© JAPI • August 2012 • VOL. 60 53 Unsuspected Pheochromocytoma Multisystem Crisis : A Fatal Outcome in a Young Male Patient A Gundgurthi * , S Gupta # , MK Garg ** , P Ganguly ## , R Bhardwaj ### Abstract Pheochromocytoma is a great mimicker and has varied presentation. It can present as medical emergency with hypertensive emergencies, acute cardiac event, neurological manifestations, systemic inflammatory response syndrome, acute respiratory distress syndrome, and metabolic emergencies 4 . Here we report a young individual who after a bout of exercise developed breathlessness and rapidly developed multiorgan failure which was fatal and post mortem examination revealed pheochromocytoma in right adrenal gland. Pheochromocytoma multisystem crisis is discussed. * Resident, ** Professor and HOD, Department of Endocrinology, # Resident, ## Classifed Specialist, ### senior Adviser, Department of Pathology, Army Hospital (Research and Referral), Delhi Cant. 110010 Received: 03.12.2010; Revised: 09.03.2010; Accepted: 30.03.2011 Introduction P heochromocytoma is a great mimicker and has varied presentation 1 . It has been compared to a metabolic volcano. 1 When dormant it remains asymptomatic but once it explodes it can be catastrophic. We present a young individual who after a bout of exercise developed breathlessness and rapidly developed multiorgan failure which was fatal. Post mortem examination revealed pheochromocytoma in right adrenal gland. In literature we could not fnd any reported case of pheochromocytoma multisystem crisis (PMC) from India. PMC is reviewed and discussed. Case Report A 27 year old male patient from defence services presented with sudden onset progressive breathlessness present at rest, difuse constricting chest pain, hemoptysis after a 5 km run. He was asymptomatic at the start of exercise. there was no other contributory history. He gave history of a similar episode about 2 years back and told that cardiac evaluation at that time was normal. On examination he was afebrile, pulse rate 100/ minute, blood pressure 160/120 mmHg; at this time he was profusely sweating, tachypnoeic, in distress, with cold moist peripheries. His respiratory auscultation revealed diffuse crackles and decreased air entry in both the lungs. Rest of systemic examination was unremarkable. Oxygen saturation at room air was 85%. He was started on high fow oxygen supplementation with which he was able to maintain adequate saturation (96%). His blood pressure normalized after few hours. He was started on parenteral antibiotics, fuid and electrolyte balance was optimized. He became febrile on day 3 of admission and he developed right sided hemiplegia. He was referred to this hospital for further evaluation and management. Initial investigations showed a hemoglobin of 13.4 gm/ dL (13.0-17.0), total leucocyte count of 13,500 cells/cu.mm (4,00-11,000), (75% neutrophils, 20% lymphocytes), EsR was 20 mm fall at the end of frst hour, blood glucose was 96 mg/ dL, sodium was 136 mEq/L, potassium was 3.9 mEq/L, blood Case Report urea nitrogen was 58 mg/dL (7-19), serum creatinine was 2.1 mg/dL (0.5-1.6), total bilirubin was 0.7 mg/dL (0.2-1.0), total protein was 7.6 g/dL (6.0-8.0), albumin was 4.8 g/dL (3.5-5.5), serum aspartate and alanine transferase were 45 and 55 u/L (<40) respectively, alkaline phosphatase was 170 u/L ( <250). urine examination showed 2+ albumin, few leucocytes and erythrocytes. Chest radiograph showed bilateral difuse non homogenous opacities with breakdown suggestive of acute respiratory distress syndrome (ARDs) (Figure 1). Bronchio alveolar lavage was subjected to culture and sensitivity which grew staphylococcus. Non-enhanced contrast computed tomography (NCCt) of head showed left middle cerebral artery territory infarct with hemorrhagic transformation and midline shift. 2D echocardiography, carotid doppler and vasculitic work up (ANA, cANCA, pANCA) were normal. His overall general condition started worsening and had to be mechanically ventilated. there was deterioration in his renal functions. He was managed as a case of severe sepsis with multiorgan system dysfunction (MODs). He was also given one gram of intravenous methylprednisolone as a desperate atempt to save young life for suspected immunological injury and septic shock not responding to ionotropic agents. Patient succumbed on day 5 after initial presentation. Post-mortem examination revealed congested lungs, liver and spleen, left ventricular hypertrophy, hemorrhagic infarct in left tempero-parietal region, enlarged right adrenal gland measuring 5 cm in maximal diameter and weighing 150 grams (Figure 2). Histopathological examination from right adrenal gland showed a well encapsulated tumor arising from medulla consistent with pheochromocytoma and stained positive for chromogranin A and synaptophysin (Figure 3). Discussion Pheochromocytoma is an intraadrenal paraganglioma arising from catecholamine-producing chromafn cells in the adrenal medulla 2 . In a case series from Mayo clinic (1928–1977) about 85% of pheochromocytomas were diagnosed at autopsy. 3 the frequency of diagnosis has increased considerably in the last few decades. However pheochromocytoma is still a great mimicker. It can present as systemic infammatory response syndrome (sIRs), acute respiratory distress syndrome (ARDs), MODs, hypertensive emergencies, acute cardiac event, neurological