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恶性胸腺瘤——葡萄牙波尔图肿瘤研究所的经验及文献综述

Malignant thymomas--the experience of the Portuguese Oncological Institute, Porto, and literature review.

作者信息

Sousa Berta, Araújo António, Amaro Teresina, Azevedo Isabel, Soares Marta, Sousa Olga

机构信息

Medical Oncology, Instituto Português de Oncologia do Porto Francisco Gentil-EPE, Rua Dr António Bernardino de Almeida, 4200-072 Porto.

出版信息

Rev Port Pneumol. 2007 Jul-Aug;13(4):553-85.

PMID:17898913
Abstract

INTRODUCTION

Epithelial thymic tumours (ETT), which comprise the majority of thymomas, are neoplasias developed from the epithelial cells of the thymus and constitute around 30% of anterior mediastinal masses in adults. Thymomas consist of cells with no cytological characteristics of malignity; malignant behaviour is determined by invasion of the capsule and adjacent structures. These tumours present a broad spectrum of clinical and morphological characteristics and the small series of known patients makes establishing a standard treatment difficult.

MATERIAL AND METHODS

A retrospective study was made into thymoma diagnosed patients admitted to the Portuguese Oncology Institute in Porto (IPO-Porto) from 1983 to 2004. Clinical characteristics were analysed and a histological classification made in accordance with World Health Organization criteria, Masaoka staging, and their relation to treatment methods. A review of the clinical records of these patients was then made, as well as a review of histological material for classification in line with 1999 WHO criteria.

RESULTS

Twenty-eight ETT patients were treated at the IPO-Porto between 1983 and 2004. Of these, 21 had invasive thymomas and these are the subject of this study. Eleven subjects were male and 10 female, with a median age of 55 years (24-79 years). The WHO histological classification was as follows: 2 patients (9.5%) type A, 6 (28.6%) type AB, 4 (19%) type B1, 2 (9.5%) type B2, 7 (33.4%) type B3. Masaoka staging was 9 patients (42.8%) with stage II, 6 (28.6%) with stage III and 6 (28.6%) with stage IVa. The majority of patients had local symptoms, with only one subject diagnosed with erythrocyte aplasia and five with Myasthenia Gravis (MG). The 6 patients who were given complete surgical resection only showed no evidence of disease recurrence (2 type A-II, 2 type AB-II, 1 type B1-II, 1 type B2- IVa), with follow-up from 8-144 months. Ten patients with complete resection received adjuvant treatment; 6 radiotherapy (4 B3-II patients, 2 B3-III patients), 2 chemotherapy (AB-IVa) and 2 chemo and radiotherapy (B1-IVa, B2-III). Only the 2 patients who underwent adjuvant chemotherapy relapsed, at 168 and 46 months, dying at 168 and 49 months, respectively. The remaining patients who were given adjuvant treatment did not present signs of disease. Of the 5 subjects having incomplete resection followed by complementary treatment (2 AB-III patients, 2 B1-IVa patients, 1 B3-III patient), 3 died, at 11 months (B3-III), 12 months (B1-IVa) and 241 months (AB-III), the latter with MG.

CONCLUSIONS

Predictive factors of bad prognosis here were incomplete resection, advanced staging and B3 histological subtype, the smallness of this series notwithstanding. It is necessary to investigate the role of adjuvant and neoadjuvant treatment in a group of subjects with advanced disease of the B3 histological subtype.

摘要

引言

上皮性胸腺瘤(ETT)占胸腺瘤的大多数,是起源于胸腺上皮细胞的肿瘤,约占成人前纵隔肿块的30%。胸腺瘤由不具有恶性细胞学特征的细胞组成;恶性行为由包膜及相邻结构的侵犯来判定。这些肿瘤呈现出广泛的临床和形态学特征,且已知患者数量较少,难以确立标准治疗方案。

材料与方法

对1983年至2004年入住波尔图葡萄牙肿瘤研究所(IPO - 波尔图)的胸腺瘤确诊患者进行回顾性研究。分析临床特征,并根据世界卫生组织标准进行组织学分类、马萨oka分期以及它们与治疗方法的关系。随后查阅这些患者的临床记录,并根据1999年世界卫生组织标准对组织学材料进行分类回顾。

结果

1983年至2004年期间,28例ETT患者在IPO - 波尔图接受治疗。其中,21例为浸润性胸腺瘤,本研究以这些患者为对象。11例为男性,10例为女性,中位年龄55岁(24 - 79岁)。世界卫生组织组织学分类如下:A型2例(9.5%),AB型6例(28.6%),B1型4例(19%),B2型2例(9.5%),B3型7例(33.4%)。马萨oka分期为II期9例(42.8%),III期6例(28.6%),IVa期6例(28.6%)。大多数患者有局部症状,仅1例诊断为红细胞再生障碍,5例诊断为重症肌无力(MG)。6例接受完整手术切除的患者未出现疾病复发迹象(2例A型 - II期,2例AB型 - II期,1例B1型 - II期,1例B2型 - IVa期),随访时间为8 - 144个月。10例接受完整切除的患者接受了辅助治疗;6例接受放疗(4例B3型 - II期患者,2例B3型 - III期患者),2例接受化疗(AB型 - IVa期),2例接受化疗及放疗(B1型 - IVa期,B2型 - III期)。仅2例接受辅助化疗的患者复发,分别在168个月和46个月复发,于168个月和49个月死亡。其余接受辅助治疗的患者未出现疾病迹象。5例接受不完全切除并辅以补充治疗的患者(2例AB型 - III期患者,2例B1型 - IVa期患者,1例B3型 - III期患者)中,3例死亡,分别于11个月(B3型 - III期)、12个月(B1型 - IVa期)和241个月(AB型 - III期)死亡,后者患有MG。

结论

尽管本研究样本量小,但预后不良的预测因素为不完全切除、晚期分期和B3组织学亚型。有必要在一组B3组织学亚型的晚期疾病患者中研究辅助治疗和新辅助治疗的作用。

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