Large cell neuroendocrine carcinoma of the submandibular gland: Case report and literature review

Abstract Large cell neuroendocrine carcinoma (LCNEC) of the salivary gland is extremely rare. We report on a case of LCNEC in the submandibular gland. A 58-year-old male had a four-month history of an enlarging mass in his left submandibular region. He underwent lymph node resection and metastasis of LCNEC was suspected. Magnetic resonance imaging of the neck showed a solid submandibular gland tumor with marginal blurring. Positron-emission tomography and upper gastrointestinal endoscopy showed no evidence of malignancy other than in the left submandibular gland. He underwent left submandibular gland resection and left upper neck dissection. The final diagnosis was LCNEC of the submandibular gland; surgical margin was negative. Fourteen months later he is free of tumors. This is the first report of LCNEC of the submandibular gland. LCNEC of the salivary gland shows high-grade malignancy like that of the lung. According to past reports, two of four patients died despite multidisciplinary treatments. There are no standard treatments for LCNEC of the salivary glands. More studies are needed to define prognostic factors and establish therapeutic methods.