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原发性纵隔类癌肿瘤

Primary mediastinal carcinoid tumors.

作者信息

Wick M R, Carney J A, Bernatz P E, Brown L R

出版信息

Am J Surg Pathol. 1982 Apr;6(3):195-205. doi: 10.1097/00000478-198204000-00001.

Abstract

A study of 15 cases of carcinoid tumor of the thymus, diagnosed and treated at the Mayo Clinic, revealed histopathologic features of the neoplasm that might cause it to be confused with other mediastinal tumors, both primary and secondary. The tumor was associated with ectopic ACTH production in six patients, of whom five had Cushing's syndrome. Metastasis, which occurred in 11 patients (73%), was delayed for as long as 8 years after initial diagnosis. Eleven patients underwent surgical resection of their primary thymic neoplasms, and nine of these received postoperative radiotherapy or chemotherapy or both. One patient was treated with chemotherapy alone, and three had no treatment for their neoplasms, which were discovered at autopsy. Eight patients are still alive. Of these, five have developed metastases, and only one is free of metastatic disease more than 5 years after diagnosis. In the two remaining cases, the discovery of the thymic tumor was recent and follow-up is not yet meaningful. Overall, four of the seven patients who died had proven metastatic disease. The mean survival after the appearance of extrathymic tumor in this group was 3 years. Of the five patients who are still alive with metastasis, all have survived at least 2 years since that spread of their disease.

摘要

一项对梅奥诊所诊断和治疗的15例胸腺类癌肿瘤的研究显示,该肿瘤的组织病理学特征可能使其与其他原发性和继发性纵隔肿瘤相混淆。该肿瘤在6例患者中与异位促肾上腺皮质激素(ACTH)分泌有关,其中5例患有库欣综合征。11例患者(73%)发生转移,转移发生时间在初次诊断后长达8年。11例患者接受了原发性胸腺肿瘤的手术切除,其中9例术后接受了放疗或化疗或两者皆有。1例患者仅接受化疗,3例肿瘤在尸检时发现,未接受任何治疗。8例患者仍存活。其中,5例已发生转移,只有1例在诊断后5年以上无转移疾病。在其余2例中,胸腺肿瘤是最近发现的,随访尚无意义。总体而言,7例死亡患者中有4例已证实有转移疾病。该组出现胸腺外肿瘤后的平均生存期为3年。5例仍有转移的存活患者,自疾病转移以来均已存活至少2年。

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