Lips K J, Van der Sluys Veer J, Struyvenberg A, Alleman A, Leo J R, Wittebol P, Minder W H, Kooiker C J, Geerdink R A, Van Waes P F, Hackeng W H
Am J Med. 1981 May;70(5):1051-60. doi: 10.1016/0002-9343(81)90866-4.
Two kindreds with the multiple endocrine neoplasia type 2A syndrome were studied. Of one of these we examined 150 members, 20 of whom were treated with thyroidectomy for medullary carcinoma and nine with bilateral adrenalectomy for pheochromocytoma. Of the second kindred 50 members were examined, seven of whom were thyroidectomized and seven treated with bilateral adrenalectomy. Pheochromocytomas were invariably found on both sides, even in four cases in which the adrenals on one side appeared to be completely normal, not only at preoperative roentgenologic examination but also on inspection during the operation. The microscopic finding of micronodules and a cluster of abnormal medullary cells identical with those found in pheochromocytomas in one of the apparently normal adrenals represents a first stage in the development of diffuse medullary hyperplasia as well as nodular hyperplasia. This is in accordance with the fact that in the MEN type 2A syndrome pheochromocytomas are always multicentric and multiple in origin. On the basis of these findings we conclude that all patients with the MEN 2A syndrome who show symptoms and signs of active pheochromocytoma should be subjected to bilateral adrenalectomy, even when one or both of the adrenals appear to be normal at roentgenologic investigation.
对两个患有2A型多发性内分泌腺瘤综合征的家族进行了研究。在其中一个家族中，我们检查了150名成员，其中20人因髓样癌接受了甲状腺切除术，9人因嗜铬细胞瘤接受了双侧肾上腺切除术。在第二个家族中，检查了50名成员，其中7人接受了甲状腺切除术，7人接受了双侧肾上腺切除术。嗜铬细胞瘤总是双侧发生，即使在4例中，一侧肾上腺在术前X线检查及手术中检查时看起来完全正常。在其中一个看似正常的肾上腺中，显微镜下发现的微小结节和一簇与嗜铬细胞瘤中发现的异常髓样细胞相同的细胞，代表了弥漫性髓样增生以及结节性增生发展的第一阶段。这与2A型多发性内分泌腺瘤综合征中嗜铬细胞瘤总是多中心起源且多发的事实相符。基于这些发现，我们得出结论，所有出现活动性嗜铬细胞瘤症状和体征的2A型多发性内分泌腺瘤综合征患者，即使在X线检查时一侧或双侧肾上腺看起来正常，也应接受双侧肾上腺切除术。
