PMID- 36802923 OWN - NLM STAT- MEDLINE DCOM- 20231023 LR - 20231023 IS - 1940-2465 (Electronic) IS - 1066-8969 (Linking) VI - 31 IP - 7 DP - 2023 Oct TI - Biphenotypic Sinonasal Sarcoma: A Genetically Confirmed Case Showing Bone Invasion Accompanying a Non-neoplastic Respiratory Epithelium. PG - 1414-1419 LID - 10.1177/10668969231152577 [doi] AB - Biphenotypic sinonasal sarcoma is a newly established tumor entity that is associated with distinct clinicopathological findings. Biphenotypic sinonasal sarcoma is a rare, low-grade spindle cell sarcoma that arises in middle-aged females, exclusively in the sinonasal tract. A fusion gene involving PAX3 is detected in most biphenotypic sinonasal sarcomas, which aids in its diagnosis. Here, we report a case of biphenotypic sinonasal sarcoma with its cytological findings. The patient was a 73-year-old woman who presented with purulent nasal discharge and dull pain in the left cheek area. Computed tomography showed a mass extending from the left nasal cavity to the left ethmoid sinus, the left frontal sinus, and the frontal skull base. She underwent a combined transcranial and endoscopic approach for en bloc resection with a safety margin. Histologically, spindle-shaped tumor cells have been thought to proliferate mainly in the subepithelial stroma. Here, nasal mucosal epithelial hyperplasia was noted, and the tumor had invaded the bone tissue accompanying the epithelial cells. Fluorescence in situ hybridization (FISH) analysis showed a PAX3 rearrangement, and next-generation sequencing identified a PAX3::MAML3 fusion. Based on FISH, split signals were observed not in respiratory cells but in stromal cells. This indicated that respiratory cells were non-neoplastic. In the diagnosis of biphenotypic sinonasal sarcoma, the inverted growth of the respiratory epithelium can be a diagnostic pitfall. FISH analysis using a PAX3 break-apart probe is helpful not only for an accurate diagnosis but also for detecting the true neoplastic cells. FAU - Muraoka, Erika AU - Muraoka E AD - Department of Diagnostic Pathology, Yokohama City University Hospital, Yokohama, Japan. RINGGOLD: 218758 AD - Department of Molecular Pathology, Yokohama City University, Graduate School of Medicine, Yokohama, Japan. RINGGOLD: 26438 FAU - Kato, Ikuma AU - Kato I AUID- ORCID: 0000-0002-7731-9509 AD - Department of Molecular Pathology, Yokohama City University, Graduate School of Medicine, Yokohama, Japan. RINGGOLD: 26438 FAU - Matsumura, Mai AU - Matsumura M AD - Department of Pathology, Yokohama City University, Graduate School of Medicine, Yokohama, Japan. RINGGOLD: 26438 FAU - Arai, Yasuhiro AU - Arai Y AD - Department of Otolaryngology, Head and Neck Surgery, Yokohama City University Hospital, Yokohama, Japan. RINGGOLD: 218758 FAU - Suenaga, Jun AU - Suenaga J AD - Department of Neurosurgery, Yokohama City University Hospital, Yokohama, Japan. RINGGOLD: 218758 FAU - Yamanaka, Shoji AU - Yamanaka S AD - Department of Diagnostic Pathology, Yokohama City University Hospital, Yokohama, Japan. RINGGOLD: 218758 FAU - Fujii, Satoshi AU - Fujii S AD - Department of Diagnostic Pathology, Yokohama City University Hospital, Yokohama, Japan. RINGGOLD: 218758 AD - Department of Molecular Pathology, Yokohama City University, Graduate School of Medicine, Yokohama, Japan. RINGGOLD: 26438 LA - eng PT - Case Reports PT - Journal Article DEP - 20230219 PL - United States TA - Int J Surg Pathol JT - International journal of surgical pathology JID - 9314927 SB - IM MH - Middle Aged MH - Female MH - Humans MH - Aged MH - In Situ Hybridization, Fluorescence MH - *Paranasal Sinus Neoplasms/diagnosis/genetics/pathology MH - *Sarcoma/pathology MH - *Soft Tissue Neoplasms MH - Respiratory Mucosa/pathology MH - Bone and Bones/pathology OTO - NOTNLM OT - PAX3::MAML3 OT - biphenotypic sinonasal sarcoma OT - cytology OT - fluorescence in situ hybridization COIS- Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article. EDAT- 2023/02/22 06:00 MHDA- 2023/10/23 12:45 CRDT- 2023/02/21 17:37 PHST- 2023/10/23 12:45 [medline] PHST- 2023/02/22 06:00 [pubmed] PHST- 2023/02/21 17:37 [entrez] AID - 10.1177/10668969231152577 [doi] PST - ppublish SO - Int J Surg Pathol. 2023 Oct;31(7):1414-1419. doi: 10.1177/10668969231152577. Epub 2023 Feb 19.