[Primary mediastinal sarcomas].

Abstract:

INTRODUCTION:Primary sarcomas of the mediastinum are rare and account for 2 to 8% of malignant mediastinal tumours. The aim of this study is to describe their clinical and pathological characteristics. PATIENTS AND METHODS:Fifteen cases of primary mediastinal sarcoma, diagnosed between 1993 and 2009, were reviewed retrospectively, noting the clinical, radiological and pathological findings, and the treatment given. RESULTS:The patient population consisted of eight females and seven males with mean age of 40 years (14 to 73 years). The symptomatology was predominately respiratory. Imaging showed a mediastinal mass invading adjacent organs in nine cases. The diagnosis was made in all cases by histological examination. These 15 mediastinal sarcoma comprised 12 malignant peripheral nerve tumours, two liposarcomas and one angiosarcoma. Three were grade III, six grade II and six grade I. Ten were treated surgically, of which seven had radical resections. Associated treatments were neoadjuvant (one case) or adjuvant (one case) chemotherapy and postoperative radiotherapy (five cases). Radiotherapy was undertaken alone in three inoperable tumours. Eight patients (53%) had died. CONCLUSION:Management of primary mediastinal sarcoma needs a multidisciplinary approach, and is based mainly on radical resection. The prognosis is poor and depends mainly on surgical excision and histological grade.

journal_name

Rev Mal Respir

authors

Zehani A,Ayadi-Kaddour A,Daghfous H,Ridene I,Marghli A,Kilani T,El Mezni F

doi

10.1016/j.rmr.2010.05.015

subject

Has Abstract

pub_date

2011-01-01 00:00:00

pages

14-24

issue

1

eissn

0761-8425

issn

1776-2588

pii

S0761-8425(10)00560-7

journal_volume

28

pub_type

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