• Korean Journal of Head & Neck Oncology
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• v.35 no.2
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• pp.45-49
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• 2019

Large cell neuroendocrine carcinoma is a rare epithelial neuroendocrine malignancy and is preferentially located in gastrointestinal tract and pancreas. Cases of large cell neuroendocrine carcinoma have been reported in many other locations, including the thymus, gallbladder, prostate, larynx, salivary glands, nasopharynx, tonsil and mastoid. However, primary sinonasal large cell neuroendocrine carcinoma never have been reported in Korea. We experienced a case of primary large cell neuroendocrine carcinoma arising from left maxillary sinus recently. A 82-year-old male patient presented with nasal obstruction and epistaxis. The biopsy revealed large cell neuroendocrine carcinoma with poor differentiation. After a general evaluation, the patient was staged as cT3N0M0. The patient was treated by combined radiotherapy and chemotherapy. We report this rare case with literature review.

• Annals of Hepato-Biliary-Pancreatic Surgery
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• v.27 no.4
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• pp.380-387
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• 2023

Backgrounds/Aims: In 2019, the grading and staging system for neuroendocrine neoplasms (NENs) was significantly changed. In this study, we report the clinicopathological characteristics and surgical outcomes of patients with extrahepatic biliary NENs who underwent curative resection with or without adjuvant treatment. Methods: We retrospectively reviewed a database of 16 patients who developed NENs, neuroendocrine carcinoma (NEC), and mixed endocrine non-endocrine neoplasms (MiNENs) after curative resection. Among them, eight patients had ampulla of Vater (AoV) tumors, and eight patients had non-AoV tumors. Results: G1 and G2 were more frequently observed in the AoV group than in the non-AoV group (12.5% and 62.5%, respectively). In contrast, NEC and MiNEN were more common in the non-AoV group (50.0%). High Ki-67 index (> 20%) and perineural invasion (PNI) were more frequently observed in the non-AoV group. Advanced age (> 65 years), mitotic count > 20 per 2 mm², and Ki-67 index > 20% were strongly correlated with patient survival (p = 0.018, 0.009, and 0.044, respectively). Advanced age (> 65 years) and mitotic count > 20 per 2 mm² were significantly correlated with disease recurrence (p = 0.033 and 0.010, respectively). Conclusions: AoV and non-AoV tumors had significant differences in the histologic grade, Ki67, and PNI. Patients with non-AoV tumors had an increased risk for survival and recurrence than those in the AoV group. For extrahepatic biliary NENs, early detection of tumors, adequate surgery, and aggressive adjuvant treatment for high-risk patients are important to achieve long-term survival and prevent disease recurrence.