Primary pleuropulmonary synovial sarcoma mimicking a carcinoid tumor: Case report and literature review

Abstract

Primary pleuropulmonary synovial sarcoma is a rare malignancy. Commonly described radiologic features in the literature include pleural disease and/or effusion, lack of calcification and high uptake on positron emission tomography computerised tomography. A 68-year-old woman presented with a 3-month history of cough. Imaging studies showed a right upper lobe mass with internal foci of calcification, endobronchial extension, and low fluorodeoxyglucose avidity on positron emission tomography computerised tomography, leading to an initial diagnosis of carcinoid tumor. However, histologic specimens suggested an unexpected diagnosis of aggressive synovial sarcoma, and the case was referred to the sarcoma MDT. Metastatic synovial sarcoma was ruled out, and radical surgical excision of the lesion was performed. This article highlights the multiple atypical features of primary pleuropulmonary synovial sarcoma as seen in this case and reviews imaging findings described in the literature. Radiologists should be aware of this unusual yet aggressive type of sarcoma.

Primary Pulmonary Synovial Sarcoma in a 49-Year-Old Male

“…There is no predilection for the right or left side of the lung, but it is most commonly seen in the apex of the lungs [ 3 ]. Smoking is not a predisposing factor in the development of the tumor [ 4 ]. Primary pulmonary synovial sarcomas grow slowly and insidiously, which can often lead to delayed diagnosis and therapy.…”

“…Clinical features and presentation of intrapulmonary tumors are mostly based on tumor location. The most common presenting symptoms are chest pain, cough, dyspnea, and occasionally hemoptysis [ 4 ]. The tumor tends to be large and, at times, can cause a partial or complete opacification of the lungs.…”

An unusual endobronchial lesion: expanding the differential diagnosis

“…Seven such reported cases are summarized in Table . These cases predominately involved female patients, who presented with symptoms of cough and haemoptysis . All cases were treated with surgical resection, and most needed no additional treatment with chemotherapy or radiation .…”

“…These cases predominately involved female patients, who presented with symptoms of cough and haemoptysis [9,[11][12][13][14][15][16]. All cases were treated with surgical resection, and most needed no additional treatment with chemotherapy or radiation [11,12,[14][15][16]. These cases had similar outcomes to other cases of synovial sarcoma without endobronchial involvement, with two cases having disease recurrence years after their resection.…”

Primary Cystic Pleuropulmonary Synovial Sarcoma Presenting as Recurrent Pneumothorax

“…Entirely cystic/bullous PPSS presenting with recurrent pneumothorax have been rarely reported [ 1 ]. These lesions may be confused with benign cystic disease, type 1 pleuropulmonary blastoma, mesenchymal cystic hamartoma, lymphangioleiomyomatosis, and metastatic endometrial stromal sarcoma [ 3 , 4 , 8 ]. Before clinical FISH testing became readily available, many of the earlier cases with this histomorphology were likely reported as other entities such as mesenchymal cystic hamartoma [ 1 , 3 ].…”