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Primary rectal monophasic synovial sarcoma.

Junlin Zhang1, Sarah K Findeis1, Benjamin J Lang1

  • 1Department of Pathology, Baylor University Medical Center, Dallas, Texas.

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|July 5, 2021
PubMed
Summary
This summary is machine-generated.

This study reports a rare case of primary rectal monophasic synovial sarcoma in a 48-year-old woman. Advanced age and large tumor size are linked to poor prognosis in digestive system synovial sarcoma.

Keywords:
Gastrointestinal tractSYT/SSX1monophasicrectumsynovial sarcoma

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Area of Science:

  • Oncology
  • Gastroenterology
  • Pathology

Background:

  • Synovial sarcoma is a rare malignant mesenchymal neoplasm, typically affecting extremities.
  • Primary synovial sarcoma in the digestive system is exceptionally rare, with fewer than 70 reported cases.
  • Rectal tumors require careful differential diagnosis, including rare mesenchymal neoplasms.

Observation:

  • A 48-year-old woman presented with a rectal spindle cell tumor.
  • Immunohistochemical analysis revealed positivity for AE1/3 (focal), vimentin, CD99, BCL2, EMA (focal), and MiB-1 (15%).
  • Molecular testing confirmed the presence of the SYT/SSX1 gene fusion, characteristic of synovial sarcoma.

Findings:

  • The rectal lesion was diagnosed as a primary monophasic synovial sarcoma.
  • The tumor exhibited specific immunohistochemical markers and a confirmed SYT/SSX1 gene fusion.
  • Literature review suggests advanced age and tumor size (≥5 cm) correlate with rapid progression in digestive system synovial sarcoma.

Implications:

  • This case expands the understanding of rare gastrointestinal malignancies.
  • Early diagnosis and molecular confirmation are crucial for accurate staging and treatment planning.
  • Identifying prognostic factors like age and tumor size is vital for risk stratification and patient management in digestive synovial sarcoma.