Masunaga Koichi, Inadome Akito, Sugiyama Yutaka, Maeda Yoshihiro, Satoji Yo, Takahashi Wataru, Yoshida Masaki, Ueda Shoichi, Ikeda Kazuyoshi, Takano Yuichi, Yatsuda Junji
Department of Urology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan.
Nihon Hinyokika Gakkai Zasshi. 2007 Nov;98(7):843-7. doi: 10.5980/jpnjurol1989.98.843.
A case of bilateral pheochromocytomas with von Hippel Lindau disease (VHL) is reported. A 32-year-old man visited Kumamoto Red Cross Hospital for further examination of hypertension. Computed tomography revealed bilateral adrenal tumors and noradrenalin levels in serum and urine were elevated. Suspecting bilateral pheochromocytoma, he was reffered to our hospital for further examination and treatment. 131I-MIBG scintigraphy showed accumulation in bilateral adrenal glands. Moreover, he had cerebellar and spinal hemangioblastomas. Bilateral adrenalectomies and left nephrectomy were performed because tumor thrombus extended into the left renal vein, and pathological diagnosis was pheochromocytoma. His sister had been diagnosed as VHL disease. We diagnosed the patient as VHL disease because of the existence of cerebellar and spinal hemangioblastomas, bilateral pheochromocytomas, missense mutation and his family history. This is the eleventh case of bilateral pheochromocytomas with VHL disease reported in Japanese literatures.
报告了一例伴有冯·希佩尔-林道病（VHL）的双侧嗜铬细胞瘤病例。一名32岁男性因高血压进一步检查就诊于熊本红十字医院。计算机断层扫描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。怀疑为双侧嗜铬细胞瘤，他被转诊至我院进行进一步检查和治疗。131I-MIBG闪烁显像显示双侧肾上腺有放射性浓聚。此外，他还有小脑和脊髓血管母细胞瘤。由于肿瘤血栓延伸至左肾静脉，遂行双侧肾上腺切除术和左肾切除术，病理诊断为嗜铬细胞瘤。他的姐姐被诊断为VHL病。由于存在小脑和脊髓血管母细胞瘤、双侧嗜铬细胞瘤、错义突变以及他的家族史，我们将该患者诊断为VHL病。这是日本文献中报道的第11例伴有VHL病的双侧嗜铬细胞瘤病例。
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