Normotensive Bilateral pheochromocytoma with Lindau Disease

Endocrine Journal

TETSUYA OISHI³  HIDEO KATAOKA³  HIROFUMI MAKINO³  MINORU NAKAGAWA²  TOSHIO OGURA¹  NOBUHIKO HAYAKAWA³  FUMIO OTSUKA³ 

[1] Department of Neurological Surgery, Okayama University Medical School;Health and Medical Center, Okayama University;Department of Medicine III, Okayama University Medical School

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

DOI  :  10.1507/endocrj.43.719

学科分类：内分泌与代谢学

来源: Japan Endocrine Society

【 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.

【 授权许可】

Unknown