriminated for 127 seconds. However, HR suddenly increased, reaching the VF zone. After the detection of 12 VF intervals, a VF episode was declared. As in the first case, therapy would have been withheld if during charging 5 SR beats had been detected (R-R interval >500 ms). During charging, the HR slowed, reaching the VT-1 zone. Because the HR remained in the tachycardia zone no intervals could be classified as SR and the first inappropriate ICD shock was delivered. After the ICD shock, the nominal post-shock redetection criteria require 5 beats in SR (<120 bpm or >500 ms) to finalize the episode; 6 intervals classified as VF (marked as F) are needed to redetect the tachycardia as VF (in this case programmed to be in the VT-2 or VF zone, again meeting both binning criteria). The intervals within the monitor zone can neither be classified as SR nor as VF (marked as -). After the first shock a fast redetection of VF occurred because 6 of the 11 intervals were inside the VT-2 or VF zone. The capacitors were charged again and a second inappropriate shock was delivered. After the second shock the HR slowed but remained above 120 bpm (VT-1), thus prolonging the redetection period until the relevant criteria were satisfied. Unfortunately, due to the ongoing tachycardia, the episode was not ended and every time 6 VF intervals were classified, therapy was delivered. This patient received 6 consecutive shocks, until therapy was exhausted. The total duration of the episode was 355 seconds.


A 31-year-old male was referred to our gastroenterology department with a history of recurrent epigastric pain, nausea, and occasional vomiting since 6 months. Epigastric pain was characterized as intermittent, mild, and gnawing in character. The patient also noticed significant weight loss in 2 months. There was no history of fever and no past or family history of tuberculosis. The patient was nondiabetic. A chest X-ray, complete blood count, and liver and renal function tests were normal. ESR was 22 mm/hr. The patient had no known comorbidities and no history of hospitalization. Physical examination only revealed direct tenderness in the epigastrium. There was no lymphadenopathy. His HIV status was negative. Computed tomography scan of abdomen suggested thick antrum with dilated stomach, without any lymph node enlargement or ascites (). Upper GI endoscopy showed an irregular mass lesion at the antrum and pylorus region with narrowing of the lumen. (). The endoscope was passed beyond this point with difficulty. The impression then was gastric outlet obstruction with enlarged antrum with a possibility of antral malignancy. The patient underwent an endoscopic guided biopsy from the mass lesion. On histopathological examination, multiple epithelioid cell granulomas with Langhans-type giant cells and small foci of caseation necrosis were noted (Figures and ).
As the clinical diagnosis of malignancy was high, the patient was again subjected to 2nd biopsy from multiple sites including a biopsy from the fundus and the duodenum. Tissue for PCR and culture for Mycobacterium tuberculosis was also taken in view of an earlier report. Histopathology showed a similar finding, and PCR for Mycobacterium tuberculosis was positive. The biopsy from the fundus and the duodenum showed normal morphology. A diagnosis of hypertrophic primary antral tuberculosis was made. The patient was put on antituberculous treatment (ATT) regimen consisting of Isoniazid 5 mg/kg, Rifampicin 10 mg/kg, Ethambutol 15 mg/kg, and Pyrazinamide 25 mg/kg body weight for the initial 2 months followed by Isoniazid and Rifampicin in the same dose for another 7 months. While on treatment, he gained weight and became symptom free. The repeat of the upper GI endoscopy after 4 months of treatment was suggestive of deformed but negotiable antrum. After a followup of 6 months, the patient was symptom free.


An 80-year-old woman was initially admitted to hospital because of abnormal findings on chest radiography screening. She had a history of left lower lobectomy for primary lung adenocarcinoma (T1N0M0) and cerebral infarction six and three years ago, respectively. She had slight right hemiparesis, but was asymptomatic. Chest radiography and CT revealed a 20 mm nodule in right S2 without any hilar or mediastinal lymphadenopathy. Bronchoscopic examination revealed epidermal growth factor receptor wild-type adenocarcinoma cells from the nodule. Bronchoscopy-guided RFA was performed because of her poor pulmonary function and advanced age. The time courses of radiographic findings on chest CT before and after RFA are shown in . The radiographic findings changed to be a mass-like shadow after RFA and remained stable in size for 4 years. However, the mass increased in size and the bronchial lumen in the mass became narrow, which were considered to be indicators of progressive disease (60 months after initial RFA). Bronchoscopy-guided RFA was then repeated, and the mass shadow remained stable for 12 months ().


A 70-year-old woman, who had undergone radical hysterectomy for cervical cancer in our hospital, was referred to our respiratory center because of abnormal findings on chest CT. The abnormal findings were noted by systemic review before surgery. Bronchofiberscopy was performed, and a diagnosis of primary adenocarcinoma in the lung was made. There were no hilar or mediastinal lymph node metastases. She was treated with internal RFA as she refused thoracic surgery. Serial chest CT findings before and after RFA were shown in . Tumor shadow, showing ground-glass opacity (GGO) with solid components, was observed in left S3 and changed to a mass-like shadow after internal RFA. The mass remained stable for 40 months.


The patient was a 47-year-old male with non-contributory past medical history. He noticed weakness in the left lower extremity upon rising from bed in the morning, but the symptom gradually resolved. On the night of the same day, he suddenly developed headache and mild paralysis of the left lower extremity and presented to our hospital. On admission, the patient was alert and had mild paralysis of the left lower extremity. His blood pressure was 138/78 mm Hg. There were no abnormalities in the blood biochemistry tests taken upon arrival. The head CT and MRI showed cerebral infarctions in the right frontal lobe and subarachnoid hemorrhage in the frontal convexity and anterior interhemispheric fissure. In MRA, DAs were observed in the bilateral anterior cerebral arteries. In the 3D-CT angiography, dissecting intima was observed in the area with saccular enlargement (). The patient was diagnosed with a combination of cerebral infarction and subarachnoid hemorrhage caused by the rupture of the DAs in the bilateral anterior cerebral arteries. In the left internal carotid angiography, saccular cerebral aneurysm was observed in the left A1–A2 junction (). In addition, stenosis was seen in the left A2 segment and opaque vascular bulging was seen in its peripheral in the late arterial phase. Right internal carotid angiography showed cerebral aneurysm at the left A1–A2 junction (). The right A2 segment was occluded and its peripheral vessels showed retrograde flow during the venous phase. The treatment plan included surgery to prevent recurrent hemorrhage and to reinforce the walls of the DAs in the anterior cerebral arteries. Surgery was performed on the fifteenth day after admission when the patient’s neurological symptoms stabilized. First, anterior interhemispheric approach in a supine position was used to confirm the presence of aneurysms in the bilateral areas from A2 to A3 segments. The arterial wall was dark purple, and the vascular diameters were irregular with bulging in one area. Wrapping was performed from A2 to A3 segments for the left anterior cerebral artery. The right peripheral vessels had weak walls and vascular anastomosis was difficult. Therefore, trapping was performed at the A2 segment. In the postoperative course, the patient had no apparent neurological deterioration. One month after the surgery his symptoms had resolved completely, and he could return to work.


A 48 year-old male admitted to our hospital with the complaint of palpitation lasting for two months. One year before, LIMA-LAD and radial artery-right coronary anastomosis had been done with OPCAB surgery. The electrocardiograms and transthoracic echocardiography (TTE) which were done before the coronary artery bypass grafting surgery showed a sinus rhythm and no thrombus in cardiac chambers. During admission, the patient was still in sinus rhythm. He had been receiving aspirin for anticoagulation. On physical examination, the blood pressure was of 110/70 mmHg and the heart rate was regular at 75 beats/min. He had no symptoms or complications such as embolism or syncope. TTE and transesophageal echocardiography (TEE) showed a 3.0x2.8 cm free floating ball thrombus in the left atrium (LA) (). TTE showed normal left ventricular dimensions with a moderate reduction in left ventricular ejection fraction (%40) and hypokinesia of the septal and anterior walls. The left atrium was noted to be of normal dimensions.
There was no evidence of valvular disease. No coagulation disorder was detected and the hemostatic parameters were within normal ranges. Just prior to the operation, the patient underwent a coronary angiography and the grafts were detected to be patent. The patient subsequently underwent an urgent cardiac operation. The intervention was carried out through a median sternotomy. Cardiopulmonary bypass was established with femoral artery cannulation and bicaval venous drainage. The aorta was cross clamped without damaging the grefts. Cardiac arrest and myocardial protection were obtained with antegrade cold blood cardioplegia. The LA was opened longitudinally and the thrombus was removed. The thrombus was round, smooth, 4 cm in diameter ().
There was no attachment of the thrombus to the atrial wall and no mural thrombus. The mitral valve was within the limits of normality. No stenotic aspect was disclosed, and valvular competence was normal. The left atrium was closed and the cardiopulmonary bypass was discontinued. Postoperatively, he received anticoagulation with warfarin. The postoperative course was uneventful and he was discharged from the hospital on the 7th postoperative day.


