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双表型鼻窦肉瘤——一种近期描述的有许多相似病变的实体:病例报告

Biphenotypic sinonasal sarcoma-A recently described entity with many mimics: A case report.

作者信息

Ingle Abhijeet, Mahendra Nagendra, Gopal Reddy G Venu

机构信息

Department of Pathology, Yashoda Hospital, Hyderabad, Telangana, India.

ENT Surgery, Yashoda Hospital, Hyderabad, Telangana, India.

出版信息

Indian J Pathol Microbiol. 2023 Apr-Jun;66(2):396-399. doi: 10.4103/ijpm.ijpm_208_21.

Abstract

Biphenotypic sinonasal sarcoma (BSNS) is a recently described, low-grade, slow-growing sarcoma with neural and myogenic features with exclusive location in sinonasal track and characteristic PAX3- MAML3 gene fusion. Differentiating this tumor from its commoner mimics needs knowledge of this entity to avoid over treatment. This tumor has unique morphology, clinical course, and genetics. We report this in a 47-year-old female who was diagnosed with such a rare, solitary fibrous tumor-hemangiopericytoma (HPC-SFT) on limited initial biopsy. On subsequent excision, typical morphology and immunohistochemistry helped to clinch the diagnosis.

摘要

双表型鼻窦肉瘤(BSNS)是一种最近被描述的低级别、生长缓慢的肉瘤,具有神经和肌源性特征,仅位于鼻窦,具有特征性的PAX3-MAML3基因融合。将这种肿瘤与其常见的相似肿瘤区分开来需要了解该实体,以避免过度治疗。这种肿瘤具有独特的形态、临床病程和遗传学特征。我们报告了一名47岁女性,她在最初的有限活检中被诊断为这种罕见的孤立性纤维瘤-血管外皮细胞瘤(HPC-SFT)。在随后切除时,典型的形态和免疫组化有助于确诊。

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