Читать книгу Anterior Skull Base Tumors - Группа авторов - Страница 53

This is a recently described low-grade spindle cell sarcoma, which occurs in the nasal cavity and ethmoid [83, 84]. Histologically, the tumour displays spindle cell proliferation with intersecting fascicles and a herringbone pattern. Similar to synovial sarcoma, markers that may be used to assist diagnosis are S-100 and SMA. Biphenotypic spindle cell sarcoma is characterised by distinctive phenotypic features and the presence of translocation t(2;4)(q³⁵;q^31.1) PAX3-MAML3 gene fusion. The cardinal morphologic features include neural and myogenic differentiation. This is a low-grade malignancy prone to local recurrence, but no reports of metastasis or death due to disease have been published [85–88].