CASE REPORTA young lady (18 years of age) presented with recurrent episodes of headache for 3 years, associated with episodic sweating and palpitations. She was recently documented to have blood pressure (200/160 mmHg) and was referred to us for further management.A detailed family history revealed the occurrence of central nervous system (CNS) tumors in her father and two of his siblings [Figure 1]. Her father had undergone C7–D3 decompression laminectomy for D2, D3 hemangioblastomas. He recovered, but subsequently developed recurrence of the spinal tumor. At this stage, he was also detected to have a right adrenal mass, and both tumors were left untreated. He developed paraplegia and eventually died following a cerebrovascular accident. His two brothers underwent surgery for cerebellar hemangioblastomas at age 46 and 50 years, and they were reportedly well following surgery. Our subject has two siblings aged 17 and 20 years, both of whom had no symptoms suggestive of VHL.Open in a separate windowFigure 1Pedigree analysis of patientClinical examination revealed no neurocutaneous markers. Her blood pressure was 170/140 mmHg (supine) and 150/120 mmHg (standing). Fundus examination revealed bilateral papilledema. Her neurological examination was normal.Her 24-hour urine metanephrine level was 100 mcg and normetanephrine level was >5700 μg [normal range up to 600 mcg (metanephrine) and 900 mcg (normetanephrine)]. She also had impaired glucose tolerance. Computed tomography (CT) scan revealed a large heterogeneous right adrenal mass with patchy peripheral enhancement, a smaller left adrenal mass and an enhancing lesion in the distal body of the pancreas [Figure 2]. Metaiodobenzylguanidine (MIBG) scan showed significantly increased uptake over both adrenal glands [Figure 3]. At surgery, she was found to have an 8-cm tumor in the right adrenal, a 2-cm tumor in the left adrenal and a 2-cm hard pancreatic neoplasm in the distal pancreas [Figure 4]. She underwent bilateral adrenalectomy (right total adrenalectomy, left cortical sparing adrenalectomy) and distal pancreatectomy with splenectomy. Histology of the tumor showed bilateral pheochromocytoma and well-differentiated neuroendocrine tumor of the pancreas. The postoperative period was uneventful, and she was advised regular follow-up. We also advised genetic screening to all her first-degree relatives. After surgery, she was asked to continue Tab. Prednislone 5 mg once daily (in the morning). This patient is on telephonic follow-up and came last month back for follow-up. We planned to taper off steroids and assess response but she herself stopped the medicines and her serum cortisols were checked which were in normal range off all medicines. She is not on any antihypertensive medication. Following surgery, she had no visual complaints and her papilledema had resolved.