多发性内分泌腺瘤病中的嗜铬细胞瘤
Pheochromocytoma in multiple endocrine neoplasia.
作者信息
Raue F, Frank K, Meybier H, Ziegler R
出版信息
Cardiology. 1985;72 Suppl 1:147-9. doi: 10.1159/000173962.
Three patients with a surgically confirmed and 1 with suspected pheochromocytoma as part of the syndrome of multiple endocrine neoplasia (MEN) type II are presented. Pheochromocytoma and medullary thyroid carcinoma (MTC) as well as other tumors are part of this heritable entity. Three cases were members of a MEN IIa family and 1 patient has suffered from a sporadic form of MEN IIb. Pheochromocytoma was diagnosed by screening procedures (catecholamine screening, computerized tomography, sonography or selective venous catheterization). In 2 of the patients there was no clinical manifestation of the pheochromocytoma. On three occasions MTC was diagnosed first, the thyroid tumor occurred prior to the pheochromocytoma by 7, 6, and 1/2 years. Pheochromocytoma occurred bilaterally in 2 patients, in 1 it is suspected in both adrenals.
本文报告了3例经手术确诊的以及1例疑似患有嗜铬细胞瘤的患者，这些患者均为Ⅱ型多发性内分泌腺瘤病（MEN）综合征的一部分。嗜铬细胞瘤、甲状腺髓样癌（MTC）以及其他肿瘤均属于这一遗传性疾病范畴。3例为MEN Ⅱa家族成员，1例患有散发型MEN Ⅱb。嗜铬细胞瘤通过筛查程序（儿茶酚胺筛查、计算机断层扫描、超声检查或选择性静脉插管）得以诊断。2例患者的嗜铬细胞瘤无临床表现。有3次是先诊断出MTC，甲状腺肿瘤比嗜铬细胞瘤分别早出现7年、6年和半年。2例患者的嗜铬细胞瘤为双侧性，1例双侧肾上腺均疑似患病。
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