Sinonasal undifferentiated carcinoma: Case series and review of the literature

OBJECTIVE AND IMPORTANCE: We report on four cases of sinonasal undifferenti ated carcinoma (SNUC), a relatively newly described clinicopathological ent ity of the nasal cavity and paranasal sinuses. SNUC tends to present with a dvanced-stage disease, often with intracranial invasion, and requires an ag gressive treatment approach that includes surgical resection. A review of t he literature identified several reports of SNUC in pathology and otolaryng ology journals since its initial description in 1986, but no report has yet appeared in the neurosurgery literature. CLINICAL PRESENTATION: Four patients presented with various symptoms relate d to the nose and/or orbit, including one or more of the following: obstruc tion, epistaxis, decreased visual acuity, diplopia, and pain. All patients were noted to have masses in the nasal cavity or paranasal sinuses, with or without intracranial extension. INTERVENTION: All four patients underwent multimodal treatment with chemoth erapy, radiotherapy (60-65 Gy), and aggressive surgical resection via a com bined bifrontal craniotomy and a subcranial approach to the anterior crania l fossa. Three of four patients died as a result of their disease, an avera ge of 15 months after diagnosis. Only one patient remains alive, although w ith metastatic intracranial disease, at 24 months after diagnosis. CONCLUSION: SNUC is a rare neoplasm with a poor prognosis despite an aggres sive multimodal approach to treatment. On the basis of our experience, we a dvocate radical resection as part of the initial combined therapy for patie nts who present with locally advanced, nonmetastatic disease but we suggest reserving surgery for patients with early brain invasion until there has b een a radiographically proven central nervous system response to adjuvant t herapy.