A 59-year-old female was admitted to Jinan Military General Hospital (Jinan, China) on March 3, 2010 after experiencing sternocostal, back and bilateral groin pain, and progressive lower-extremity weakness for more than 2 years. The cause of the pain was not evident initially. The patient described the pain as being intermittent and dull. Gradually, both lower extremities became weaker, weakness and pain were aggravated following exercise and labor and relieved by rest, and the condition worsened. However, the patient did not present morning stiffness, low fever or night sweats, and the extent of pain was not affected by weather changes. At 1 year prior to admission, magnetic resonance imaging (MRI) of the lumbar vertebrae had been performed on the patient, which revealed L3/4 and L4/5 disk herniation. MRI of the thoracic vertebra showed ligamentum flavum hypertrophy and calcification at the T6/7 vertebral level, resulting in spinal cord compression. A conservative treatment regimen was initiated, but there was no improvement in the severity of the symptoms. The patient had increasing difficulty rolling over and getting up, and upon hospitalization, the patient was not able to walk independently. Thus, the patient was admitted to the Department of Osteopathy, Jinan Military General Hospital for thoracic spinal stenosis.
Osteopathic surgeons considered that the cause of the clinical manifestations was unlikely to be thoracic spinal stenosis and tests on anti-neuron antibodies [anti-Hu, 70.080 ng/ml (normal, <76.8 ng/ml); anti-Ri, 31.7412 ng/ml (normal, <26.5 ng/ml) and anti-Yo, 16.598 ng/ml (normal, <18.0 ng/ml)] showed that the concentrations of anti-Hu and anti-Ri were significantly increased above normal levels. Therefore, the patient was diagnosed with suspected paraneoplastic syndrome and was transferred to the Department of Neurology, Jinan Military General Hospital. Following admission, heart, lung and abdominal examinations showed no significant abnormities. Neurological examinations showed that the patient had full consciousness and fluent speech, cranial nerve examination was normal, double upper-limb myodynamia classified as Grade V, double lower-limb distal myodynamia classified as Grade III- and proximal myodynamia classified at Grade V-. The muscle tone of all four limbs was normal, the double upper-limb tendon reflex was symmetrical and normal, the double lower-limb tendon reflex was symmetrical and reduced, and the finger-to-nose test was performed stably and accurately. However, the heel-knee-tibia test on the two lower limbs could not be completed. Sensation examination showed no significant abnormities, the test for pathological reflexes was negative, the neck was soft and flexible, and meningeal irritation signs were negative. The pressing pain at the sternum, multiple ribs, bilateral hip joints and groin was significant. Since the onset of the illness, drinking, eating, urination and defecation were normal, and the body weight had not reduced significantly. Past medical history and family history revealed nothing of note. The results of blood examinations were normal (red blood cells, 3.31×1012/l; hemoglobin, 95 g/l; and platelets, 568×109/l). The alkaline phosphatase level was elevated [339 U/l (normal, 26–150 U/l)], the blood phosphorus level was reduced [0.64 mmol/l (normal, 0.80–1.60 mmol/l)] and the calcium [2.22 mmol/l (normal, 2.10–2.60 mmol/l)] and magnesium concentrations were normal. The levels of tumor-marker antibodies [sialic acid: 82.1 mg/dl (normal, 45.6–75.4 mg/dl), carcinoembryonic antigen (CEA), α-fetoprotein (AFP), carbohydrate antigen 72-4 (CA72-4), carbohydrate antigen 12-5 (CA12-5), carbohydrate antigen 19-9 (CA19-9), carbohydrate antigen 15-3 (CA15-3), cytokeratin 19 fragment (CY21-1) and neuron-specific enolase (NSE)] were within the normal range, as were the erythrocyte sedimentation rate (31 mm/h), serum protein electrophoresis, blood parathormone level [21.55 ng/l (normal, 15–65 ng/l)] and albumin levels. Results from the 5-item thyroid function test [thyroid stimulating hormone, (TSH); free triiodothyronine (FT3); free thyroxine (FT4); thyroid peroxidase antibodies (TPO); and thyroglobulin antibody (TGAb)], 6-item sex hormone test (estradiol, progesterone, testosterone, prolactin, luteinizing hormone and follicle stimulating hormone), 5-item hepatitis B and C virus tests and HIV antibody test did not show any anomalies, and the Bence Jones urinary protein test was negative. Bone marrow puncture, gynecological ultrasonography, abdominal ultrasonography, echocardiography and double hip-joint computed tomography (CT) scan showed no obvious abnormalities. Electromyography showed no characteristic changes and the repetition-frequency stimulation test showed no significant alternation. Pulmonary CT demonstrated an increase in double lung markings, the presence of pleural thickening and calcification in the right liver lobe. Thyroid color ultrasonography revealed multiple thyroid nodules, localized neoplasia, locally coexisting Hashimoto's disease, the nature of left nodule was undetermined, parathyroid lesions could not be excluded and cervical lymph nodes were swollen. No significant abnormal nuclide intake was demonstrated following bilateral parathyroid ECT examination () and clinical tests repeatedly showed that the parathormone and 24-h urine phosphorus and urine calcium levels were normal. Therefore, a diagnosis of a parathyroid disease was not considered.
Whole-body bone emission computed tomography (ECT; via the intravenous administration of 99 mTc - MDP) showed that nuclide intake was increased in several locations (), and the cause was considered to be malignant. Whole-body positron-emission tomography CT (PET-CT) examination () showed that several ribs and double femoral neck (incompletely) had discontinuity and numerous old fracture lines (loose lines) were present, as well as mild T12 compression changes. Local fluorodeoxyglucose (FDG) metabolism increased in the cervical vertebra-2 accessory bone, but did not increase in the regions of reduced local density of the left thyroid gland, which were considered to be thyroid nodules. Bilateral hip joint swelling was accompanied by an increase in FDG metabolism and these regions were classified as inflammatory hip lesions. Bone mineral density tests showed that left forearm bone density had reduced by 17.96% [T-score: −1.43 (−1>T-score >−2.5 is suggestive of bone loss)]. The results of blood phosphorus tests (0.48–0.64 mmol/l) led to the clinical consideration that the patient may be diagnosed with hypophosphatemic osteomalacia. However, re-examination of the patient following the administration of sodium glycerophosphate and Rocaltrol® (Roche, Shanghai, China) demonstrated that the treatment was ineffective (the blood phosphorus concentration did not increase). Tests of blood from the right subclavian vein indicated that the concentration of fibroblast growth factor (FGF)-23 was 32.44 ng/l (normal, 40–90 ng/l).
To ensure a definitive diagnosis, a tibialis anterior muscle biopsy was conducted on March 27, 2010 (). Single or small angular atrophic muscle fibers were apparent but no necrotic muscle fibers or inflammatory cell infiltration were observed (). The proportion of the two types of muscle fibers was approximately normal, although there was some clustering of the muscle fiber types and the atrophic muscle mainly comprises type I fibers (). Gomori staining () and Oil Red O staining () showed no abnormalities. Electron microscopy results () showed fractured or missing muscle fibers and the presence of lipid grains between the fractured muscle fibers. The muscle fibers had a sparse transverse arrangement and relatively few mitochondria. In addition, an accumulation of glycogenosomes was observed beneath the muscle cell membrane.
On April 16, 2010, a thyroid nodulectomy was performed to exclude TIO as the cause of the symptoms. The postoperative pathological results showed nodular goiter in the left lobe. Partial follicular lesions had adenomatous hyperplasia, with abundant cells and active growth. The results of immunohistochemical staining () were positive for thyroid transcription factor-1 (TTF-1), CK8/18, FGF-7 and thyroglobulin (TG), and negative for calcitonin (CT). At 1 week after the surgery, although the sternal pain had been alleviated, the patient continued to experience significant pain in the bilateral rib arch, back and groin, and was unable to roll over, get up or walk. A re-examination was performed on May 20, 2010 and the whole-body bone imaging ECT results showed no significant changes. The results of a postoperative re-examination were as follows; anti-Hu, anti-Ri and anti-Yo antibody levels were normal, the erythrocyte sedimentation rate was 25 mm/h, post-operative continuous phosphorus supplementation was ineffective (phosphorus: 0.48–0.56 mmol/l), calcium and magnesium levels were normal, and the 24-h urine calcium and urine phosphorus concentrations were normal. Following thyroid tumor resection, the condition of the patient did not improve significantly, therefore, a diagnosis of TIO was not considered. The vitamin 1,25-(OH)2D3 concentration was significantly lower than normal [<4.00 ng/ml (normal concentration: ≥30.00 ng/ml)], which was considered to be caused by hypophosphatemic vitamin D-resistant osteomalacia which was subsequently treated with neutral phosphate preparations (73.1 g disodium hydrogen phosphate and 6.4 g potassium dihydrogen phosphate in an aqueous solution of final volume 1,000 ml). Every 4 h, 20 ml of the preparation was administered orally (5 times daily), in addition to a 1-ml vitamin D3 intramuscular injection once every 2 weeks (or oral vitamin D3 tablets and calcium). The condition of the patient improved gradually, and after 2 months, the sternocostal, back and groin pain was relieved significantly. The phosphorus concentration at the 6-month follow-up visit was 0.79 mmol/l, and had increased to 0.98 mmol/l at the 1-year follow-up. The whole-body pain had ceased and the patient was able to roll over, get up alone and walk slowly with assistance. When walking, the patient continued to experience weakness in both legs, but to a lesser extent than previously.


