Abstract
Objectives Low-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.
Design Retrospective case series.
Setting Academic medical center.
Participants There were three patients who met inclusion criteria with a confirmed LGSSNMF.
Main Outcome Measures Imaging and histopathological characteristics, treatments, survival and recurrence
outcomes, complications, morbidity, and mortality.
Results Patients 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.
Conclusions The surgical outcomes and imaging of this unique, locally aggressive skull base tumor
are characterized.
Keywords
low-grade sinonasal sarcoma with neural and myogenic features - skull base malignancy
- imaging characteristics - surgical outcomes - hyperostotic bone formation - locally
aggressive - sinonasal sarcoma
