Primary neuroendocrine carcinoma of the jugular foramen

Abstract Background Neuroendocrine neoplasms (NENs) are a rare type of tumor that arise from neuroendocrine cells. Primary neuroendocrine tumors of the head and neck are exceedingly rare and range from benign-behaving neoplasms to aggressive neuroendocrine carcinomas (NEC). Objectives The main purpose of this article is to report a rare case of well-differentiated neuroendocrine carcinoma (WD-NEC) (Grade 1) of the jugular foramen with uncharacteristic high-grade features and aggressive pattern of spread reminiscent of poorly-differentiated neuroendocrine carcinoma (PD-NEC). In addition, we review the scientific literature and describe differences in current World Health Organization (WHO) classification system for various organ systems. Methods A 49-year-old man with right ear infections developed acute facial paralysis. Imaging showed otomastoiditis and incidental right jugular foramen (JF) tumor suspicious for meningioma. After facial nerve decompression, he developed right vocal cord paralysis and dysphagia. His facial nerve function recovered completely; however, repeat imaging showed rapid growth of the JF mass. The patient underwent transtemporal approach for definitive resection. Results Pathology showed Grade 1, WD-NEC with aggressive features: mitotic index up to 2, focal necrosis, Ki-67 > 20%. Nuclear medicine scans confirmed the jugular foramen was the primary site. Chemoradiation was initiated; however, the patient developed metastasis and passed away. Conclusion We present a rare case of WD-NEC of the jugular foramen with atypical presentation and aggressive features. We review diagnostic work-up, WHO classification systems for NEC, and highlight the potential role of Ki-67 in NECs of the head and neck.