Retroperitoneal paraganglioma: a chameleon masquerading as an adrenal pheochromocytoma

Retroperitoneal paragangliomas are rare tumours arising from neural crest cells located near or in the autonomic nervous system; right from the skull bass to the pelvic floor. Often called the great masqueraders, their diagnosis and treatment is often difficult and fraught with danger, considering the close proximity of these tumours to important retroperitoneal organs. A 29-year-old woman presented to the outpatient department with complaints of paroxysms of headache, sweating and palpitations for one year. She was found to have elevated urinary metadrenalines (metanephrines) and a suprarenal mass on contrast-enhanced computed tomography of the abdomen, which demonstrated increased tracer activity on I-131 iodine-123 meta-iodobenzylguanidine scintigraphy. Adrenal pheochromocytoma was diagnosed and she underwent laparoscopic adrenalectomy after stabilisation of her blood pressure. Laparoscopy revealed a large suprarenal mass with dense adhesions to the inferior vena cava that was carefully separated from the vein and surrounding structures. The cut section revealed a heterogeneous mass encasing a normal adrenal gland and histopathology confirmed the same, confirming the final diagnosis of retroperitoneal paraganglioma.