A 36-year-old Latin American woman, G3 P3, had initially presented to the emergency department with a two-week history of bilateral lower quadrant pain at an outside hospital from which she was referred to out institution after initial testing. The pain had started 2 weeks ago coinciding with mild vaginal bleeding. She denied any other symptomatology. She had undergone a cesarean section for fetal distress during her third pregnancy (G3) which was followed by cesarean hysterectomy due to uncontrollable bleeding. A supracervical hysterectomy was performed at that time which was approximately 6 years ago. Physical examination revealed normal vital signs. Her abdomen was soft and mildly tender to palpation. Recent laboratory studies from an outside lab showed a beta-HCG level of 10,587 mIU/mL (normal: 0–4). Initially, an ultrasound was obtained which was followed by an MRI examination for further characterization.
On transabdominal ultrasound, uterus was absent. A 10 cm heterogeneous mass was present in the midline pelvis without any significant Doppler flow. Additionally, there was a 5 cm complex heterogeneous left adnexal mass without any Doppler flow. Transvaginal ultrasound also demonstrated the large heterogeneous mass measuring 10 cm × 6.5 cm × 8.4 cm in the central pelvis with no color flow on Doppler examination later confirmed with MRI (Figures and ). It was thought to represent an organized hematoma and less likely a neoplasm. Additionally, the left adnexal region demonstrated a 5.2 cm × 3.8 cm × 4.1 cm heterogeneous mass containing an anechoic, well-circumscribed, rounded, cystic structure measuring 2.3 cm × 2.5 cm × 2.8 cm (). The anechoic cystic structure showed slightly thick and echogenic walls with an encircling vascularity on color Doppler exam (an appearance frequently termed as “ring of fire” appearance) (). This was reported to either represent a corpus luteum cyst of the left ovary or an ectopic gestational sac. A normal ovary was not clearly visualized either by transabdominal or transvaginal approach on either sides.
MRI depicted postsurgical changes from supracervical hysterectomy with a nonenhancing T1 hyperintense and T2 hypointense mass in the midpelvis measuring approximately 11 cm × 10 cm compatible with blood products (Figures and ). Additionally, there was a left pelvic mass which showed heterogeneous enhancement and was considered to represent a tubo-ovarian mass complex (Figures , and ). There was a rounded thick-walled cystic structure along the anterior aspect which showed wall enhancement ().
Patient was taken to the operating room for laparoscopic surgery. Hemoperitoneum was evacuated, and omental adhesions to the anterior abdominal wall were dissected free. Multiple adhesions were noted in the left pelvis, with enlarged left fallopian tube and ovary surrounded by clots. The right adnexa was not visualized and appeared to be surgically absent. The left adnexa was adhered to the vaginal cuff. The adhesions were dissected, the pedicle was excised, and the specimen of the tubo-ovarian complex was obtained and sent to pathology. Patient had an uncomplicated postoperative course. She was discharged from the hospital on postoperative day 1 with appropriate instructions. At discharge, her beta-HCG was noted to have decreased to 4401 mIU/mL from 10,587 mIU/mL.
On pathologic examination of surgical specimens, midpelvic tissue sample was identified to be fat and organized blood. Left ovary showed chorionic villi and blood. The diagnosis was made of a tubo-ovarian complex with ectopic pregnancy within the left ovary.
On post-operative follow-up visit one month later, a repeat measurement of Quantitative beta-HCG showed a normal level of <3.0 mIU/mL (normal: 0–4).


A 59-year-old woman [height 154 cm; weight 146 kg (ideal: 52 kg)] with a giant ovarian tumor was scheduled for tumor resection. She had no significant medical history. Her preoperative abdominal circumference was 194 cm, and she could not assume a supine position. She was assessed as grade 5 according to the Hugh-Jones classification for assessment of breathlessness. Transcutaneous oxygen saturation on room air was 94–96% in a semirecumbent position. Preoperative pulmonary function tests indicated restrictive impairment [vital capacity (VC): 1910 ml (%VC: 77.3%); forced expiratory volume in 1 second (FEV1.0): 1440 ml (%FEV1.0: 75.4%)]. Tumor markers were within normal ranges. Chest X-ray revealed elevation of the bilateral diaphragm, and thoracoabdominal computed tomography revealed a right giant cystic ovarian tumor with an estimated mass of 100 kg. Evidence of malignant tumor, atelectasis, and pleural effusion was not observed (Figure ).
To facilitate precise hemodynamic control, standard American Society of Anesthesiologists monitors were applied and a right radial arterial line was inserted to measure arterial blood pressure; cardiac output (CO) and stroke volume variation (SVV) were measured with a FloTrac™/Vigileo™ system (Edwards Lifesciences, Tokyo, Japan). For intubation under conscious sedation, we sprayed topical 4% lidocaine from the posterior pharynx to the glottis while oxygenating with a mask at 10 l/min over 10 min. After the administration of 1 mg midazolam and 50 μg fentanyl, the trachea was intubated using the Airway Scope® (Hoya, Tokyo, Japan) in a semirecumbent position. Anaesthesia was then induced with 80 mg propofol, and maintained with 1.5% sevoflurane and 0.2 mg/h remifentanil (0.06 μg·kg-1·min-1 for 60 kg body weight). Spontaneous respiration was maintained and pressure support ventilation consisted of positive end-expiratory pressure (PEEP) set at 5 cmH2O with pressure support set at 17 cmH2O above PEEP. A catheter was placed in the right internal jugular vein under ultrasound guidance to monitor central venous pressure (CVP) and to administer inotropic agents.
Surgery commenced in the semirecumbent position to prevent supine hypotensive syndrome and respiratory failure. The incision site was infiltrated with 20 ml of 0.75% ropivacaine, a double-balloon suction catheter (SAND®, Hakko, Nagano, Japan) was inserted into the tumor, and the tumor fluid was extracted at a rate of 500 ml/min. We administered 12–24 mg/h dopamine with a transfusion because arterial blood pressure gradually decreased during fluid extraction. Draining the tumor fluid improved respiratory compliance; therefore, we decreased the pressure support to 11 cmH2O. Aspiration of the tumor fluid took 160 min with a total volume of 83 l. The patient was then moved into a supine position. Residual tumor resection and bilateral salpingo-oophorectomy were performed. Spontaneous respiration ceased when we increased the dose of remifentanil to 0.6 mg/h; therefore, we switched from pressure-supported ventilation to pressure-cycled ventilation. The total operation time was 667 min and the anesthesia time was 811 min. Postoperative analgesia included a continuous infusion of 40 μg/h fentanyl.
The patient was extubated on postoperative day 1 and her respiratory status was good thereafter. Her weight decreased to 50 kg postoperatively. The final pathology diagnosis of the extracted tumor was right benign ovarian cyst.


