TY - JOUR T1 - Imaging Appearance of <em>SMARCB1</em> (INI1)-Deficient Sinonasal Carcinoma: A Newly Described Sinonasal Malignancy JF - American Journal of Neuroradiology JO - Am. J. Neuroradiol. SP - 1925 LP - 1929 DO - 10.3174/ajnr.A4841 VL - 37 IS - 10 AU - D.R. Shatzkes AU - L.E. Ginsberg AU - M. Wong AU - A.H. Aiken AU - B.F. Branstetter IV AU - M.A. Michel AU - N. Aygun Y1 - 2016/10/01 UR - http://www.ajnr.org/content/37/10/1925.abstract N2 - SUMMARY: SMARCB1 (INI1)-deficient sinonasal carcinomas were first described in 2014, and this series of 17 cases represents the first imaging description. This tumor is part of a larger group of SMARCB1-deficient neoplasms, characterized by aggressive behavior and a rhabdoid cytopathologic appearance, that affect multiple anatomic sites. Clinical and imaging features overlap considerably with other aggressive sinonasal malignancies such as sinonasal undifferentiated carcinoma, which represents a common initial pathologic diagnosis in this entity. SMARCB1 (INI1)-deficient sinonasal tumors occurred most frequently in the nasoethmoidal region with invasion of the adjacent orbit and anterior cranial fossa. Avid contrast enhancement, intermediate to low T2 signal, and FDG avidity were frequent imaging features. Approximately half of the lesions demonstrated calcification, some with an unusual “hair on end” appearance, suggesting aggressive periosteal reaction. ER -