Nonsinonasal Neuroendocrine Carcinoma of the Head and Neck: Case Series and Review of Literature

Objective: To describe three patients with nonsinonasal neuroendocrine carcinoma (NSNEC) of the head and neck and present a systematic review of literature. Study design: Retrospective chart review Methods: Three patients with pathologically proven NSNEC of the head-neck who presented to our institution were identified. Relevant demographic, clinic-pathological, and radiological data was recorded. A web-based search was conducted to identify relevant scientific literature on “neuroendocrine carcinoma (NEC) of the head and neck” and a systematic review of literature is presented. Results: Two female and one male patient aged 44 to 66 years presented to our service with NEC of the supraglottis (2/3), and of the thymus (1/3). Diagnosis was confirmed with immunohistochemical staining such as Ki-67, synaptophysin, chromogranin, and also with octreotide scanning. The first case, a T4bN0M0 large cell NEC of the thymus with tracheal invasion received conservative airway management and emergent radiotherapy. The second case, moderately differentiated NEC of the supraglottis was managed with a laser supraglottic laryngectomy followed by adjuvant treatment. The third case, a laryngeal NEC was treated with chemoradiotherapy. Conclusions: NSNEC of the head and neck are extremely rare tumors with variability in clinical presentation that present challenges in diagnosis and treatment planning. Consideration of the histological subtype and staining characteristics of the individual tumor, accurate diagnosis, and classification of the tumor is vital in order to tailor therapeutic intervention.