Management of Primary Pulmonary Synovial Sarcoma: A Case Report from Northern Pakistan and Review of Literature- Juniper Publishers

JUNIPER PUBLISHERS- OPEN ACCESS JOURNAL OF CASE STUDIES

Management of Primary Pulmonary Synovial Sarcoma: A Case Report from Northern Pakistan and Review of Literature

Authored by Hadia Fatima

  

Abstract

Primary pulmonary synovial sarcoma is a rare lung tumor and has mesenchymal origin. It is difficult to diagnose clinically and should be differentiated from other primary and metastatic lung tumors. The definitive diagnosis should be made via careful histopathological evaluation along with immunohistochemistry. Cytogenetic studies detect SYT-SSX-1 variant which has got diagnostic and prognostic value. The disease has poor outcome and complete cure is difficult to achieve. We hereby report a case of 40 year old female who was diagnosed and treated for metastatic primary pulmonary synovial sarcoma at Atomic Energy Cancer Hospital, NORI.

Introduction

Primary pulmonary synovial sarcoma (PPSS) is an extremely unusual malignancy associated with aggressive behavior and poor clinical outcome [1]. The tumor originates from mesenchymal cells [1]. Synovial sarcomas make up to 8% of soft tissue sarcomas most commonly seen in extremities [1]. Pulmonary sarcomas are even rare making up to just 0.1-0.5% of primary lung malignancies [2]. The differential diagnosis includes metastatic sarcomas and another primary lung cancer [3]. Primary pulmonary synovial sarcoma behaves like soft tissue synovial sarcoma [4]. PPSS may arise from lung parenchyma, pleura, chest wall and mediastinum [5].

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