Biphenotypic Sinonasal Sarcoma A Review and Update

被引:31
作者
Carter, Cody S. [1 ]
East, Ellen G. [1 ]
McHugh, Jonathan B. [1 ]
机构
[1] Michigan Med, Dept Pathol, Room 2G332 UH,1500 East Med Ctr Dr, Ann Arbor, MI 48109 USA
关键词
SOLITARY FIBROUS TUMOR; NUCLEAR BETA-CATENIN; SYNOVIAL SARCOMA; MYOGENIC FEATURES; NASAL CAVITY; GENE FUSIONS; EXPRESSION; PAX3; IMMUNOHISTOCHEMISTRY; HEMANGIOPERICYTOMA;
D O I
10.5858/arpa.2018-0207-RA
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
Context.-Biphenotypic sinonasal sarcoma (BSNS) is a rare, slow-growing soft tissue sarcoma of the sinonasal tract, typically presenting with nonspecific obstructive nasal symptoms. Although recurrences are common, no metastases have been reported, and only 1 patient has died of disease thus far. It characteristically demonstrates rearrangements of PAX3 with multiple fusion partners, the most common of which is MAML3. Objectives.-To highlight the most important diagnostic features, including morphologic, immunohistochemical, and molecular findings, and to provide comparisons to other entities in the differential diagnosis. We also aim to provide a summary of the clinical features and outcomes in cases reported to date. Data Sources.-Recently published literature encompassing BSNS and its synonym, low-grade sinonasal sarcoma with neural and myogenic differentiation. Conclusions.-BSNS is a sinonasal tumor that is important to recognize because its biologic behavior differs from most of the entities in the differential diagnosis. The diagnosis can typically be rendered through a combination of morphology, immunohistochemical stains, and ancillary testing for characteristic PAX3 rearrangements.
引用
收藏
页码:1196 / 1201
页数:6
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