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肺大细胞神经内分泌癌:一种具有侵袭性、可能通过手术治疗的疾病。

Large cell neuroendocrine carcinoma of the lung: an aggressive disease potentially treatable with surgery.

作者信息

Doddoli Christophe, Barlesi Fabrice, Chetaille Bruno, Garbe Louise, Thomas Pascal, Giudicelli Roger, Fuentes Pierre

机构信息

Department of Thoracic Surgery, Université de la Méditerranée (Aix-Marseille II), Faculty of Medicine, Sainte-Marguerite Hospital, France.

出版信息

Ann Thorac Surg. 2004 Apr;77(4):1168-72. doi: 10.1016/j.athoracsur.2003.09.049.

DOI:10.1016/j.athoracsur.2003.09.049
PMID:15063228
Abstract

BACKGROUND

Assessment of clinical and pathologic features of large cell neuroendocrine carcinoma to confirm its specificity in the setting of high grade neuroendocrine pulmonary tumors.

METHODS

From 1989 to 2001, 123 patients with a neuroendocrine carcinoma were surgically treated in a curative intent at a single institution. According to the 1999 World Health Organization classification, 20 patients were reviewed as having a large cell neuroendocrine carcinoma. Clinical data as well as detailed pathologic analysis and survival were collected.

RESULTS

There were 18 men and 2 women. The median age was 62 years. Four patients had a preoperative diagnosis of large cell neuroendocrine carcinoma. The resections consisted of 14 lobectomies and 6 pneumonectomies. There was no operative death. Complications occurred in 7 patients (35%). Four patients had a stage I of the disease, 4 had stage II, 9 had stage III, and 3 had stage IV. At follow-up (median, 46 months), 13 patients died from general recurrence and 7 patients were still alive. Median time to progression was 9 months (range, 1 to 54 months). The 5-year survival rate was 36% (median, 49 months) and it seemed to be negatively influenced by the disease stage (54% for stage I-II vs 25% for stage III-IV; p = 0.07), the presence of metastatic lymph node (45% for N0/N1 vs 17% for N2; p = 0.12), or vessel invasion (66 vs 25%; p = 0.18).

CONCLUSIONS

Large cell neuroendocrine carcinoma predominantly occurred in men. An accurate tissue diagnosis was rarely obtained preoperatively. Although overall survival after resection was substantial, large cell neuroendocrine carcinoma frequently showed pathologic features of occult metastatic disease, such as lymph node or vessel invasion, or both.

摘要

背景

评估大细胞神经内分泌癌的临床和病理特征,以确认其在高级别神经内分泌性肺肿瘤中的特异性。

方法

1989年至2001年,123例神经内分泌癌患者在单一机构接受了根治性手术治疗。根据1999年世界卫生组织分类,20例患者被诊断为大细胞神经内分泌癌。收集了临床数据以及详细的病理分析和生存情况。

结果

男性18例,女性2例。中位年龄为62岁。4例患者术前诊断为大细胞神经内分泌癌。手术切除包括14例肺叶切除术和6例全肺切除术。无手术死亡。7例患者(35%)出现并发症。4例患者为疾病I期,4例为II期,9例为III期,3例为IV期。随访(中位时间46个月)时,13例患者死于全身复发,7例患者仍存活。中位进展时间为9个月(范围1至54个月)。5年生存率为36%(中位时间49个月),似乎受疾病分期(I-II期为54%,III-IV期为25%;p = 0.07)、转移淋巴结的存在(N0/N1为45%,N2为17%;p = 0.12)或血管侵犯(66%对25%;p = 0.18)的负面影响。

结论

大细胞神经内分泌癌主要发生在男性。术前很少能获得准确的组织诊断。尽管切除术后总体生存率较高,但大细胞神经内分泌癌常表现出隐匿性转移疾病的病理特征,如淋巴结或血管侵犯,或两者皆有。

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