High-Grade Large Cell Neuroendocrine Cancer of Ethmoid Sinus

Author(s): Batoul Sadek, DO, Mohamed Hashem, DO, Fisnik Cake, DO

Large-cell neuroendocrine carcinoma (LCNEC) is a rare high-grade malignancy usually found in the lung and gastrointestinal tract. It is uncommon for LCNEC to occur in the paranasal sinuses. Computerized tomography (CT) or magnetic resonance imaging (MRI) with contrast and biopsy by nasal endoscopy are used to diagnose and stage the tumors. Histological confirmation is made by the presence of large cells with immunohistochemical staining positive for synaptophysin and chromogranin-A. We present the case of a 55-year-old White male with no significant past medical history who presented to his primary care doctor with three months of unresolving headaches. He had a rapid progression of symptoms including loss of taste and smell and eventual hypoesthesia of the right palate, diplopia, and pain. CT with and without contrast demonstrated a 4.9x2.9x2.6 cm mass in the posterior ethmoid sinus invading the cribriform plate and fovea ethmoidalis. Nasal endoscopy with a biopsy of the ethmoid sinus mass revealed that the tumor was a high-grade LCNEC. He underwent craniofacial resection of the tumor for debulking. Repeat CT six weeks after demonstrated regrowth of the mass and invasion of the sphenoid sinus, anterior clinoid process, right inferior and superior orbital fissures, and pterygopalatine fossa with sphenoid roof destruction. He underwent chemotherapy with six cycles of cisplatin and etoposide with complete response and is currently undergoing sequential radiation treatment. This case demonstrates a rare case of LCNEC tumor of the ethmoid sinus, which typically has a poor prognosis. There is limited data on treatment for this rare condition in the literature. Hence, we find it important to document and publish each case to aid clinicians in determining appropriate treatment for patients with similar condition. We have also done a SEER database analysis and literature review of all reported cases of sinonasal LCNEC.

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