von Hippel–Lindau Disease with Multi-organ Cysts and Ticking Pheochromocytoma: A Rare Case

Abstract

Objective: A rare disease. Background: von Hippel-Lindau (VHL) disease is an autosomal dominant familial syndrome with a multitude of benign and malignant multivisceral tumors and one-third of them harbor pheochromocytoma (PCC). We intend to present one such rare case and challenges in its surgical management. Case: A 38-year-old lady presented with hypertension and episodic headache. Biochemical evaluation was suggestive of hypercatecholaminism. Magnetic resonance imaging (MRI) showed the presence of a heterogeneous intensity lesion in the left suprarenal area suggestive of PCC. Multiple cysts were seen in both the kidneys as well as in the pancreatic head and tail. Imaging of the brain and spine showed a left cerebellar and spinal cord hemangioblastoma, respectively. I-131 metaiodobenzylguanidine (MIBG) showed high uptake in the left suprarenal region. After adequate preparation, she underwent left adrenalectomy. Histopathology was consistent with adrenal PCC and the patient was biochemically cured of hypercatecholaminism. The presence of PCC and other manifestations of VHL type 2A were evident in this patient. Conclusion:The presence of multiple renal and pancreatic cysts can camouflage adrenal tumors intraoperatively. Careful identification, dissection, and differentiation of PCC from these cysts are crucial. Multicentricity and multifocality of PCC are not uncommon in VHL and these patients need lifelong close follow-up for other tumors as well.