A-52-year old woman presented with 3-year history of fullness and dull pain involving the left knee. She had been aware of a mass in the knee joint that had been progressively increasing in size. She was unable to flex her knee, sit cross-legged, or squat. There was no history of trauma or constitutional symptoms.
On physical examination, there were fullness on the medial aspect of left knee and palpable nodular mass of approximately 6 × 4 cm over the infrapatellar region medial to the patellar tendon (). The mass was nontender with no local warmth, bony hard in consistency, and immobile. The mass was more prominent on flexion than on extension of knee. The range of motion of the knee joint was limited to 100° of active flexion. Neurovascular examination was normal. Plain radiographs of the knee revealed a mineralized mass situated between the anterior tibial tubercle and the patella. The mass appeared to occupy the entire region of the infrapatellar fat pad. The femoral condyles and tibial plateau were normal (Figures and ). Subsequently an MRI was performed. MRI revealed a large mineralized mass lying in the Hoffa body with no attachment to the skeletal tissue (). It was decided to perform an excision biopsy.
Under regional anaesthesia with the patient supine in a bloodless field furnished by a pneumatic tourniquet, a 7 cm longitudinal incision was made medial to the patellar tendon. The mass was lying medially and behind the patellar tendon in Hoffa's fat pushing the tendon laterally () and impinging on medial femoral condyle and intercondylar notch (). It was dissected and excised in toto (Figures and ). The medial tibial plateau, patella, menisci, and the patellar tendon were not involved in the process. The mass did not involve the joint space or synovium and was not continuous with femur, tibia, or patella but rather originated from joint capsule and was completely intra-articular. The capsule and the subcutaneous tissue were sutured with 1/0 vicryl and skin with 2/0 ethilon. 12 G drain was kept. Compression dressing was applied. Weight bearing was allowed in the immediate postoperative period.
Postoperative recovery was uneventful. Sutures were removed on the 12th postoperative period. The patient returned to her daily activities regaining full flexion within two weeks after surgery. She was on regular followup. When last reviewed 24 months after excision, she was asymptomatic with no clinical and radiographic signs of recurrence of the lesion (Figures , (1), and (2)).
Grossly, the resected specimen measured 7 × 6 × 5 cm with a large cartilaginous cap and was surrounded by adipose tissue and a fibrous capsule. Histologically, the cut sections showed numerous lamellar bony trabeculae with mature cartilaginous cap. Fibrofatty tissue was seen at the periphery (). There were foci of active endochondral ossification without any evidence of malignancy. No mitotic figures were seen.


A 55-year-old man was referred to our hospital due to intolerant severe short of breathing and persistent cough for two hours. During the 2 years preceding presentation he suffered from less severe similar attacks, which were treated in a local clinic by bronchodilators inhalations with improvement. His past medical history revealed ischemic heart diseases and chronic obstructive airway disease. He used to smoke 30 cigarettes daily for the past 25 years. On physical examination, he had normal temperature of body 37°C, dyspnoea up to 40 breaths per minute, and, on auscultation to his lungs, perception of wheezing on the left hemithorax and normal sounds of breathing on the right one. He denied any aspiration of foreign body. Pulmonary function test () revealed FEV1—53%. Axial slice of chest computed tomography showed intrabronchial 0, 5 cm mass “polyp” in the left main bronchus ( white arrow). This mass was endoscopically resected by laser without complications and histology revealed benign squamous papilloma. After the resection of the polyp, the patient did well as the symptoms regressed abruptly. Repeated pulmonary function test after resection of the polyp resulted in improvement of FEV1, which skyrocketed up to—76% (). Because of technical reasons, HPV DNA was not tested. After one year of follow-up, the patient remains asymptomatic. Axial CT of chest () “lung window” showed that the bronchus still free of masses.


A 17-year-old female presented with a three-month history of left hip pain. Plain radiography revealed an osteolytic lesion with an osteosclerotic change to the femoral neck (). A periosteal reaction was not observed. Computed tomography (CT) revealed an osteolytic lesion with an osteosclerotic change and a pathological fracture of the femoral neck (). T2-weighted coronal magnetic resonance imaging (MRI) showed a heterogeneous bone tumor arising from the femoral neck and extending to the trochanteric site, which was hypointense compared with the bone (). Hyperintensity was observed in the capsule of the hip joint due to hemorrhage (). Chest radiography and CT revealed no evidence of lung metastasis. The initial differential diagnosis was of a benign bone tumor, including a solitary bone tumor, an aneurismal bone cyst or fibrous dysplasia. An open biopsy was performed. The results from the examination of the intraoperative frozen section were consistent with a fibrous benign tumor. Due to the presence of the pathological fracture, tumor curettage, hydroxyapatite (HA) granule (Apaceram®; Hoya Co., Tokyo, Japan) transplantation and internal fixation using a compression hip screw (CHS) system (Omega Plus T1; Stryker Japan Co., Tokyo, Japan) were performed to prevent displacement and avascular necrosis of the femoral neck (). However, a histopathological study of the resected specimen revealed that the tissue contained atypical tumor cells and exhibited a formation of osteoid or immature bone matrix (). Immunohistochemically, the majority of the tumor cells were strongly positive for vimentin and alkaline phosphatase (ALP). Based on these data, a histopathological diagnosis of conventional osteoblastic OS was made.
The patient was administered multiagent chemotherapy, including high-dose methotrexate (HD-MTX; 10 g/m2 day 1), cisplatin (CDDP; 120 mg/m2 day 1) and adriamycin (ADM; 30 mg/m2 day 2), according to the neoadjuvant chemotherapy for osteosarcoma (NECO) 95-J protocol (). When the neoadjuvant chemotherapy was completed, the response to chemotherapy was evaluated using MRI, CT and plain radiography and a partial response was observed.
The patient subsequently underwent a wide resection and reconstruction using a pasteurized autograft-prosthesis composite, as described later in this study (). The histopathological response to chemotherapy was grade IV according to the criteria of Rosen et al(). The patient was administered adjuvant chemotherapy, as above. No recurrence or metastases were identified during a follow-up period of three years. Radiography revealed a partial union of the medial junction of the distal femur after 1 year (). The functional score, according to the study by Enneking et al(), was 92%. The patient showed a slight Trendelenburg gait due to disorder of the gluteus medius muscle.
The patient was placed in a lateral position. A long posterolateral incision was made to contain the biopsy scar and all the potentially contaminated areas that were included in the scars from the drains. Contaminated areas, including the subcutaneous tissue, iliotibial band and vastus lateralis muscle, were resected and an adequate wide resection of the bone was performed. The distal femur was resected 5 cm away from the tip of the CHS plate. The pelvis was resected according to the rotational acetabular osteotomy (RAO) technique described previously (). Briefly, the osteotomy was performed using a specially curved osteotome that was designed to approximately correspond to the circumferential curvature of the acetabulum. The line of the osteotomy followed the proximal side of attachment of the capsule to the pelvis. The osteotomy was carried out anteriorly and posteriorly around the circumference of the acetabulum, with the osteotome being allowed to follow its own curve. En bloc resection of the hip joint was performed without breaking the capsule (). Pasteurization of the resected tissue was then performed (). Briefly, the bone was heated in physiological saline at 65°C for 30 min following curettage of the tumor tissue and intramedullary reaming. The capsule of the hip joint was removed from the bone. The acetabular cartilage was removed from the pasteurized bone using curets and careful reaming, with an attempt to leave the subchondral bone of the acetabulum intact. The pasteurized acetabulum was fixed to the ilium, ischium and pubic bone using a SuperFIXSORB screw (Takiron Co., Osaka, Japan). For further reinforcement of the acetabulum, a Kerboull-type (KT) plate (Japan Medical Materials Corp., Osaka, Japan) was installed. Following the fixation of the graft to the host bone, the pasteurized acetabulum was resurfaced with a polyethylene cup that was cemented in place. The distal side of the pasteurized femur was fixed with wire. Conventional cemented total hip arthroplasty (THA; Stryker Japan, Co.) was performed using an Omnifit Cemented Long Stem. To prevent infection, the bone cement was mixed with antibiotics. Every 40 g of cement contained 1 g vancomycin hydrochloride and 400 mg amikacin sulfate.


