title: Imaging Appearance ofSMARCB1(INI1)-Deficient Sinonasal Carcinoma: A Newly Described Sinonasal Malignancy.
abstract: 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 ofSMARCB1-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.
authors.affiliation: From the Department of Radiology (D.R.S., M.W.), Lenox Hill Hospital, Northwell Health, New York, New York DShatzkes@northwell.edu.
authors.forename: D R
authors.identifier: http://orcid.org/0000-0002-1559-7298
authors.initials: DR
authors.lastname: Shatzkes
authors.affiliation: Department of Radiology (L.E.G.), The University of Texas MD Anderson Cancer Center, Houston, Texas.
authors.forename: L E
authors.identifier: http://orcid.org/0000-0002-3110-7754
authors.initials: LE
authors.lastname: Ginsberg
authors.affiliation: From the Department of Radiology (D.R.S., M.W.), Lenox Hill Hospital, Northwell Health, New York, New York.
authors.forename: M
authors.identifier: http://orcid.org/0000-0002-6694-4624
authors.initials: M
authors.lastname: Wong
authors.affiliation: Department of Radiology (A.H.A.), Neuroradiology Division, Emory University Hospital, Atlanta, Georgia.
authors.forename: A H
authors.identifier: http://orcid.org/0000-0003-1096-3540
authors.initials: AH
authors.lastname: Aiken
authors.affiliation: Department of Radiology (B.F.B.), Presbyterian Hospital, Pittsburgh, Pennsylvania.
authors.forename: B F
authors.identifier: http://orcid.org/0000-0002-8502-6909
authors.initials: BF
authors.lastname: Branstetter
authors.affiliation: Department of Radiology (M.A.M.), Medical College of Wisconsin, Milwaukee, Wisconsin.
authors.forename: M A
authors.identifier: http://orcid.org/0000-0003-0112-5078
authors.initials: MA
authors.lastname: Michel
authors.affiliation: Department of Radiology (N.A.), Johns Hopkins Medicine, Baltimore, Maryland.
authors.forename: N
authors.identifier: http://orcid.org/0000-0003-4631-0533
authors.initials: N
authors.lastname: Aygun