Pulmonary carcinoid tumors with Cushing's syndrome: An aggressive variant or not?

Pulmonary carcinoid tumors with Cushing's syndrome: An aggressive variant or not?
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DOI:
10.1016/j.athoracsur.2004.07.021
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发表时间:
2005-04-01
影响因子:
4.6
通讯作者:
Pairolero, PC
Pairolero, PC
中科院分区:
医学2区
文献类型:
--
作者:
Deb, SJ;Nichols, FC;Pairolero, PC

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背景分泌促肾上腺皮质激素(ACTH)的肺类癌被认为是类癌的一种侵袭性变体。目前的知识是基于有限数量的报告和少数患者。方法。本文回顾了1966年11月至1998年4月在我院行肺切除术的肺类癌所致的库欣综合征患者。研究组由10名男性和13名女性组成。中位年龄为39岁(范围:14-71岁)。4例患者出现肺部症状。胸部X线检查发现13例患者(57%)异常,胸部计算机断层扫描(CT)发现所有20例检查患者异常。肺切除术前,垂体切除术和双侧肾上腺切除术各7例(30%)。从发病到肺切除术的中位时间间隔为17个月(范围:1-228)。16例患者行肺叶切除术,4例患者行肺段切除术,各1例患者行双叶切除术、全肺切除术和楔形切除术。没有手术死亡。21例患者(91%)被确定为典型类癌,2例患者(9%)被确定为非典型类癌。中位肿瘤直径为1.3 cm(范围:0.3-10)。19例(83%)行纵隔淋巴结清扫,6例(32%)发生淋巴结转移(N1 4例,N2 2 2例)。中位随访时间为78个月(范围:1-432)。所有患者的CS均消退。CS合并PC复发4例,单纯CS复发1例。2例患者接受了根治性再切除术。2例患者(1例播散性PC)在末次随访时死亡。尽管手术治疗延迟时间较长,但肺切除术治疗ACTH分泌型PC预后良好。纵隔淋巴结清扫联合解剖切除术可减少局部复发。虽然罕见,但这些肿瘤似乎并不像以前报道的那样具有侵袭性,是典型类癌的一种变体。(c)2005年,美国胸外科医师协会(Society of Thoracic Surgeons)
Background. Adrenocorticotropic hormone (ACTH)secreting pulmonary carcinoid is considered an aggressive variant of carcinoid tumors. Current knowledge is based upon a limited number of reports with few patients.Methods. All patients with Cushing's syndrome (CS) resulting from pulmonary carcinoid (PC) who underwent pulmonary resection at our institution from November 1966 through April 1998 were reviewed.Results. The group studied consisted of 10 males and 13 females. The median age was 39 years (range: 14-71). Pulmonary symptoms were present in 4 patients. Chest radiographs identified an abnormality in 13 patients (57%) and chest computerized tomography (CT) identified an abnormality in all 20 patients examined. Before pulmonary resection, hypophysectomy and bilateral adrenalectomy were performed in 7 patients (30%) each. Median time interval from presentation to pulmonary resection was 17 months (range: 1-228). Lobectomy was performed in 16 patients, segmentectomy was performed in 4 patients, and bilobectomy, pneumonectomy, and wedge excision was performed in 1 patient each. There were no operative deaths. Typical carcinoid was identified in 21 patients (91%) and atypical carcinoid was identified in 2 patients (9%). The median tumor diameter was 1.3 cm (range: 0.3-10). Nineteen patients (83%) underwent mediastinal lymphadenectomy and lymph node metastasis was found in 6 patients (32%) (N1 in 4 patients, N2 in 2 patients). The median follow-up was 78 months (range: 1-432). CS resolved in all of the patients. CS with PC recurred in 4 patients and CS alone recurred in 1 patient. Two patients underwent curative re-resection. Two patients, one with disseminated PC, died at last follow-up.Conclusions. Despite long delays in surgical therapy, pulmonary resection for ACTH-secreting PCs results in a favorable prognosis. Anatomic resection with complete mediastinal lymphadenectomy decreases local recurrence. Although rare these tumors do not seem to be as aggressive a variant of typical carcinoid tumors as previously reported. (c) 2005 by The Society of Thoracic Surgeons.