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). The 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.
Methods: The 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.
Results: The 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 evidence for the diagnosis of CS, and thoracic computed tomography (CT) was used to detect thymic tumors. Surgical treatment significantly 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.
Conclusions: pNECT-caused CS is a rare disease with aggressive characteristics and unclear prognosis. Early diagnosis and therapy is a challenge for clinicians. Thoracic 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.
背景与目的 胸腺原发神经内分泌癌为一种罕见疾病，部分肿瘤可以分泌促肾上腺皮质激素导致库欣综合征，手术切除肿瘤是治疗的关键。本研究回顾性总结并探讨胸腺原发神经内分泌癌所致库欣综合征临床诊断及手术治疗方法，并行预后分析，提高对此罕见病种的认识及诊疗水平。方法 回顾性分析北京协和医院1987年11月-2013年6月间收治的14例由胸腺原发神经内分泌癌导致的库欣综合征患者的临床资料。结果 14例患者，男性8例，女性6例，中位年龄29岁，中位病程4.0个月（1个月-44个月），术前多项内分泌检查及影像学检查诊断并定位胸腺病变，经手术切除后血皮质醇及血促肾上腺皮质激素均下降（P<0.01）。围手术期死亡1例。经术后综合治疗及随访，中位生存期为38个月。结论 胸腺原发神经内分泌癌所致库欣综合征为一种罕见疾病，侵袭性强，目前治疗效果欠佳。早期诊断及早期治疗对临床医师而言存在很大难度，胸部增强计算机断层扫描（computed tomography, CT）是诊断并定位的重要手段，需要多种检查手段及多学科医师共同会诊并制定以手术为主的综合诊治方案。.
背景与目的 胸腺原发神经内分泌癌为一种罕见疾病，部分肿瘤可以分泌促肾上腺皮质激素导致库欣综合征，手术切除肿瘤是治疗的关键。本研究回顾性总结并探讨胸腺原发神经内分泌癌所致库欣综合征临床诊断及手术治疗方法，并行预后分析，提高对此罕见病种的认识及诊疗水平。方法 回顾性分析北京协和医院1987年11月-2013年6月间收治的14例由胸腺原发神经内分泌癌导致的库欣综合征患者的临床资料。结果 14例患者，男性8例，女性6例，中位年龄29岁，中位病程4.0个月（1个月-44个月），术前多项内分泌检查及影像学检查诊断并定位胸腺病变，经手术切除后血皮质醇及血促肾上腺皮质激素均下降（P<0.01）。围手术期死亡1例。经术后综合治疗及随访，中位生存期为38个月。结论 胸腺原发神经内分泌癌所致库欣综合征为一种罕见疾病，侵袭性强，目前治疗效果欠佳。早期诊断及早期治疗对临床医师而言存在很大难度，胸部增强计算机断层扫描（computed tomography, CT）是诊断并定位的重要手段，需要多种检查手段及多学科医师共同会诊并制定以手术为主的综合诊治方案。.