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肢端肥大症和库欣综合征与前肠类癌肿瘤相关。

Acromegaly and Cushing's syndrome associated with a foregut carcinoid tumor.

作者信息

Leveston S A, McKeel D W, Buckley P J, Deschryver K, Greider M H, Jaffe B M, Daughaday W H

出版信息

J Clin Endocrinol Metab. 1981 Oct;53(4):682-9. doi: 10.1210/jcem-53-4-682.

DOI:10.1210/jcem-53-4-682
PMID:6270170
Abstract

We report an 18-yr-old youth with a metastatic foregut carcinoid tumor, Cushing's syndrome, and hypersomatotropic gigantism. Administration of cyproheptadine caused a dramatic fall in urinary cortisol excretion and plasma ACTH levels associated with clinical remission of the Cushing's syndrome. GH secretion was not affected by cyproheptadine administration. Ectopic ACTH secretion was confirmed by RIA of tumor extracts and immunohistochemical demonstration of ACTH-containing cells in hepatic metastases. There were two sources of GH production demonstrated in this patient. Ectopic secretion of GH by the carcinoid hepatic metastases was documented by both RIA and immunohistochemical techniques. A somatotrophic pituitary tumor was also present. The histological characteristics of this tumor suggest adenomatous hyperplasia rather than de novo neoplastic change as the likely mechanism of its pathogenesis. GH releasing factor-like activity was demonstrated in extracts of plasma and in extracts of the carcinoid tumor. We conclude that cyproheptadine exerted an effect on the ectopic ACTH-producing cells but not on the ectopic GH-producing cells or on adenohypophyseal GH secretion. Production of a GH releasing factor-like activity by the carcinoid tumor may have caused the pituitary somatotrophic tumor.

摘要

我们报告了一名18岁的青年,患有转移性前肠类癌肿瘤、库欣综合征和生长激素分泌过多性巨人症。给予赛庚啶后,尿皮质醇排泄量和血浆促肾上腺皮质激素(ACTH)水平显著下降,同时库欣综合征临床缓解。赛庚啶给药未影响生长激素(GH)分泌。通过肿瘤提取物的放射免疫分析(RIA)和肝转移灶中含ACTH细胞的免疫组织化学证实了异位ACTH分泌。该患者存在两种GH产生来源。通过RIA和免疫组织化学技术均证实类癌肝转移灶异位分泌GH。同时还存在一个生长激素垂体瘤。该肿瘤的组织学特征提示腺瘤样增生而非新发肿瘤性改变是其发病机制的可能原因。在血浆提取物和类癌肿瘤提取物中均证实有生长激素释放因子样活性。我们得出结论,赛庚啶对异位产生ACTH的细胞有作用,但对异位产生GH的细胞或腺垂体GH分泌无作用。类癌肿瘤产生的生长激素释放因子样活性可能导致了垂体生长激素瘤。

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