A 19-year-old female patient presented with a large right-sided adrenal primary tumor with bone and bone marrow metastases. At diagnosis, a pre-chemotherapy bone marrow biopsy was obtained and had >90% tumor infiltration (labeled as Met1). The patient was treated with the standard induction chemotherapy as per the Children’s Oncology Group (COG) high-risk neuroblastoma protocol A3973. After four cycles of induction chemotherapy, there was an improvement in clinical symptoms, but with a mixed radiographic response. At this point the patient underwent a surgical procedure to remove the primary tumor. The bulk primary tumor was macro dissected into 4 different sections for further genomic studies; and they all contained ≥65% viable tumor cells (labeled as PT for Primary Tumor). Despite subsequent multiple rounds of salvage therapy, the patient continued to have progressive disease and developed extensive metastases to lung, bone, brain, and liver. Eventually, the patient succumbed to the disease 3.5 years after the initial diagnosis. A metastatic tumor from liver was taken for genomic analysis (>90% tumor cells; labeled as Met2), while normal skin and unaffected liver were taken as germ line samples.


A 26-year-old Saudi Arabian male, with a 9-month diagnosis of BD, presented on October 2010 with sudden onset of headache and generalized tonic clonic seizure. His initial computed tomography (CT) scan of the brain report revealed a significant SAH in the right sylvian fissure. He was then transferred to our institution for definitive management.
General physical examination revealed normal vital signs, with mild neck rigidity. He had a mild sclera injection, and there were with no skin or mucosal lesions. The neurological examination revealed an alert, oriented patient without focal neurological deficit. Routine blood investigations, including complete blood count, electrolytes, and renal and coagulation profiles, were within normal values. A subsequent brain CT scan, on the 5th SAH day, was negative for SAH but 3D-CT angiogram revealed one large distal fusiform and another 2 smaller aneurysms: one at the right MCA and another at the right PCA (Figures and ). A conventional four-vessel cerebral angiogram (DSA) revealed an ectatic fusiform dilatation of insular M3-segment of the right MCA with 4 mm proximal (M2-segment) aneurysm and 8 mm distal (M3-segment) fusiform aneurysms (Figures and ). The left carotid and posterior circulations were normal, apart from a 3 mm proximal PCA aneurysm.
The patient was deemed ineligible for conventional therapy due to the small size of the parent artery which makes its preservation and protection not possible and carries a higher risk of rerupture and bleeding. The procedure was discussed with the patient and his family and an informed consent was obtained. Endovascular embolization using Onyx within the ruptured aneurysm was considered as a viable treatment option.
Under general anesthesia, a cerebral angiogram was performed through a right femoral artery puncture. A size 6 French guiding catheter (Guider Soft-tip, Boston Scientific) was placed in the right internal carotid artery. A Marathon microcatheter (1.5F/1.7F eV3 Neurovascular) was advanced over a silver speed 10 microwire and placed in the ruptured aneurysm against the wall. Onyx 18 was injected slowly into the aneurysm with slow lamination of the precipitated amount within the aneurysm (). Control of Onyx 18, to avoid any penetration of the normal distal artery, was assured to allow retrograde filling of the distal normal artery via collaterals. Complete occlusion of the ruptured aneurysm was successful and filling with Onyx 18 achieved. This also resulted in cessation of flow within the fusiform dilated parent artery and the associated proximal aneurysm. Immediate retrograde filling of normal branch distal to the aneurysm was observed on the late arterial phase. A postprocedural cerebral angiogram confirmed the complete obliteration of the diseased segment in the right MCA, including the ruptured fusiform aneurysm, and the smaller aneurysm was left for medical therapy.
Postprocedure period was uneventful; the patient was extubated and had no neurological deficits. There was no evidence of postendovascular complications on follow-up magnetic resonance imaging (MRI) and MR angiogram (MRA) scans in the next day. An area of low intensity at the site of embolic material has been revealed with resolution of the ruptured MCA aneurysm (). Furthermore, there was no evidence of any ischemic insult (). The patient was started on a 6-month course of prednisolone (1 mg/kg/day), azathioprine (150 mg/day), and colchicine (1 mg/day) for treatment of the remaining small aneurysm.
At 6 months of follow-up, DSA showed persistent occlusion of the right MCA fusiform aneurysm with 40% decrease in the size of the proximal MCA aneurysm, and resolution of proximal PCA aneurysm (Figures and ).


A 57-year-old man was admitted to the hospital with complaints of gross hematuria and general weakness. Examination revealed a left kidney tumor, 150 mm × 120 mm in size, extending up to the intrapericardial segment of the inferior vena cava (). Distant and regional metastases were not found. Ultrasonography demonstrated thrombus apex flotation, which suggested the absence of adhesions of intraluminal tumor to the vena cava endothelium.
The patient underwent laparotomy through “chevron” incision. The duodenum was mobilized using Kocher's method. The inferior vena cava was exposed in the subrenal and subhepatic segment. The left renal artery was ligated. Falciform, both triangular and coronary, ligaments of the liver were transected. Suprahepatic infradiaphragmatic part of the inferior vena cava with the terminal portions of the major hepatic veins was exposed. The right lobe of the liver was rotated medially. A great number of dorsal hepatic veins were found to drain into the area of retrohepatic inferior vena cava front wall. Hepatic tissue at this level surrounds no more than half the circumference of the IVC. After careful exposure of major hepatic vein mouths, directly below them between the front surface of the IVC and the rear surface of the liver, a tunnel about 1.0 cm wide was formed bluntly and sharply ().
After T-shaped dissection of the diaphragm in the area of its caval opening, the intrapericardial inferior vena cava was exposed without opening the pericardium. The thrombus was milked manually below the mouths of the major hepatic veins. Through the preformed tunnel under the mouths of the major hepatic veins, a vascular clamp was passed and placed above the top of the thrombus (). Isolation of the intraluminal portion of the tumor was finished by clamping the right renal vein and IVC below the thrombus. A slight decrease of blood pressure (to 100/60 mm Hg) was noted. The IVC was dissected longitudinally in its subhepatic segment. The tumor thrombus was removed along with the left kidney. The IVC was covered with twisted 4/0 prolene suture. The wound was drained and stitched. The postoperative period was uneventful. The patient was discharged from the hospital 10 days after the surgery.


A 64-year-old male presented to his local physician with drenching night sweats, sixteen-pound unintentional weight loss, and cough productive of dark green sputum for two months. Pertinent negatives included the absence of fever, hemoptysis or dyspnea. Initial chest X-ray (CXR) () demonstrated a right upper lobe cavitary lesion. A 10-day course of ciprofloxacin was provided for a provisional diagnosis of pneumonia without improvement of symptoms. CXR demonstrated progressive cavitation leading to a computed tomography (CT) of the chest () which revealed an 8.6 × 5.9 cm cavity with thick, irregular borders in the right upper lobe with tree-in-bud opacities scattered through the remaining right lung. A sputum sample was negative for M. tuberculosis by RNA amplification analysis, and Quantiferon-Gold test was negative. Sputum samples ultimately grew Mycobacterium avium intracellulare (MAI) prompting treatment with azithromycin, ethambutol, and rifampin which was started almost five months after the onset of symptoms. He presented to our clinic for a second opinion. Further history was notable for current tobacco abuse of 40 pack-year duration and no obvious risk factors for HIV infection. He reported that one of his coworkers was recently placed on isolation for presumed tuberculosis, but this diagnosis was subsequently excluded. He denied any other exposure to tuberculosis or recent travel. Upon presentation, the patient looked well, without respiratory distress, and was afebrile. Physical examination was unremarkable.
Pertinent laboratory testing revealed normal complete blood count, renal and hepatic panel, negative HIV serology, and normal CD4 helper cells and immunoglobulin levels. CD8 lymphocytes were slightly decreased to 169/μL (normal values were between 180 and 1170/μL).
A bronchoscopy with bronchoalveolar lavage (BAL) was performed because his clinical course was atypical for MAI infection, and he had not responded to therapy. Scopulariopsis spp. and acid fast bacilli were present on direct examination of smears, but RNA amplification was negative for M. tuberculosis. No MAI was isolated.
Based on the initial BAL results, voriconazole, was added and azithromycin was replaced by clarithromycin. Repeated chest CT scans showed disease progression with new involvement of the left lung.
Ultimately, Mycobacterium interjectum was identified in the BAL cultures through DNA sequencing 2 months after presentation to our clinic, and levofloxacin was added to his antibiotic regimen. Susceptibility testing showed sensitivity to rifampin (MIC < 0.5 mcg/mL), rifabutin (MIC < 0.12 mcg/mL), ethambutol (MIC = 4 mcg/mL), clarithromycin (MIC < 4 mcg/mL), streptomycin, and clofazimine and resistance to ciprofloxacin. Rifampin and ethambutol demonstrated synergy. Further susceptibility testing differed in that the isolate was resistant to ethambutol and sensitive to Trimethoprim/sulfamethoxazole (TMP/SMX) and amikacin. Thus, treatment was changed to rifampin, intravenous amikacin, TMP/SMX, and voriconazole. Ethambutol was discontinued but subsequently restarted by his local physician in view of the discrepant results in susceptibility testing between the 2 laboratories. Voriconazole was later discontinued due to its interaction with rifampin and the impression that Scopulariopsis most likely represented a saprophyte or contaminant. Amikacin was discontinued after 2 months in face of radiologic and clinical improvement.
Following antimicrobial therapy, night sweats and weight loss resolved, and cough and sputum production markedly decreased. Chest CT scan performed after six months of current therapy showed stability of the right apical cavity and substantial improvements in the remaining parenchymal disease. Sputum was negative for acid fast bacilli and fungus at 6 months. The patient has continued to improve clinically and is planned to continue therapy with rifampin, ethambutol, clarithromycin, and TMP/SMX for one year after the first negative sputum culture for mycobacteria. He has tolerated drug therapy well with no recognized complications of treatment. The patient declined consideration of surgical resection of the persistent cavity.


