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Primary Small Cell Neuroendocrine Carcinoma of the Sublingual Gland Treated With Definitive Chemoradiotherapy: A Case Report

Taka-aki Tokura, H. Dehari, K. Nishiyama, Sho Miyamoto, Takanori Sasaki, Kazuhiro Ogi, Taro Sugawara, Masanori Someya et al.

Clinical Case Reports, 2026

Abstract

Primary sublingual gland small cell neuroendocrine carcinoma (SCNEC) is extremely rare, with only one prior case reported, and no site‐specific standard of care has been established. We report a case of primary sublingual gland SCNEC treated with definitive concurrent chemoradiotherapy (CRT). A 69‐year‐old man presented with a 21 × 15‐mm right sublingual mass showing fluorodeoxyglucose uptake (SUVmax 9.9) on PET/CT. No nodal or distant disease was found. Biopsy revealed small cell morphology positive for INSM1/synaptophysin and pan‐cytokeratin, with a high Ki‐67 index (> 95%). IMRT delivered 70 Gy in 35 fractions to the primary tumor with elective bilateral nodal irradiation (levels I–V, 46 Gy). Cisplatin (80 mg/m2; day 1) and etoposide (100 mg/m2; days 1–3) were administered every 3 weeks for four cycles. Grade 3 oral mucositis and grade 2 neutropenia resolved. At 24 months post‐CRT, the patient remains disease‐free with preserved daily function. Definitive concurrent CRT appears to be a feasible and effective organ‐preserving treatment option for limited‐stage primary sublingual gland SCNEC, with durable disease control at 24 months in this case.

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