Irvin G L, Fishman L M, Sher J A
Surgery. 1983 Dec;94(6):938-40.
During a 15-year period, 24 patients were treated operatively for pheochromocytoma at this medical center. In this group a father and three daughters were recognized to have the familial variety of this disease. Malignant hypertension, which began at an early age, was the most prominent characteristic. Operative treatment of the three sisters occurred at the ages of 9, 15, and 17 years. All pheochromocytomas were bilateral and were confined to the adrenal glands except for hepatic extension in the father. In contrast to most recent reports of familial pheochromocytoma, there was no evidence of multiple endocrine adenomatosis in this family. Total adrenalectomy was performed in only the youngest patient and, although normotensive, she requires close observation and steroid replacement. Three patients underwent a controversial partial adrenalectomy after excision of bilateral tumors. The father has remained hypertensive for 8 years and has evidence of a residual tumor in the liver. The two daughters who have had subtotal adrenalectomies remain normotensive, require no medication, and have normal urine catecholamine values 7 and 3 years after operation. These results show that removal of bilateral pheochromocytomas without total adrenalectomy may allow normal adrenal function free of recurrence for several years in patients without multiple endocrine adenomatosis.
在15年期间，该医疗中心对24例嗜铬细胞瘤患者进行了手术治疗。在这组患者中，发现一名父亲和三个女儿患的是家族性这种疾病。早年就开始的恶性高血压是最突出的特征。三姐妹的手术治疗分别在9岁、15岁和17岁时进行。所有嗜铬细胞瘤均为双侧性，除父亲的肿瘤有肝脏转移外，均局限于肾上腺。与最近关于家族性嗜铬细胞瘤的报道不同，该家族没有多发性内分泌腺瘤病的证据。仅最年轻的患者进行了双侧肾上腺切除术，尽管血压正常，但她需要密切观察并进行类固醇替代治疗。三名患者在切除双侧肿瘤后接受了有争议的部分肾上腺切除术。父亲已持续高血压8年，并有肝脏残留肿瘤的证据。接受次全肾上腺切除术的两名女儿血压仍正常，无需用药，术后7年和3年尿儿茶酚胺值正常。这些结果表明，对于没有多发性内分泌腺瘤病的患者，不进行双侧肾上腺全切而切除双侧嗜铬细胞瘤可能使肾上腺功能在数年内保持正常且无复发。