Following a hyperflexion injury of the left knee, a 40-year-old male presented to the clinic with difficulty in sleeping because of a painful snapping sensation during flexion and extension of the knee joint. Examination revealed a swelling over the medial joint line, over which a tendon could be felt snapping during flexion and extension of the knee.
Magnetic resonance imaging (MRI) of the knee demonstrated a 20 mm lobulated medial parameniscal cyst, but it was unable to confirm the cause of snapping (Figures and ). Dynamic real-time ultrasonography demonstrated both a meniscal cyst and a meniscal tear (). With the knee in neutral extension, ultrasonography showed that the sartorius tendon was anterior to the cyst, with the tendon of gracilis lying posteriorly (). On flexion of the knee, the sartorius tendon snapped over and came to lie posterior to the cyst to sit at the anterior margin of gracilis (). On extension to neutral with active quadriceps contraction, the sartorius tendon moved rapidly forwards and over the cyst, accompanied by a painful snapping sensation (). contrasts the findings of the fat-saturated proton density MRI with ultrasound of the left knee.
The patient underwent arthroscopy where a large meniscal cyst was seen (). The cyst was arthroscopically decompressed, with complete resolution of symptoms.


A 51-year-old cirrhotic man presented with abdominal pain for one day. He then had a sudden upper gastrointestinal bleeding and a contrast-enhanced computed tomography (CT) demonstrated that a ruptured SAA located near to splenic hilum and formation of hematoma in left epigastric region (). Further ultrasonography confirmed liver cirrhosis and ascites. He underwent an urgent laparotomy. After dissection of severely adhered tissues, a pulsatile mass approximate 4.8 cm×5.6 cm×4.9 cm was found closely adhensive to stomach and splenic hilum. SAA resection, splenectomy, gastric suture and conventional splenorenal shunt were performed thereafter and the recovery was uneventful.
A 42-year-old man presented with sudden abdominal pain and hematochezia for one day. He had a past history of splenoectomy three years prior for traffic injury. He was alcoholic and was once diagnosed as alcoholic cirrhosis. On admission, he was hypotensive at 91/60 mm Hg, and tachycardia with a pulse of 118 beats/min. Upon physical examination, he was found to have a distended abdomen and slight tenderness in periumbilical region, and the shifting dullness sign were noted. Ultrasonography of the abdomen revealed liver cirrhosis, massive ascite. Apart from slightly decreased hemoglobin 11.6g/dL, all other laboratory examinations were unremarkable. He was admitted to gastroenterology ward under the diagnosis of bleeding esophageal varices or peptic ulcer and decompensated alcoholic cirrhosis with ascites.
After his admission, he complained of a progressive distending abdomen and underwent a paracentesis which showed a bloody ascite. Another check of hemoglobin decreased to 8.9 g/dL and an urgent contrast-enhanced CT demonstrated giant hematoma in lesser sac and peri-pancreatic space. Subsequently, he underwent emergency laparotomy and two liters of blood and was found in the abdomen. Severe adhesions were encountered in the peritoneal cavity. Following a careful dissection of severely adhered tissues and removal of clots, blood was suspected to be oozing from the gastric coronary vein, which was sutured subsequently and the patient was sent to intensive care unit. From then on, hemoglobin fluctuated around 11.5 g/dL in the following 66 hrs, followed by a sudden decrease to 9.6 g/dL and 7.5 g/dL on 4th day postoperation. Bedside ultrasonography and contrast-enhance CT demonstrated hematoma again in lesser sac and peri-pancreatic space. Therefore, digital subtraction angiography (DSA) of celiac and superior mesenteric arteries were performed which showed a 2-cm-diameter saccular aneurysm that arised from the middle-distal splenic artery. The aneurysm disappeared after embolization with multiple coils placed in splenic artery segments proximal and distal to the aneurysmal neck (). Unfortunately, the patient underwent a second laparotomy because of his hemoglobin kept dropping. After careful clean of 1000 ml blood and clots, a ruptured and bleeding SAA was found to be tightly adhensive to the neighbouring posterior wall of the stomach. Partial pancreatectomy involving pancreatic tail and aneurysm resection, and gastric suture were performed, after which the patient had an unremarkable recovery and was discharged 20 days later.
A 63-year-old man presented to the emergency department with epigastric pain, malaise, and hematochezia for one day. Except for a history of aortic valve replacement three years ago, there was no other remarkable pertinent finding in his medical history. The patient was afebrile, conscious and haemodynamically stable at triage. After a non-enhanced abdominal CT which demonstrated left renal infarction and suspected acute pancreatitis, the patient was admitted to gastroenterology ward with the diagnosis of upper gastrointestinal bleeding, left renal infarction and possible acute pancreatitis. Upon his arrival on the ward, he suddenly became hypotensive and collapsed, and was found to have profuse sweating. After fluid resuscitation, the patients regained consciousness soon. Emergency contrast-enhanced abdominal CT revealed splenic artery aneurysm rupture and intraabdominal bleeding, left renal and splenic infarction (). Unfortunately, he developed massive bleeding from upper gastrointestinal tract and subsequently cardiopulmonary arrest. Cardiopulmonary resuscitation was unsuccessful and the patient expired. The case was finally diagnosed as infective endocarditis complicated with ruptured infections splenic artery aneurysm and infarction of spleen and left kidney.


A 32-year-old man was referred for clinical evaluation because of a persistent leg ulcer. He had suffered from leg ulcers for 15 years associated with superficial venous insufficiency treated with two phlebectomies. Family history revealed a complete loss of teeth at the age of 30 for his mother and a heterozygous alpha-1-antitrypsin deficiency in his paternal uncle and grandmother, with no known venous insufficiency.
He weighed 79 kg for 1.73 m (BMI = 26 kg·m−2). He had a 6 by 9 cm ulcer on the anterior aspect of the left leg () and bilateral brownish atrophic plaques on the pretibial areas mimicking necrobiosis lipoidica (). Distal pulses were present. His skin was thin with visible venous pattern on the trunk (). Four atrophic scars were noted on his right knee and both thighs (). There was no skin hyperextensibility or joint hypermobility. He had periodontitis with apical root resorption and gingival recession ().
Laboratory studies disclosed normal values for total blood count, coagulation test, renal and liver function tests, serum protein electrophoresis, and hemoglobin electrophoresis. Antinuclear antibodies, antineutrophil cytoplasmic antibodies, antiphospholipid antibodies and cryoglobulinemia were negative. Karyotype and urinary amino acids chromatography were normal. Alpha-1-antitrypsin level was normal, and the patient had a homozygous wild-type genotype for alpha-1-antitrypsin gene. Aortic and lower limb doppler ultrasound ruled out an arterial disease and demonstrated varicose veins on the internal aspect of the left leg emerging from incontinent neosaphenous and perforating veins. Large arteries were normal on CT-scan. Dental X-ray showed generalized alveolar bone resorption.
The diagnosis of EDS-VIII was suspected on clinical grounds. Pathological examination of skin biopsies from the ulcer demonstrated pseudoepitheliomatous hyperplasia without malignancy. A biopsy from brownish atrophic periulceral skin demonstrated dermal fibrosis and hemosiderin deposition without images of necrobiosis lipoidica nor vasculitis. Transmission electron microscopy analysis on skin biopsy revealed irregular interfibrillar spaces (), collagen fibers with variable diameter, and collagen cauliflowers (). Electrophoretic migration pattern of the collagen type I, III, and V proteins from the patient's cultured fibroblasts was presented with a normal profile.
During the year after his first visit, he had all his permanent teeth removed because of multiple dental mobilities and infections. The leg ulcer slowly healed thanks to daily care, type IV venous compression, and sclerotherapy of neosaphenous and perforating veins.


