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A giant cystic pheochromocytoma presenting with myocardial infraction - a case report

 

GP Samarasinghe1, KPL Madushani1, DDK Abeyaratne1, NP Somasundaram1,

K Dissanayake2, ASK Banagala3, AS Pallewaththa4, 1Diabetes & Endocrinology unit, NHSL Colombo, Srilanka, 2Department of Pathology, NHSL Colombo, Srilanka

3Department of Surgery, NHSL Colombo, Srilanka, 4Department of Radiology, NHSL Colombo, Srilanka

 

 

Introduction: Cystic pheochromocytoma is a rare neuroendocrine tumour, usually asymptomatic at presentation making pre-operative diagnosis difficult. We report a case of giant pheochromocytoma which appeared as a large cystic adrenal lesion acutely presenting with a ST elevation myocardial infarction.

 

Case Description: 42 years old female presented with Electrocardiographic evidence of acute anterolateral ST elevation myocardial infarction with a normal coronary Angiogram. She had a history suggestive of adrenergic spells and poorly controlled diabetes, hypertension for 5 years. 24 hour total urinary Metanephrines was 45.7mg/24 hours (<1mg/24 hours) and contrast enhanced CT abdomen revealed a purely cystic right suprarenal lesion with enhancing thin wall, measuring 8cm x 8.5cm x 7.5 cm. Whole body Magnetic Resonance Imaging (MRI) showed the same mass, 6.3x 6.2x7.1cm as hypointense in T1W images with a hyperintense rim and heterogeneous hyperintensity. She underwent surgery following optimal pre-operative preparation and macroscopically the lesion was circumscribed with a size of 10.5x 90x 7.5cm filled with homogenous brownish material, falling in the category of giant cystic pheochromocytoma. Histology and Immunohistochemical analysis showing strong positivity for Chromogranin and synaptophysin confirmed the diagnosis of pheochromocytoma with a Ki 67 index of 6.5%. Imaging was negative for distant metastasis.

 

Conclusion: Pheochromocytoma is heterogenous in clinical presentation as well as in radiological appearance. Therefor high degree of suspicion and use of highly sensitive investigations in ruling in or out of diagnosis are crucial.