Primary Cystic Pleuropulmonary Synovial Sarcoma Presenting as Recurrent Pneumothorax

Abstract

Primary pleuropulmonary synovial sarcomas are quite rare, representing 0.1–0.5% of all pulmonary malignancies. We report an entirely cystic monophasic synovial sarcoma in a 25-year-old male who presented with recurrent pneumothorax and no evidence of a mass lesion on imaging. The purpose of this case report is to increase awareness of neoplasms clinically presenting as a pneumothorax with no imagining evidence of a mass-forming lesion and emphasize the significance of fluorescent in situ hybridization testing in nontypical synovial sarcoma cases.

Pediatric pleuropulmonary synovial sarcoma: A case report in a recurrent spontaneous pneumothorax

“…PPSS masses often lack calcification or associated lymphadenopathy. Recurrent pneumothorax has also been reported in few case reports [ 4 , 5 ]. However, most of these patients were part of the typical demographic of young adults or middle aged adults.…”

“…However, most of these patients were part of the typical demographic of young adults or middle aged adults. The youngest patient described in the literature is a fourteen-year-old female with similar presentation [ 4 ]. Our patient is well below the expected age of presentation and exhibits atypical clinical features of the malignancy.…”

Persistent spontaneous pneumothorax as a primary manifestation of primary synovial sarcoma of the lung: a case report

“…Such lesion characteristics warrant a differential diagnosis with benign cysts, type 1 pleuropulmonary blastoma, mesenchymal cystic hamartoma, and lymphangioleiomyomatosis. 6,7 Diagnostic suspicion is confirmed by a pathological study of the pleuropulmonary tissue. Histopathology classifies it into monophasic or biphasic.…”

“…6,8 PPSS manifests most frequently as an asymptomatic lung mass or with chest pain, cough, dyspnea, and even hemothorax. 7 On chest X-ray, it is seen as a well-defined, rounded-edged lung or pleural mass usually accompanied by ipsilateral pleural effusion. On HRCT of the chest, it is seen as a mass with heterogeneous enhancement and well-delimited borders, without bone involvement or calcifications inside.…”

“…4,5 It usually presents as a progressively growing periarticular tumor mass in the limbs. 6,7 If SS occurs primarily in the lung, pleuropulmonary synovial sarcoma (PPSS) has a poor prognosis and, in general, an aggressive course. 8 Occasionally, pneumothorax has a poor course and is persistent or recurrent, 9,10 which makes it necessary to perform studies to look for the underlying cause.…”