Rare Head and Neck Neuroendocrine Neoplasms: A Retrospective Study of Prognosis and Treatment Outcomes

Neuroendocrine neoplasms (NEN) of the head and neck (HN) region are rare, with limited reported cases. NENs are classified into neuroendocrine tumors (NET) grades G1, G2, and G3, and neuroendocrine carcinomas (NECs), with varying treatment strategies. This study investigated patient outcomes of HN-NENs and proposed a treatment algorithm based on pathological classification.

This retrospective study analyzed 24 HN-NEN cases treated at Kyushu University Hospital (2007-2023). Tumors were classified using the 2022 WHO criteria, and overall survival rates were evaluated using the Kaplan-Meier method.

Among the 24 patients, 29% had NETs and 71% had NECs. The most common primary sites were the sinonasal cavity (42%) and larynx (29%). Seven-year survival rates were 100% for NET G1 and G2, 50% for NET G3, and 43% for NEC. Two NET cases treated with somatostatin analogs (SSA) and radionuclide therapy (PRRT) showed tumor reduction.

Prognosis of HN-NENs varies significantly by pathological grade. While NET G1 and G2 showed favorable outcomes, NET G3 and NEC had poorer survival. SSA and PRRT may be effective options for selected HN-NETs. A preliminary treatment algorithm is proposed to guide management, warranting validation in larger studies.