Primary rectal monophasic synovial sarcoma

Junlin Zhang; Sarah K Findeis; Benjamin J Lang; Gerald O Ogola; Atin Agarwal

doi:10.1080/08998280.2021.1902191

Primary rectal monophasic synovial sarcoma

Proc (Bayl Univ Med Cent). 2021 Apr 6;34(4):512-516. doi: 10.1080/08998280.2021.1902191.

Authors

Junlin Zhang¹, Sarah K Findeis¹, Benjamin J Lang¹, Gerald O Ogola², Atin Agarwal¹

Affiliations

¹ Department of Pathology, Baylor University Medical Center, Dallas, Texas.
² Baylor Research Institute, Baylor Scott & White Health, Dallas, Texas.

Abstract

Synovial sarcoma is a rare malignant mesenchymal neoplasm that often occurs in the extremities. Less than 70 cases of primary synovial sarcoma occurring in the digestive system have been reported. We present a case of a 48-year-old woman with a spindle cell tumor in the rectum that stained positive for AE1/3 (focal), vimentin, CD99, BCL2, EMA (focal), and MiB-1 (15%). Ultimately, the lesion was diagnosed as a primary rectal monophasic synovial sarcoma and confirmed by molecular testing for SYT/SSX1 gene fusion. Analysis of previous publications indicated that patients of advanced age or a large tumor size (≥5 cm) have a higher risk of progressing rapidly to death after diagnosis of synovial sarcoma in the digestive system.

Keywords: Gastrointestinal tract; SYT/SSX1; monophasic; rectum; synovial sarcoma.

Publication types

Case Reports