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Biphenotypic sinonasal sarcoma-A recently described entity with many mimics: A case report
Abhijeet Ingle1, Nagendra Mahendra2, G Venu Gopal Reddy3
1Department of Pathology, Yashoda Hospital, Hyderabad, Telangana, India.
Indian Journal of Pathology & Microbiology
|April 20, 2023
Summary
Biphenotypic sinonasal sarcoma (BSNS) is a rare tumor with neural and myogenic features. Accurate diagnosis is crucial to distinguish it from mimics and prevent overtreatment.
Area of Science:
- Oncology
- Pathology
- Genetics
Background:
- Biphenitional sinonasal sarcoma (BSNS) is a rare, low-grade malignancy.
- It exhibits both neural and myogenic differentiation.
- BSNS is exclusively found in the sinonasal tract and characterized by a specific PAX3-MAML3 gene fusion.
Observation:
- The case involved a 47-year-old female initially misdiagnosed with a solitary fibrous tumor-hemangiopericytoma (HPC-SFT) on biopsy.
- The tumor presented as a rare, solitary lesion.
Findings:
- The definitive diagnosis of BSNS was established after surgical excision.
- Typical morphology and specific immunohistochemical markers confirmed the diagnosis.
- The characteristic PAX3-MAML3 gene fusion is a key diagnostic feature.
Implications:
- Accurate differentiation of BSNS from its mimics is essential for appropriate patient management.
- Knowledge of BSNS's unique features can prevent misdiagnosis and overtreatment.
- This case highlights the importance of comprehensive evaluation for rare sinonasal tumors.
