Otsuka F, Ogura T, Nakagawa M, Hayakawa N, Kataoka H, Oishi T, Makino H
Department of Medicine III, Okayama University Medical School, Japan.
Endocr J. 1996 Dec;43(6):719-23. doi: 10.1507/endocrj.43.719.
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.
一名46岁男性在磁共振成像（MRI）检查中发现有多个小脑血管母细胞瘤。他否认有任何症状，也没有高血压病史，但其家族史中有一位父亲死于肾细胞癌，这一点值得注意。腹部计算机断层扫描（CT）显示双侧肾上腺嗜铬细胞瘤，在131I-间碘苄胍（MIBG）明显积聚的区域有显著强化。内分泌检查显示血浆和尿儿茶酚胺浓度升高，对甲氧氯普胺和胰高血糖素负荷试验反应非常敏感，血压无明显变化。双侧嗜铬细胞瘤切除术后，他接受了小脑肿瘤手术。由于与林道或冯·希佩尔-林道（VHL）病相关的嗜铬细胞瘤在血压正常的患者中有多发倾向，我们建议有VHL病变家族史的患者应接受头颅MRI、腹部CT、MIBG闪烁扫描和内分泌检查。
