A Rare Laryngeal Tumor: Poorly Differentiated Primary Small Cell Neuroendocrine Carcinoma

Laryngeal small-cell neuroendocrine carcinoma is a rare and aggressive type of tumor. Due to the poor degree of differentiation, laryngeal small cell neuroendocrine carcinoma has particularly aggressive biological behavior with a tendency to early and widespread dissemination resulting in a poor prognosis. Based on presenting symptoms and imaging appearance, no differentiation is possible between laryngeal small cell neuroendocrine carcinoma and other laryngeal malignancies. Laryngeal small-cell neuroendocrine carcinoma is distinguished from other types of malignancies with histological examination complemented by immunohistochemistry. Due to its rareness, an evidence-based approach is lacking. The treatment principles are deducted from treatment protocols of small cell lung cancer and individual clinician’s experience. The suggested preferred treatment of laryngeal small cell neuroendocrine carcinoma is a combination of chemotherapeutic agents. The prognosis is poor with a reported 5-year disease-specific survival of 18%-30%. Systemic dissemination at initial presentation is the only variable associated with a worse prognosis and treatment modality does not affect prognosis. Cite this article as: Arensman AF, Pingnet L, Lammens M, Nicolay S, Vanderveken OM. A rare laryngeal tumor: Poorly differentiated primary small cell neuroendocrine carcinoma. B-ENT. 2023;19(3):188-191.