Lin S, Guo R Y, Liu K Y, Mi H Y, Wang M Y, Fu H J, Li R
Depertment of Oral and Maxillofacial Surgery, The First Affiliated Hospital of Zhengzhou University, Zhengzhou 450052, China.
Zhonghua Kou Qiang Yi Xue Za Zhi. 2023 Feb 9;58(2):151-157. doi: 10.3760/cma.j.cn112144-20220803-00436.
To investigate the clinicopathological features, treatment and prognosis of maxillofacial neuroendocrine carcinoma. A total of 11 patients with maxillofacial neuroendocrine carcinoma diagnosed in the Department of Pathology of The First Affiliated Hospital of Zhengzhou University from December 2010 to July 2022 were retrospectively enrolled, including 8 males and 3 females, aged (65.2±9.5) years (ranged from 49 to 87 years), with a disease course of 0.5 to 6.0 months. The clinicopathological data including head and neck CT, MRI and treatment methods were analyzed. Submandibular gland and maxilla were involved in 3 cases, parapharynx in 2 cases, and face, tongue root and soft palate in 1 case respectively. Clinically, the initial symptom is a rapidly growing painless or tender mass, which may be accompanied by restricted mouth opening, dysphagia, and local numbness after invasion of masticatory muscles and nerves. The tumors were all invasive and low-density, with unclear boundaries from the surrounding tissues. Among the patients, 9 received surgical treatment, and 5 received adjuvant treatment after surgery (2 received chemotherapy, 3 received radiotherapy+chemotherapy). According to the 5th edition of the World Health Organization classification of head and neck tumors in 2022, there were 1 case (1/11) with poorly differentiated large cells and 10 cases (10/11) with poorly differentiated small cells. Histologically, the macrocell type is composed of large cells with rough chromatin, obvious vacuolar nucleolus, protruding nucleolus, and necrosis. The small cell type is dominated by small blue round cells with neuroendocrine characteristics, with active growth and multifocal necrosis. Immunohistochemical staining showed that cytokeratin (CK), epithelial membrane antigen (EMA) and synaptophysin (Syn) were diffusively expressed, 10 cases expressed CD56, 8 cases expressed p63, 6 cases expressed weakly punctated chromograin-A (CgA), and S-100 was not expressed. The Ki-67 index ranges from 20 to 90 percent. By the end of follow-up (0.5 to 127.0 months), 3 patients were alive, and the mean progression-free survival (21.0 months) of postoperative chemoradiotherapy patients was significantly longer than that of surgery and/or chemotherapy alone (3.3 months). Maxillofacial neuroendocrine carcinoma is characterized by low differentiation of small cells, high degree of malignancy and poor prognosis. Radical surgery combined with chemoradiotherapy has better local control effect.
探讨颌面部神经内分泌癌的临床病理特征、治疗方法及预后。回顾性纳入2010年12月至2022年7月在郑州大学第一附属医院病理科确诊的11例颌面部神经内分泌癌患者，其中男性8例，女性3例，年龄（65.2±9.5）岁（49～87岁），病程0.5～6.0个月。分析其临床病理资料，包括头颈部CT、MRI及治疗方法。3例累及下颌下腺及上颌骨，2例累及咽旁，1例分别累及面部、舌根及软腭。临床上，首发症状为迅速生长的无痛性或压痛性肿块，侵犯咀嚼肌和神经后可伴有张口受限、吞咽困难及局部麻木。肿瘤均呈浸润性生长，密度较低，与周围组织边界不清。患者中9例行手术治疗，5例术后接受辅助治疗（2例接受化疗，3例接受放疗+化疗）。根据2022年世界卫生组织头颈部肿瘤分类第5版，低分化大细胞型1例（1/11），低分化小细胞型10例（10/11）。组织学上，大细胞型由染色质粗糙、核仁明显呈空泡状、核仁突出及有坏死的大细胞组成。小细胞型以具有神经内分泌特征的小蓝圆形细胞为主，生长活跃，多灶性坏死。免疫组化染色显示细胞角蛋白（CK）、上皮膜抗原（EMA）和突触素（Syn）呈弥漫性表达，10例表达CD56，8例表达p63，6例弱点状表达嗜铬粒蛋白A（CgA），S-100不表达。Ki-67指数为20%～90%。至随访结束（0.5～127.0个月），3例存活，术后放化疗患者的平均无进展生存期（21.0个月）明显长于单纯手术和/或化疗患者（3.3个月）。颌面部神经内分泌癌以小细胞低分化、恶性程度高、预后差为特点。根治性手术联合放化疗有较好的局部控制效果。