A 71-year-old male, with a body weight of 50 kg and a height of 1.62 m, presented with a visible superficial ulcer with poorly-defined margins on the right side of the tongue. The patient complained of oral pain and dysphagia that had lasted for several months. The patient had a history of drinking 1 beer/day and a smoking history of 30 pack years. The past medical history included heavy chronic obstructive pulmonary disease (COPD), pulmonary tuberculosis, arterial hypertension, peripheral arterial occlusive disease with bilateral stenosis of the internal carotid arteries and liver cirrhosis. The biopsy revealed a poorly-differentiated squamous cell carcinoma of the floor of the mouth.
The surgery involved a tracheotomy to assure the airways remained open and a dissection of levels I–IV on the right side and I–III on the left side of the neck. A radical surgical tumor excision and reconstruction, with a microvascular anastomosed free flap from the anterolateral thigh (ALT), was then performed. Due to continued heavy tracheal secretion as a result of severe COPD, the tracheal cannula was not removed.
The definitive tumor-node-metastasis (TNM) status was pT2 (2.5 cm) pN2b (3/39 with extracapsular extension) G3 Pn1 L0 V0 R0 (≥0.2 cm to the floor of the mouth, ≥1 cm to all the other sites). The multidisciplinary head and neck tumor board recommended adjuvant therapy. Prior to adjuvant therapy, the patient underwent a percutaneous endoscopic gastrostomy (PEG). Every time the patient was placed in the supine position during the radiotherapy setup, the patient experienced severe coughing attacks. No coughing was observed while sitting or lying on the side in a lateral position. Several changes of the tracheal cannula did not improve the coughing. A lateral position was decided upon () utilizing a head mask and a vacuum cushion (BodyFIX; Medical Intelligence, Schwabmünchen, Germany).
Due to the age of the patient, comorbidities and insufficient renal function (clearance 47 ml/min), no platinum-based chemotherapy was administered.
In order to contour the planning target volume (PTV), software was required that allowed the rotation of the CT images at 90 degrees to the normal view that radiation oncologists are used to while contouring (). The contouring was performed using Eclipse (Varian Medical Systems, Palo Alto, CA, USA). The tumor clinical target volume (CTV) was defined as the gross tumor volume (GTV) delineated on a pre-operative CT, including the visible post-operative changes on the post-operative CT plus a safety margin of 10 mm. The elective nodal-CTV was defined according to the literature (,).
In-ward radiotherapy was performed using a TomoTherapy machine (Accuray Incorporated, Sunnyvale, CA, USA). Daily image guidance (IG) was performed using an incorporated mega-voltage CT (MVCT). The overall treatment time was 6.5 weeks. The daily setup errors in the translational directions and the rotational direction (roll) are presented in .
The patient tolerated the treatment well. At the end of the radiotherapy, the patient presented with Common Terminology Criteria for Adverse Events (CTCAE) grade 3 mucositis and CTCAE grade 1 dermatitis. A combined PEG-oral food intake was possible during the entire treatment time.
The patient underwent regular follow-up sessions every three months. Seven months after the surgery, the patient had experienced no more problems with deglutition and the tracheotomy was closed. At nine months after radiotherapy, the patient had no xerostomia, no swallowing dysfunction, localized submental edema, mild voice alteration and moderate skin induration. However, the CT revealed a tumor recurrence at the floor of the mouth, which was histologically proven.
An evaluation of the planned dose was insufficient in this case. An assessment of the impact of setup and soft tissue shrinkage on the actual daily delivered dose was necessary. Therefore, retrospective recalculations of the doses were performed on each of the 32 daily MVCTs. All the daily doses were summed up (). depicts the actual delivered 95% isodose on the last MVCT. The recurrence was localized in field, within the 95% isodose.
The multidisciplinary head and neck board recommended palliative chemotherapy, however the patient succumbed due to tumor bleeding prior to the start of chemotherapy.


A 53-year-old male was referred to Konkuk University Chungju Hospital (Chungju, Korea) for generalized cutaneous nodules on the face, anterior chest wall and legs (), as well as a sore throat. The patient had no history of medical systemic disease, with the exception of hypertension. Furthermore, there was no history of fever, trauma, weight loss, bleeding or any systemic illness. However, the patient was a chronic alcoholic. At admission, the hemoglobin level was 7.0 g/dl, the platelet count was 10.3×103/μl and the white blood cell (WBC) count was 20.8×103/μl. A differential count revealed the following: Neutrophils, 10%; monocytes, 25%; blast cells, 24%; carcinoembryonic antigen, 1.0 ng/ml; carbohydrate antigen 19-9, 12.2 U/ml; α-fetoprotein (AFP), 1,650 ng/ml; erythrocyte sedimentation rate, 99 mm/h; and C-reactive protein, 14.1 mg/l. The serology studies, which included hepatitis B surface antigen, antibodies to hepatitis C virus and a serum antibody titer against HIV, Mycobacterium and Cytomegalovirus, were all negative. Hematoxylin and eosin (H&E) staining of the upper and deep dermis and a periappendiceal tissue skin biopsy specimen demonstrated atypical monomorphic tumor cells with round to oval vesicular nuclei and a moderate amount of cytoplasm infiltration (). In addition, the immunohistochemical staining revealed that the tumor cells were positive for leukocyte common antigen and CD43, but negative for CD34, CD68, C-kit, cytokeratin, myeloperoxidase and S-100 protein. H&E staining of the bone marrow biopsy revealed packed marrow with increased atypical cells, large nuclei with nuclear membranes, dispersed chromatin and abundant cytoplasm. Furthermore, the cells were positive for myeloperoxidase, CD43 and CD68 (). H&E staining of the right tonsillar punch biopsy demonstrated several irregular fragments of tonsils that revealed diffuse infiltration of the atypical cells. The immunohistochemical staining revealed that the cells were positive for myeloperoxidase, but negative for CD34, CD43 and CD20 (). Liver magnetic resonance imaging (MRI) demonstrated a 6-cm ill-defined mass in hepatic segments 4 and 8. H&E staining of the liver biopsy revealed immature blasts infiltrating the hepatocellular carcinoma that were positive for CD3 and CD68, but negative for myeloperoxidase and CD34 ().
A diagnosis of acute myelomonocytic leukemia (AML-M4) was confirmed with skin, tonsil and hepatic involvement and HCC. The patient was administered standard induction chemotherapy with idarubicin (12 mg/m2/IV for 1–3 days) and cytarabine (100 mg/m2/IV for 1–7 days). On the 36th day, the bone marrow examination demonstrated complete remission with 2.2% blast cells. With regard to the HCC, the patient was treated by transcatheter hepatic arterial chemoembolization (TACE). The patient was scheduled to undergo consolidation chemotherapy, but the complete blood count revealed a WBC count of 21.7×103/μl (72% blasts). Therefore, relapse of AML was suspected. The patient did not wish to undergo further treatment and succumbed shortly thereafter, due to the progression of AML.


