Small Biopsy Samples: Are They Representative for Biphenotypic Sinonasal Sarcoma?

(1) Background: Biphenotypic sinonasal sarcoma (BSNS) is a rare low-grade neoplasm of the sinonasal tract. It is characterized by specific PAX3 gene rearrangements and both myogenic and neural differentiation. The purpose of the study was to describe the histologic, immunohistochemical and molecular features of BSNS and indicate important clues for small incisional biopsy diagnostics. (2) Methods: Archival samples from patients with nasal cavities or ethmoid sinuses tumors were searched for BSNS cases. Inclusion criteria were the presence of spindle cell morphology and low-grade appearance. Both biopsy and resection specimens were stained for identical IHC panels including, i.a., S100, SMA, SOX10 and PAX3. FISH for PAX3 and SS18 was performed on biopsy specimens. (3) Results: BSNS diagnosis was made in 6 cases included in the study and confirmed by PAX3 rearrangement by FISH in 5 specimens. The pattern of IHC expression was identical for paired biopsy and resection samples apart from one BSNS case. (4) Conclusions: Incisional biopsy seems to be a sufficient method to establish BSNS diagnosis in most cases. Characteristic morphological features together with S100, SOX10 and SMA as the screening markers are useful for confirming the diagnosis. In cases of divergent morphology and immunoprofile evaluation of PAX3 rearrangement is vital.