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类固醇细胞卵巢肿瘤伴症状性库欣综合征和高雄激素血症:一例报告

Symptomatic Cushing's syndrome and hyperandrogenemia in a steroid cell ovarian neoplasm: a case report.

作者信息

Sedhom Ramy, Hu Sophia, Ohri Anupam, Infantino Dorian, Lubitz Sara

机构信息

Department of Internal Medicine, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.

Division of Endocrinology, Metabolism & Nutrition, Department of Medicine, Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.

出版信息

J Med Case Rep. 2016 Oct 12;10(1):278. doi: 10.1186/s13256-016-1061-x.

DOI:10.1186/s13256-016-1061-x
PMID:27729065
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5059940/
Abstract

BACKGROUND

Malignant steroid cell tumors of the ovary are rare and frequently associated with hormonal abnormalities. There are no guidelines on how to treat rapidly progressive Cushing's syndrome, a medical emergency.

CASE PRESENTATION

A 67-year-old white woman presented to our hospital with rapidly developing signs and symptoms of Cushing's syndrome secondary to a steroid-secreting tumor. Her physical and biochemical manifestations of Cushing's syndrome progressed, and she was not amenable to undergoing conventional chemotherapy secondary to the debilitating effects of high cortisol. Her rapidly progressive Cushing's syndrome ultimately led to her death, despite aggressive medical management with spironolactone, ketoconazole, mitotane, and mifepristone.

CONCLUSIONS

We report an unusual and rare case of Cushing's syndrome secondary to a malignant steroid cell tumor of the ovary. The case is highlighted to discuss the complications of rapidly progressive Cushing's syndrome, an underreported and often unrecognized endocrine emergency, and the best available evidence for treatment.

摘要

背景

卵巢恶性类固醇细胞瘤罕见,常伴有激素异常。对于如何治疗快速进展的库欣综合征这一医疗急症,尚无相关指南。

病例介绍

一名67岁白人女性因分泌类固醇的肿瘤导致库欣综合征的体征和症状迅速发展,前来我院就诊。她库欣综合征的身体和生化表现不断进展,由于高皮质醇的衰弱作用,她无法接受传统化疗。尽管使用螺内酯、酮康唑、米托坦和米非司酮进行了积极的药物治疗,她快速进展的库欣综合征最终还是导致了死亡。

结论

我们报告了一例罕见的由卵巢恶性类固醇细胞瘤继发的库欣综合征病例。强调该病例是为了讨论快速进展的库欣综合征的并发症,这是一种报道不足且常未被认识的内分泌急症,以及现有的最佳治疗证据。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d91/5059940/8ace15a819c4/13256_2016_1061_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d91/5059940/23ad1271c1f9/13256_2016_1061_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d91/5059940/84b4604601fb/13256_2016_1061_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d91/5059940/2a4bec5fbb74/13256_2016_1061_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d91/5059940/8ace15a819c4/13256_2016_1061_Fig4_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d91/5059940/23ad1271c1f9/13256_2016_1061_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d91/5059940/84b4604601fb/13256_2016_1061_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d91/5059940/2a4bec5fbb74/13256_2016_1061_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8d91/5059940/8ace15a819c4/13256_2016_1061_Fig4_HTML.jpg

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