A 44-year old male residing at Howrah, West Bengal, India, businessman by profession, presented with severe respiratory distress, high grade fever and productive cough for the past 3 months. The patient was apparently well 3 months before, when he first noticed rise of temperature and left sided chest pain along with cough and expectoration. Fever was high from the beginning(104-105 F), intermittent in nature and not associated with chill or rigor that would relieve only after taking medications. Chest pain was pleuritic and localized in the left side. Cough was initially mild but it became severe within few days accompanying by thick, yellow and foul smell sputum about 50 ml per day. Afterwards, gradually increasing respiratory distress ensued, but it was not associated with hemoptysis. There was no history of weight loss during this period. He was admitted to a local hospital, where he received few intravenous antibiotics and was discharged almost asymptomatic with anti-tuberculosis drugs (ATD) on empirical basis. Thereafter, patient was apparently well for next two months. Meanwhile, he again suffered from high grade fever with chest pain over the same site along with productive cough. He was re-treated with some oral medications and subsequently fever subsided but other symptoms persisted, for which he was brought to our hospital for the first time and was immediately admitted.
Clinical examination revealed polycythemia, mild cyanosis and grade 2 clubbing (Figure 1). There was no significant palpable lymph node anywhere in the body. Though he was hemodynamically stable, tachypnea persisted for long. Respiratory system examination revealed woody dullness over left infrascapular region on percussion and bronchial breath sound along with coarse crackles over the same area on auscultation. Blood biochemical examination revealed Hb = 18.9 mg/dl, TLC = 20200 ( Neu = 85%, Lymph = 12%,Eos = 1%), fasting blood sugar (FBS) = 71 mg/dl, urea = 23 mg/dl, creatinine = 1.1 mg/dl with normal serum electrolytes and liver function tests. Chest X-ray revealed left sided consolidation with pleural reaction and right sided diffuse pneumonitic changes (Figure 2). Repeated pleural tap was unsuccessful. Thoracic HRCT was suggestive of consolidation at left mid zone with pleural effusion at the base of the left lung (Figure 3). CT guided fine needle aspiration from left sided pleural effusion showed blood elements, a few foam cells and mesothelial cells. No malignant cell was seen. He was treated again with higher dosage of intravenous antibiotics and ATDs was also administered concomitantly. He was discharged with ATDs and advised bronchoscopy, as his coughs persisted. Since he got some relief from dyspnea and chest pain, he refused to undergo bronchoscopy.
After an event free interval of 3 months he felt similar problem as in previous episodes. These symptoms were sudden onset and patient was rushed to the emergency department and admitted accordingly. Dyspnea was severe and fever was high, continuous and associated with chill. The amount of sputum was huge with whitish color and foul smell. On physical examination, cyanosis and clubbing were present with respiratory rate of 30/min, pulse rate of 110/min and BP of 110/80 mmHg. No lymph nodes were palpable throughout in the body. On respiratory system examination, trachea was central in position, chest movement was diminished in left side, breath sounds decreased in both sides (more at the left side), and coarse crepitation was present in both sides of chest. Other system examinations showed no abnormality except scrotal examination which revealed a hard mass involving right testis with loss of testicular sensation. Left testis was normal. He developed a new symptom, hyperpigmented macules over the lower lip, margin of tongue and buccal mucosa. This time, his baseline investigations showed higher level of hemoglobin with other parameters being within normal range. Results of biochemical analysis revealed normal serum levels of thyroid stimulating hormone (TSH), free thyroxin (FT4), alpha fetoprotein, sex hormone binding globulin, testosterone and estradiol, assayed on two different occasions. The levels of luteinizing hormone (LH), follicle stimulating hormone (FSH), prolactin and angiotensin converting enzyme did not reveal any abnormality. The 24-hour urinary steroid excretion was normal. The serum estrogen level was undetectable. Serum LDH was 1254 U/L(N < 240 U/L),beta-hCG was 1.71 mIUv/ml(normal adult male/normal adult non pregnant female-0.1-5.7). HIV-COMB test to detect HIV antibody was non reactive. Apart from routine investigations, bronchoscopy was performed and broncho-alveolar lavage (BAL) fluid was cultured and diagnosed as Klebsiella pneumonia. Culture was negative for tuberculosis in BACTEC media and cytology could not detect malignant cells. Keeping in mind the newly developed oral mucosal pigmentations, Barium meal x-ray and follow-through was performed which showed gastric dilatation with sub-optimal mucosal coating; no obvious gastric polyps were seen. Upper gastrointestinal endoscopy was also inconclusive. However, colonoscopy detected multiple polyps (Figure 4) which upon histopathological examination were proved to be of hamartomatous nature without any malignant character, confirming the diagnosis of PJS. There was no history of exposure to any drugs and no family history of PJS.
Antibiotics were administered according to the drug sensitivity report. Chest X-ray was taken showing left sided non resolving pneumonia. He was treated with injectable piperacillin, tazobactam and gentamicin, and nebulisation with salbutamol. Abdominal Ultrasound was normal, but scrotal ultrasound revealed a large (10cm X 7.8 cm) mixed echogenic mass lesion involving the whole right testicular substance with dispersed necrotic areas suggestive of testicular malignancy. Left testis and epididymis were normal. High inguinal orchidectomy on right side was performed and histopathological examination of biopsy specimen showed large cell calcifying Sertoli cell tumor (Figure 5). Immunohistochemistry showed tumor cells expressing Inhibin, Calretinin and CD 56 and were immune negative for CK-7 and EMA. The tumor also stained positively for erythropoietin. Thus, a testicular tumor secreting ectopic erythropoietin explained the polycythemia in this patient. The aromatase activity was measured in the testicular biopsies and the levels detected were as follows: right testis, 1183 pmol/h/g protein and left testis 1358 pmol/h/g protein. These values were much higher than what is found in normal adult testis (33 pmol/h/g protein). Chest CT revealed variegated mass lesion and CT-guided FNA from lung lesion showed evidence suggestive of metastasis from testicular tumor. Abdominal CT revealed no obvious peri-or para-aortic lymphadenopathy (skip metastasis) and brain CT appeared to be normal.
The patient was put on a chemotherapy regimen comprising of bleomycin, etoposide and cisplatin. Though he tolerated the induction phase well, subsequently developed sepsis secondary to pancytopenia in the late consolidation phase and ultimately died despite all possible medical supportive therapy.
Thoracic HRCT showing consolidation at left mid zone with pleural collection in left base.