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Primary tracheal synovial sarcoma: a rare clinical entity with diagnostic challenges
Navin Kumar1, Seema Kaushal2, Kanaklata Kanaklata3
1Department of Surgical Oncology, Institute Rotary Cancer Hospital, All India Institute of Medical Sciences (AIIMS), F 23, Ansari Nagar (West), New Delhi, 110029, India.
Summary
Tracheal synovial sarcoma (SS) is a rare tumor. Diagnosis requires genetic testing and immunohistochemistry, with surgical resection as the primary treatment for this challenging condition.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Primary tracheal tumors are exceptionally rare.
- Tracheal synovial sarcoma (SS) represents an extremely infrequent subtype.
- Accurate diagnosis relies on immunohistochemistry and chromosomal translocation studies.
Observation:
- A case of tracheal SS presented with stridor and chronic cough.
- Histological examination revealed potential mimicry with Ewing's sarcoma.
- Immunohistochemistry confirmed positivity for cytokeratin, epithelial membrane antigen, vimentin, and S100.
Findings:
- The characteristic chromosomal translocation t(X;18)(p11;q11) is a definitive diagnostic marker for SS.
- Margin-free surgical resection is the established gold standard for treatment.
- Diagnosis is often challenging due to rarity and overlapping symptoms with other tracheal pathologies.
Implications:
- Multidisciplinary team approach is crucial for effective diagnosis and management.
- Tumor coring can optimize lung perfusion prior to surgical resection.
- Long-term follow-up is essential for patients diagnosed with tracheal SS.
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