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mediastinal synovial sarcoma

Monday 26 March 2018

Definition: Synovial sarcoma (SS) is a translocation-associated sarcoma characterized by SS18-SSX1/2 fusion. It presents most often in the extremities of young adults. While SS regularly occurs in the pleuropulmonary parenchyma, the mediastinum is an exceedingly rare primary site.

Mediastinal SS is a rare and aggressive malignancy most common in patients younger than 50 years. Most are monophasic and reach large size before detection. Poorly differentiated morphology is common.

SS should be included in the differential diagnosis of spindle cell, biphasic and poorly differentiated mediastinal tumors. Because of the rarity of SS at this site, molecular testing is recommended to confirm the diagnosis.

Paywall references

- Mediastinal Synovial Sarcoma: Clinicopathologic Analysis of 21 Cases With Molecular Confirmation. B S P Terra S, Aesif SW, Maleszewski JJ, Folpe AL, Boland JM. Am J Surg Pathol. 2018 Mar 14. doi : 10.1097/PAS.0000000000001050 PMID: 29543673