Key result
Tracheal resection and tracheoplasty successfully treated a rare primary tracheal synovial sarcoma, with the patient remaining disease-free at one year follow-up.
Why the study?
Primary tracheal tumors and specifically tracheal synovial sarcoma are extremely rare, posing diagnostic challenges due to common presenting symptoms that mimic other tracheal pathologies.
Case Report (n=1)
No
Primary tracheal synovial sarcoma is an extremely rare entity that can be successfully managed with margin-free surgical resection and accurately diagnosed using chromosomal translocation studies.
Case report of tracheal synovial sarcoma supports awareness for rare stridor etiologies; leaves optimal management open pending larger series.
BACKGROUND: The incidence of primary tracheal tumors is very low. Tracheal synovial sarcoma (SS) is even an extremely rare entity. Diagnosis of tracheal SS can be achieved with chromosomal translocation studies along with immunohistochemistry. Margin-free resection is the gold standard treatment. CASE PRESENTATION: We report a case of tracheal SS, which presented with stridor with a history of chronic cough and was diagnosed with a battery of clinical investigations and managed successfully with tracheal resection surgery. In histology, it may mimic Ewing's sarcoma. Immunohistochemically, SS stains positive for cytokeratin, epithelial membrane antigen, vimentin, and S100. Chromosomal translocation t(X;18) (p11;q11) is found in almost all SS. This genetic signature is the gold standard diagnostic modality for these tumors. CONCLUSION: Diagnosis of tracheal synovial sarcoma is challenging because of the rarity of the disease and common presenting symptoms to other tracheal pathology and is supplemented with chromosomal translocation study along with histopathology and immunohistochemistry. Tumor coring before definite surgical resection facilitates lung perfusion in obstructive tracheal pathology. A multidisciplinary team approach for diagnosis and management along with long-term follow-up are warranted.
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Kumar et al. (2020) conducted a case report in Primary tracheal synovial sarcoma (n=1). Tracheal resection and tracheoplasty was evaluated on Locoregional control and distant metastasis. Tracheal resection and tracheoplasty successfully treated a rare primary tracheal synovial sarcoma, with the patient remaining disease-free at one year follow-up.
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