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分泌促肾上腺皮质激素的甲状腺髓样癌:病例系列

ACTH-secreting medullary thyroid cancer: a case series.

作者信息

Wijewardene Ayanthi A, Glastras Sarah J, Learoyd Diana L, Robinson Bruce G, Tsang Venessa H M

机构信息

Departments of Medicine.

Departments of Endocrinology, Diabetes & Metabolism, Royal North Shore Hospital, SydneyAustralia.

出版信息

Endocrinol Diabetes Metab Case Rep. 2017 May 17;2017. doi: 10.1530/EDM-16-0110. eCollection 2017.

Abstract

UNLABELLED

Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour that originates from the parafollicular cells of the thyroid gland. The most common presentation of MTC is with a single nodule; however, by the time of diagnosis, most have spread to the surrounding cervical lymph nodes. Cushing's syndrome is a rare complication of MTC and is due to ectopic adrenocorticotrophic hormone (ACTH) secretion by tumour cells. Cushing's syndrome presents a challenging diagnostic and management issue in patients with MTC. Tyrosine kinase inhibitors (TKI) previously used for the management of metastatic MTC have become an important therapeutic option for the management of ectopic ACTH in metastatic MTC. The article describes three cases of ectopic ACTH secretion in MTC and addresses the significant diagnostic and management challenges related to Cushing's syndrome in metastatic MTC.

LEARNING POINTS

Medullary thyroid cancer (MTC) is a rare neuroendocrine tumour.Cushing's syndrome is a rare complication of MTC that has a significant impact on patients' morbidity and mortality.Tyrosine kinase inhibitors (TKI) provide an important therapeutic option for the management of ectopic ACTH in metastatic MTC.

摘要

未标注

甲状腺髓样癌(MTC)是一种罕见的神经内分泌肿瘤,起源于甲状腺的滤泡旁细胞。MTC最常见的表现是单个结节;然而,在诊断时,大多数已经扩散到周围的颈部淋巴结。库欣综合征是MTC的一种罕见并发症,是由于肿瘤细胞异位分泌促肾上腺皮质激素(ACTH)所致。库欣综合征在MTC患者中呈现出具有挑战性的诊断和管理问题。先前用于治疗转移性MTC的酪氨酸激酶抑制剂(TKI)已成为治疗转移性MTC中异位ACTH的重要治疗选择。本文描述了3例MTC中异位ACTH分泌的病例,并探讨了与转移性MTC中库欣综合征相关的重大诊断和管理挑战。

学习要点

甲状腺髓样癌(MTC)是一种罕见的神经内分泌肿瘤。库欣综合征是MTC的一种罕见并发症,对患者的发病率和死亡率有重大影响。酪氨酸激酶抑制剂(TKI)为治疗转移性MTC中的异位ACTH提供了重要的治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0357/5445425/68a7cf0714b1/edmcr-2017-160110-g001.jpg

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