Normotensive Bilateral pheochromocytoma with Lindau Disease

[摘要] References(13)Cited-By(1)A 46-year-old man was found to have numerous cerebellar hemangioblastomas on magnetic resonance imaging (MRI). He denied any symptoms and had no history of hypertension, but his family history was remarkable for a father who died of renal cell carcinoma. Computed tomography (CT) of the abdomen revealed bilateral adrenal pheochromocytomas with significant enhancement in the regions where 131I-metaiodobenzylguanidine (MIBG) had noticeably accumulated. Endocrinological examinations demonstrated high plasma and urine catecholamine concentrations which were very responsive to metoclopramide and glucagon loading tests, without a significant change in blood pressure. After resection of bilateral pheochromocytomas, he underwent an operation for the cerebellar tumors. Since pheochromocytomas associated with Lindau or von Hippel-Lindau (VHL) disease have a tendency to multiple occurrence in normotensive patients, we suggest that patients with a family history involving VHL lesions should undergo cranial MRI, abdominal CT, MIBG scintigraphy and endocrinological examinations.

[学科分类] 内分泌与代谢学

[关键词] von Hippel-Lindau disease;Cerebellar hemangioblastoma;Renal cell carcinoma;MRI;131I-metaiodobenzylguanidine (MIBG);Metoclopramide;Glucagon