Primary Neuroendocrine Carcinoma of Thymus Caused Cushing Syndrome：Surgical Treatment and Prognosis Analysis
10.3779/j.issn.1009-3419.2015.07.12
- VernacularTitle:胸腺原发神经内分泌癌所致库欣综合征手术治疗及预后分析
- Author:
LI LI
1
;
CHEN YEYE
;
LI SHANQING
;
LIU HONGSHENG
;
HUANG CHENG
;
QIN YINGZHI
Author Information
1. 中国医学科学院北京协和医院胸外科
- Keywords:
Neuroendocrine carcinoma;
hTymus;
Cushing syndrome;
Surgical procedures;
Prognosis
- From:
Chinese Journal of Lung Cancer
2015;(7):451-456
- CountryChina
- Language:Chinese
-
Abstract:
Background and objective Primary neuroendocrine carcinoma of thymus (pNECT) is a rare thymic neoplasm. Some pNECTs could produce an adrenocorticotropic hormone and cause Cushing syndrome (CS). hTe aim os this study is to discuss the diagnostic technique and surgical management of pNECT-caused CS and analyze prognosis factors to improve the clinical experience of the disease.MethodshTe outcome of surgery and follow-up of 14 cases (eight males and six females) of pNECT-caused CS were retrospectively analyzed from November 1987 to June 2013.ResulthTe median age of the patients was 29, and the median duration of the disease was four months (1 month-44 months). All cases exhibited clinical evi-dence for the diagnosis of CS, and thoracic computed tomography (CT) was used to detect thymic tumors. Surgical treatment signiifcantly decreased the concentration of both serum cortisol and adrenocorticotropic hormone (P<0.01) but caused one death in the perioperative period. With multidisciplinary therapy, the median survival was 38 months.Conclusion pNECT-caused CS is a rare disease with aggressive characteristics and unclear prognosis. Early diagnosis and therapy is a challenge for clinicians. hToracic CT is important for disease location and preoperative evaluation and should be routinely applied to all CS patients to allow early surgery and improved prognosis.
