Adult‐Onset Primary Pleura Ewing Sarcoma With Recurrent Pleural Effusion: A Case Report and Literature Review

TL;DR

A rare case of adult-onset Ewing sarcoma in the pleura is reported, highlighting its unusual presentation with pleural effusion and aggressive progression.

Contribution

This case report adds to the limited literature on adult-onset primary pleural Ewing sarcoma with recurrent pleural effusion.

Findings

Pleural effusion is an unusual and diagnostic-challenging manifestation of Ewing sarcoma.

The disease had an aggressive clinical course despite multi-agent chemotherapy.

CT scans and thoracoscopy were essential for confirming the diagnosis.

Abstract

Extraskeletal Ewing sarcoma (EES) is a rare malignant tumour within the “Ewing family of tumours,” first described by Tefft et al. in 1969. It accounts for less than 1 case per million, typically affecting adolescents and young adults. We report a rare case of pleural EES in a 42‐year‐old woman presenting with chest pain and low‐grade fever persisting for one month. Physical examination showed decreased breath sounds in the right lung base on auscultation. Ultrasound demonstrated right‐sided pleural effusion, an unusual manifestation of EES that can hinder accurate diagnosis. CT scans identified multiple pulmonary nodules, further confirmed by thoracoscopy. Biopsy established the diagnosis of EES. The patient was subsequently treated with multi‐agent VAC/IE chemotherapy. Despite initiation of systemic therapy, the disease followed an aggressive course, and the patient succumbed during follow‐up. We also review existing literature to highlight clinical, pathological, and radiological features of pleural EES, aiming to expand knowledge of this rare presentation.

We present a case of a 42‐year‐old woman presenting with pleural effusion, a rare manifestation of Ewing sarcoma.

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