Kubota Y, Furuya Y, Ueda T, Ichikawa T, Isaka S, Ito H, Matsuzaki O
Department of Urology, Chiba University School of Medicine.
Nihon Hinyokika Gakkai Zasshi. 1998 Aug;89(8):726-9. doi: 10.5980/jpnjurol1989.89.726.
A case of bilateral pheochromocytomas with von Hippel-Lindau's disease (VHL) is reported. A 46-year-old woman who had hyperglycemia was admitted to our hospital because of abdominal tumors. Her elder sister and niece had been diagnosed as VHL. Ultrasonography, CT, and MRI revealed bilateral adrenal tumors. Noradrenaline levels in serum and urine were elevated and 131I-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Moreover, she had bilateral renal cysts and cerebellar hemangioblastoma. Bilateral adrenalectomies were performed and pathological diagnosis was pheochromocytoma. This is the seventh case of bilateral pheochromocytomas with VHL reported in Japan.
报告一例伴有冯·希佩尔-林道病（VHL）的双侧嗜铬细胞瘤病例。一名患有高血糖症的46岁女性因腹部肿瘤入住我院。她的姐姐和侄女被诊断为VHL。超声、CT和MRI检查显示双侧肾上腺肿瘤。血清和尿液中的去甲肾上腺素水平升高，131I-间碘苄胍闪烁显像显示双侧肾上腺有放射性浓聚。此外，她还有双侧肾囊肿和小脑成血管细胞瘤。进行了双侧肾上腺切除术，病理诊断为嗜铬细胞瘤。这是日本报道的第七例伴有VHL的双侧嗜铬细胞瘤病例。
