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胸腺癌:单中心 37 例临床病理研究。

Thymic carcinomas: clinicopathologic study of 37 cases from a single institution.

机构信息

Department of Pathology, Centre Chirurgical Marie Lannelongue, 133 avenue de la Résistance, Le Plessis Robinson, 92350 Paris, France.

出版信息

Virchows Arch. 2013 Mar;462(3):307-13. doi: 10.1007/s00428-013-1371-y. Epub 2013 Jan 15.

Abstract

Thymic carcinomas represent the rarest and the more aggressive form of thymic epithelial tumors. We retrospectively reviewed a series of 37 consecutive cases seen in our hospital over a 15-year period. The patient group consisted of 14 female and 23 male patients, aged 31 to 80 years (mean = 57). Nineteen patients were smokers (mean 29 PY). Two nonsmokers had undergone radiotherapy for breast cancer, respectively, 9 and 15 years earlier. Twenty-four cases were squamous cell carcinomas (SCCs) expressing CD5 (90 %) and CD117 (87 %) and displaying a c-Kit mutation (n = 3). Ten cases were atypical carcinoids, including four associated with MEN1 and three others with Cushing syndrome. Three cases were undifferentiated large cell carcinomas including one associated with a type A thymoma. Twenty-seven patients had undergone a total (n = 25) or subtotal (n = 2), often extended resection. The overall survival (OS) rate was 66.6 % at 36 months, and median OS was 94 months. Carcinoid tumors (P = 0.007), surgical resection (P = 0.009), and Masaoka-Koga stage II (P = 0.049) were significantly associated with better OS. The TNM and three-grade staging systems were also significantly associated with survival but were not superior to the Masaoka-Koga system. Mediastinal lymph node recurrences treatable by reoperation and pulmonary metastases were the most frequent events in carcinoid tumors and SCCs, respectively. In conclusion, our case series suggests that smoking and radiation might constitute previously unrecognized risk factors. It confirms that SCCs express both CD5 and CD117 and possibly a c-Kit mutation. Lymph node dissection should be systematic when resection is performed, especially for carcinoid tumors.

摘要

胸腺癌是胸腺上皮肿瘤中最罕见和侵袭性最强的类型。我们回顾性分析了我院 15 年间连续收治的 37 例患者。患者组包括 14 名女性和 23 名男性,年龄 31-80 岁(平均 57 岁)。19 名患者为吸烟者(平均吸烟 29 包年)。2 名不吸烟者分别在 9 年前和 15 年前因乳腺癌接受过放疗。24 例为鳞状细胞癌(SCCs),表达 CD5(90%)和 CD117(87%),并存在 c-Kit 突变(n=3)。10 例为非典型类癌,其中 4 例与 MEN1 相关,3 例与库欣综合征相关。3 例为未分化的大细胞癌,其中 1 例与 A 型胸腺瘤相关。27 例患者接受了全(n=25)或次全(n=2),常为广泛切除术。36 个月时总生存率(OS)为 66.6%,中位 OS 为 94 个月。类癌肿瘤(P=0.007)、手术切除(P=0.009)和 Masaoka-Koga 分期 II 期(P=0.049)与更好的 OS 显著相关。TNM 和三级分期系统也与生存显著相关,但不如 Masaoka-Koga 系统。可通过再次手术治疗的纵隔淋巴结复发和 SCCs 中的肺转移分别是类癌和 SCCs 中最常见的事件。总之,我们的病例系列表明,吸烟和辐射可能是以前未被认识到的危险因素。它证实 SCCs 同时表达 CD5 和 CD117,并且可能存在 c-Kit 突变。当进行切除时,应进行系统的淋巴结清扫,尤其是对于类癌肿瘤。

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