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[胸腺瘤和胸腺癌——基于波兰儿童实体肿瘤研究组经验的文献综述及临床特征]

[Thymoma and thymic carcinoma--review of literature and clinical characteristics based on the Polish Paediatric Solid Tumours Study Group experience].

作者信息

Stachowicz-Stencel Teresa, Bień Ewa, Balcerska Anna, Godziński Jan, Madziara Wojciech, Perek-Polnik Marta, Peregud-Pogorzelski Jarosław, Pietras Wojciech, Pobudejska Aneta, Kurylak Andrzej, Mańkowski Przemysław

机构信息

Ośrodki Onkologii i Chirurgii Dzieciecej PPGGL z Gdańska, Poland.

出版信息

Med Wieku Rozwoj. 2007 Jul-Sep;11(3 Pt 2):313-8.

Abstract

Thymomas and thymic carcinomas are rare neoplasms derived from the epithelial tissue of thymus, very infrequently developing in young adults and children. The estimation of thymomas' invasiveness has been the matter of discussion for many years reflected by numerous clinical and histological classifications. In 1999 the WHO classification was created, joining all the most important issues present in previously used systems. It is believed that histological structure is the most important prognostic factor in thymic carcinomas while in less aggressive types of thymomas the clinical stages influence the outcome. Staging of thymomas is most commonly based on the Masoka classification. Independent evaluation of the stage and histological aggressiveness are necessary to predict the clinical course and outcome in thymomas. Thus the term 'malignant thymoma' has been replaced by 'invasive thymoma' in clinical practice. The treatment strategy depends on the clinical stages of thymoma. Complete resection of the tumour is the treatment of choice with supplementing radiotherapy in more advanced clinical stages. Chemotherapy in invasive thymomas has been reported to play an increasingly important role as induction, supplementing and palliative therapy. It has been proved that combined treatment improves the outcome in invasive thymomas, especially in thymic carcinomas. This paper reviews the literature data concerning the histology, clinical issues and treatment of thymomas and thymic carcinomas. The clinical data on nine children with thymic carcinomas treated between 1992 and 2006 in the Polish oncological and surgical centres were also analysed and presented. Based on multicentre data we were able to conclude the following: 1. Thymic carcinomas in children are very rare and that is why early diagnosis is often difficult. 2. At diagnosis most cases are already inoperable, which results in poorer prognosis. 3. Complex adjuvant chemo- and radiotherapy in childhood thymic carcinomas seem to prolong overall survival. 4. Further detailed analysis in all the cases of thymic carcinomas in children is recommended in order to estimate the optimal strategy of treatment.

摘要

胸腺瘤和胸腺癌是源自胸腺上皮组织的罕见肿瘤,在年轻人和儿童中极少发生。多年来,关于胸腺瘤侵袭性的评估一直是讨论的焦点,众多临床和组织学分类都反映了这一点。1999年创建了世界卫生组织(WHO)分类,纳入了先前使用系统中所有最重要的问题。人们认为,组织学结构是胸腺癌最重要的预后因素,而在侵袭性较低的胸腺瘤类型中,临床分期影响预后。胸腺瘤的分期最常基于马索卡(Masoka)分类。独立评估分期和组织学侵袭性对于预测胸腺瘤的临床病程和预后至关重要。因此,在临床实践中,“恶性胸腺瘤”一词已被“侵袭性胸腺瘤”所取代。治疗策略取决于胸腺瘤的临床分期。肿瘤的完整切除是首选治疗方法,在更晚期的临床阶段需辅以放疗。据报道,化疗在侵袭性胸腺瘤的诱导、辅助和姑息治疗中发挥着越来越重要的作用。事实证明,联合治疗可改善侵袭性胸腺瘤的预后,尤其是在胸腺癌中。本文回顾了有关胸腺瘤和胸腺癌的组织学、临床问题及治疗的文献数据。还分析并呈现了1992年至2006年期间在波兰肿瘤学和外科中心接受治疗的9例儿童胸腺癌的临床数据。基于多中心数据,我们能够得出以下结论:1. 儿童胸腺癌非常罕见,因此早期诊断往往困难。2. 在诊断时,大多数病例已无法手术,这导致预后较差。3. 儿童胸腺癌的综合辅助化疗和放疗似乎可延长总生存期。4. 建议对所有儿童胸腺癌病例进行进一步详细分析,以评估最佳治疗策略。

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