Cystic Primary Pulmonary Synovial Sarcoma Presenting as Recurrent Pneumothorax: Report of 4 Cases

Abstract

Pleuropulmonary synovial sarcoma is a rare malignancy that often presents like any other thoracic tumor with symptoms such as chest pain or cough. Here we describe 4 young adults who underwent surgery for apparently benign recurrent pneumothoraces and who, unexpectedly, were found upon histologic and molecular examination of the resection specimen to have cystic primary pleuropulmonary synovial sarcoma. These cases highlight (a) the importance of cytogenetic analysis in making the diagnosis, as confusion with other spindle cell sarcomas or cystic neoplasms can occur and (b) the importance of thorough examination of all resected tissue in cases of recurrent pneumothorax.

DICER1-Pleuropulmonary Blastoma Familial Tumor Predisposition Syndrome

“…Primitive, monomorphous spindle cell sarcomas in older children and young adults should raise the possibility of a primary pleuropulmonary synovial sarcoma in a patient without a known prior history of synovial sarcoma. 37 PPBs are typically more heterogeneous than synovial sarcomas, but the spindle cell components of synovial sarcoma and PPB are remarkably similar. Staining for cytokeratin and epithelial membrane antigen and/or identifying gene fusions typical of synovial sarcoma should readily differentiate synovial sarcoma from PPB.…”

“…Staining for cytokeratin and epithelial membrane antigen and/or identifying gene fusions typical of synovial sarcoma should readily differentiate synovial sarcoma from PPB. 37 DICER1 somatic mutation testing may also be of benefit in this circumstance. Finally pulmonary blastoma differs from solid PPB in that it is a biphasic tumor with malignant epithelial elements and mesenchyme and has a wide age range.…”

Spontaneous pneumothorax from cryptococcal pneumonia in systemic sclerosis: a case report

“…Spontaneous pneumothorax can be a complication of infection from invasive necrotizing organisms such as anaerobic bacteria [5], Staphylococcus [6], Klebsiella pneumoniae [7], tuberculosis [8,9], aspergillosis [10,11], Pneumocystis jiroveci [12], Scedosporium apiospermum mycetoma [13] or be associated with pre-existing lung disease such as chronic obstructive pulmonary disease [5], status asthmaticus [5], cystic fibrosis [5], cancer [14], thoracic endometriosis [15] or connective tissue disease [5]. All of the above would be termed secondary spontaneous pneumothorax (SSP).…”

In Reply

“…We are grateful to Drs Askin and Argani for their interest in our recent study, Cystic Primary Pulmonary Synovial Sarcoma Presenting as Recurrent Pneumothorax: Report of 4 Cases, 3 and for bringing to our attention their previous case report, 2 which describes a very similar patient.…”