Neuroendocrine carcinoma of tonsil presented with axillary lymph node metastasis: Management dilemma

High-grade neuroendocrine carcinoma (NEC) of the tonsil is rare and has a poor prognosis. The usual presentation is a neck mass with locoregional cervical lymphadenopathy. An axillary lymphadenopathy as a primary presentation of NEC of the tonsils is uncommon and challenging to treat. Tonsil neuroendocrine tumors display aggressive behaviors associated with early recurrence and metastasis after surgical resection. Managing this condition is demanding compared to NECs of gastrointestinal origin since, to date, the management of head-and-neck neuroendocrine tumors is still not well established. We present a 49-year-old female with a rare case of NEC of the tonsil presenting primarily with axillary lymph nodes metastasis. The patient's axillary lymph node was biopsied and revealed a Grade III neuroendocrine tumor. A positron emission tomography (PET) scan was done in searching of a primary lesion and showed a highly metabolic mass of the left tonsil as well as a left axillary lymph node suggestive of metastasis. The patient has been managed with a multimodality approach, with a combination of chemotherapy regimen and surgical resection of the axillary lymph node. Subsequent PET scan evaluation showed a complete response of the primary tumor with residual left axillary lymph node metastasis. NECs of tonsil presented with axillary lymph nodes metastasis is rare and has a poor prognostic outcome. It poses a dilemma with regard to management, as surgical resection of the metastasis is not promising given the possibility of early recurrence.