title: Imaging and Outcomes for a New Entity: Low-Grade Sinonasal Sarcoma with Neural and Myogenic Features.
abstract: ObjectivesLow-grade sinonasal sarcoma with neural and myogenic features (LGSSNMF) is a new, rare tumor. Our goal is to describe the imaging characteristics and surgical outcomes of this unique skull base malignancy.DesignRetrospective case series.SettingAcademic medical center.ParticipantsThere were three patients who met inclusion criteria with a confirmed LGSSNMF.Main Outcome MeasuresImaging and histopathological characteristics, treatments, survival and recurrence outcomes, complications, morbidity, and mortality.ResultsPatients presented with diplopia, facial discomfort, a supraorbital mass, and nasal obstruction. Magnetic resonance imaging and computed tomography imaging in all cases showed an enhancing sinonasal mass with associated hyperostotic bone formation that involved the frontal sinus, invaded the lamina papyracea and anterior skull base, and had intracranial extension. One patient underwent a purely endoscopic surgical resection and the second underwent a craniofacial resection, while the last is pending treatment. All patients recovered well, without morbidity or long-term complications, and are currently without evidence of disease (mean follow-up of 2.1 years). One patient recurred after 17 months and underwent a repeat endoscopic skull base and dural resection.ConclusionsThe surgical outcomes and imaging of this unique, locally aggressive skull base tumor are characterized.
authors.affiliation: Division of Otolaryngology - Head & Neck Surgery, Department of Surgery, University of Utah School of Medicine, Salt Lake City, Utah, United States; Department of Radiology, University of Utah School of Medicine, Salt Lake City, Utah, United States.
authors.forename: Richard B
authors.initials: RB
authors.lastname: Cannon
authors.affiliation: Division of Otolaryngology - Head & Neck Surgery, Department of Surgery, University of Utah School of Medicine, Salt Lake City, Utah, United States.
authors.forename: Richard H
authors.initials: RH
authors.lastname: Wiggins
authors.affiliation: The Huntsman Cancer Institute, Salt Lake City, Utah, United States; Department of Pathology, University of Utah School of Medicine, Salt Lake City, Utah, United States.
authors.forename: Benjamin L
authors.initials: BL
authors.lastname: Witt
authors.affiliation: The Huntsman Cancer Institute, Salt Lake City, Utah, United States.
authors.forename: Yusuf
authors.initials: Y
authors.lastname: Dundar
authors.affiliation: The Huntsman Cancer Institute, Salt Lake City, Utah, United States.
authors.forename: Tawni M
authors.initials: TM
authors.lastname: Johnston
authors.affiliation: Division of Otolaryngology - Head & Neck Surgery, Department of Surgery, University of Utah School of Medicine, Salt Lake City, Utah, United States; The Huntsman Cancer Institute, Salt Lake City, Utah, United States.
authors.forename: Jason P
authors.initials: JP
authors.lastname: Hunt
keywords: hyperostotic bone formation; imaging characteristics; locally aggressive; low-grade sinonasal sarcoma with neural and myogenic features; sinonasal sarcoma; skull base malignancy; surgical outcomes