A 41 year old male was referred to us in 2008 with a diagnosis of Complex regional pain syndrome (CRPS) of bilateral wrists, following an electric current burn during welding in 2003. He described the pain as sharp, like "needle pricks", with occasional radiation up to the elbow. Once or twice a month he felt as though "bugs were crawling underneath his skin" over left wrist. In the past, he had tried gabapentin, amytriptyline, propoxyphene, acetaminophen with codeine and physical therapy without benefit. He reported some pain relief with topiramate and tramadol. He was not able to continue these medications due to financial reasons, except acetaminophen with hydrocodone. On examination, there were no clinical features of complex regional pain syndromehe did not meet the physical criteria for CRPS except for non-dermatomal pain and hyperalgesia over the wrist. His imaging studies where unremarkable. For diagnostic purposes, a In order to determine if the pain is sympathetically mediated, a local anesthetic left stellate ganglion block was performed, after informed consent, under ultrasound guidance with immediate relief of pain and complete pain relief for over five weeks. Having established the pain as responsive to stellate ganglion block, we elected to repeat the block under ultrasound guidance with steroid. Following informed consent and establishment of peripheral intravenous access, standard ASA monitors were applied. A preliminary scout scan including color flow Doppler was performed. Pain medicine fellow, experienced in performing ultrasound guided blocks, performed the procedure. With the patient supine on the procedure table, the left side of the neck was prepped with betadine and sterile drapes applied. A skin wheal was raised with 1% lidocaine over the posterolateral part of the transducer for an in-plane approach. Under ultrasound guidance, a 3.5 inch, 25 G needle was advanced to approximately 2-3 cms during attempts to visualize the needle tip (). The patient experienced severe paraesthesia in the arm which necessitated immediate removal of the needle. Following recovery from the paraesthesia, in approximately five minutes, the needle was reintroduced at a different angle. Only portions of the needle were visualised along with tissue movement. As the needle tip could not be visualized clearly, 1 ml of injectate from a 10ml mixture containing equal volumes of 1% lidocaine, 0.25% bupivacaine and 40mg of Depomedrol was injected after a color flow Doppler exam and negative aspiration for blood. Immediately following the injection, the patient experienced severe ringing in both ears. Aspiration at this time was positive for blood. The needle was completely withdrawn and preparations made for resuscitation anticipating convulsions. Oxygen was administered via face mask while his vital signs continued to be monitored. Gradually, over 15 minutes, ringing in his ears decreased. He did not develop any other symptoms or signs of local anesthetic toxicity. At the patient's request, as he had good pain relief with the prior left stellate ganglion block, the attending physician changed to an out of plane approach to avoid the brachial plexus and completed the ultrasound guided stellate ganglion block, without any further complications. A repeat test dose was administered and visualized real time under ultrasonography. Following the test dose, 10ml of a mixture of 1% Lidocaine and 0.25% Bupivacaine with 40mg of Depomedrol was injected, in small increments. Vital signs remained stable. There was an approximately 20C increase in temperature compared to the right hand. His pain in the wrist also decreased dramatically. At the conclusion of the procedure, patient was noted to have features of Horner’s syndrome including a left sided ptosis, without hoarseness of voice or swallowing difficulty. He was then discharged home after monitoring for 30 minutes post procedure. Two days post procedure, patient reported a mild discomfort in his throat and chest. He declined our recommendation to go to the Emergency room for evaluation. During his follow up visit in two months, he reported occasional feelings of fullness in his ears and tinnitus which also resolved over the next few months. He declined further interventions and elected to manage his pain conservatively with oxycodone 3 times daily.


A 58-year-old Chinese male was admitted to our hospital with a chief complaint of chest tightness and shortness of breath after activities for more than 4 months. Physical examinations show his heart rate of 96 beats/min, blood pressure of 130/80 mmHg, and no obvious mass in the neck. Hematological examinations show thyroid function was normal. Computed Tomography (CT) of the neck and chest showed a goiter of low density in the right thyroid, and a giant cystic nodule on the back of the right thyroid which grew into the right posterior mediastinum (Figure
A). The tumor was located between the spine and the dorsal part of trachea and esophagus, its lower edge extended beyond the aortic arch and compressed the trachea to the left (Figure
B-D). Other laboratory tests revealed no abnormalities. In March 2012, surgery was done by right posterolateral thoracotomy of the fourth intercostal space, a posterior mediastinal tumor (10.0 × 9.0 × 9.0 cm) fitting the location on CT was seen, and the right gland lobe was not excided. The mass was completely encapsulated with large tension (Figure
E), and this cyst-solidary mass was hypervascular. Microscopy showed thyroid hyperplasia without malignancy. The final diagnosis was a secondary giant thyroid goiter in posterior mediastinum. In December 2012, the latest follow-up showd that patient now had no symptoms after activities or thyroid dysfunction.
Intrathoracic goiter refers to a goiter where most of its mass is found within the mediastinum. According to the originations of thyroid tissue, intrathoracic goiter can be divided into primary intrathoracic goiter and secondary intrathoracic goiter. The vast majority of intrathoracic goiters are secondary ones which arise from the lower part of one lobe or both lobes of cervical thyroid or isthmus and grow down through the thoracic inlet. Swallowing, gravity and thoracic negative pressure help the growing goiter direct into the chest cavity. Anatomically speaking, goiter in the chest cavity generally grows to the position of relatively low resistance. At first, the tumor will grow into the anterior superior mediastinum between trachea and sternum, forming the common retrosternal thyroid goiter. Because there are thymus (may atrophy), left and right brachiocephalic veins and superior vena cava in the front, aortic arch and its three branches (phrenic nerve and vagus nerve have smaller resistance) in the middle left of retrosternal space, tumor growth will be resisted there. Right posterior mediastinum has relatively low resistance than left posterior mediastinum, and it helps form right posterior mediastinal goiter. The primary intrathoracic goiter only accounts for 0.2 ~ 1% of all the intrathoracic goiters, it affects females more often (male: female = 1 : 3 or 1 : 4)
[]. Its causes are totally different from the ones of secondary intrathoracic goiter. During the embryonic developmental period of thyroid gland, part or all of the thyroid blastoma leaves primordium and is pulled into the thoracic cavity by the descendent heart and great vessels, then continues to develop in the thoracic cavity, forming the final primary intrathoracic goiter. Because of different originations, secondary posterior mediastinal goiter is often continued with the cervical thyroid gland, with blood supply from inferior thyroid artery and its branches while primary posterior mediastinal goiter maintains little or no connection with the cervical thyroid gland, and has a blood supply derived from intrathoracic arteries
[].
Patient generally has no symptoms when the goiter is small, many cases are only found in occasional chest radiographic examination or autopsy. As the goiter increases in size, a variety of clinical symptoms may appear due to compression of surrounding organs and tissues (i.e. trachea, esophagus, lungs, or even superior vena cava). Most investigators agree that respiratory symptoms are caused by compression of the airway
[]. Thyroid function test has a low susceptibility in predicting goiter, for most patients are normal and only 10 ~ 15% show hypothyroxinemia.
Radiographic image is the most effective and necessary diagnostic method for intrathoracic goiter. CT scan is the most common one for preoperative evaluation. On CT films, intrathoracic goiter usually manifests as a clear boundary mass, its density varies due to the amount of iodine contained: when the amount of iodine in the mass is low, its density is close to the soft tissue of chest wall, and when the amount of iodine is high, its density could be greatly higher than soft tissue. In addition, its density can be uneven due to colloid cysts and calcified plaque. Radionuclide scan is also one of the common diagnostic methods, but it is not so effective when compared with its usage in thyroid goiter of other regions because the intrathoracic goiter does not always uptake iodine.
The differential diagnosis of intrathoracic goiter are of great variety, it should be differentiated from lymphadenopathy, branchial cleft cyst, arterial aneurysm, neurogenic tumour, pheochromocytoma, spinal cord injury, hiatus hernia, etc.
When the trachea, esophagus or vena cava is compressed, surgical resection of intrathoracic goiter must be done. Preventive operation is also feasible for asymptomatic patients in order to avoid future compression. Secondary intrathoracic goiter is always taken out through inferior cervical collar incision, but posterior mediastinal goiters which extended beyond the aortic arch may require additional extracervical incisions
[]. A variety of operation modes exist, including sternotomy, clavicular resection, anterior posterolateral thoracotomy and Video Assisted Thoracoscopic Surgery (VATS). A specific mode depends on the location, size of the mass and its relationship with surrounding important organs. Attention should be payed to some special points during anesthesia. Similar to the anesthesia of mediastinal giant tumors, intravenous anesthesia combined with tracheal intubation is used. But because the tumor is located in the posterior mediastinum, compressing adjacent organs, especially the carina, plus various factors such as mental stress, thick sputum or postural changes, extreme hypoxia or heart arrest may occur at any time, resulting in failure of anesthesia induction and tracheal intubation, in which cardiopulmonary bypass (CPB) may be needed. When patient needs to be changed to lateral position after anesthesia induction, compression of heart or great vessels by the tumor should be watched out. Chest must be opened as soon as possible to decompress the heart when cardiac output decreases and blood pressure drops sharply. Common surgical complications include postoperative airway collapse, respiratory tract infection and bleeding
[].