Li Ran, Cao Jintao, Chen Liucheng, Cui Fangqin, Chen Shaohua, Feng Zhenzhong, Li Nan
Department of Pathology, The First Affiliated Hospital of Bengbu Medical College, Bengbu Medical College, Bengbu, Anhui, People's Republic of China.
Department of Radiology, The First Affiliated Hospital of Bengbu Medical College, Department of Imaging Diagnosis, Medical Imaging College, Bengbu Medical College, Bengbu, Anhui, People's Republic of China.
Onco Targets Ther. 2020 Sep 30;13:9731-9740. doi: 10.2147/OTT.S275633. eCollection 2020.
Gastrointestinal neuroectodermal tumors (GNETs) are uncommon malignant tumors derived from ectodermal primitive neural cells.
We retrospectively analyzed 2 GNET cases at our hospital and the remaining 94 cases in the literature to determine clinicopathological prognostic factors.
The patients had a mean age of 36 years and a median tumor size of 4.5 cm. A total of 67.0% of the tumors were located in the small intestine, and 76.4% of the patients presented recurrence or metastasis. There was a significant difference in sex and presence of osteoclast-like cells (<0.01). Microscopically, most cells were round or short spindle-like in shape, with weak eosinophilic or clear cytoplasm. Neoplastic cells were always arranged in solid sheets, nests, and pseudoalveoli. Immunohistochemistry showed strong, diffuse S100 and SOX10 expression, with a complete absence of HMB45 and Melan-A expression. A total of 72.9% of the cases revealed genetic EWSR1 recombination, including our 2 cases. The median time to death and first metastasis was 61 months and 12 months, respectively. K-M analysis showed a great difference in survival according to lymph node invasion or distant metastasis (M+N), independent lymph node metastasis (N), lower histological grades (G2), and aggressive chemoradiotherapy (=0.026, =0.027, =0.039 and =0.037). However, independent T, independent M, and postoperative routine adjuvant therapy showed no statistical influence on overall survival or disease-free survival.
GNET is a new entity distinct in its clinical, morphological, immunochemical, and genetic features. Radical excision, close follow-up and adjuvant therapy may be effective for prolonged survival.
胃肠道神经外胚层肿瘤（GNETs）是源自外胚层原始神经细胞的罕见恶性肿瘤。
我们回顾性分析了我院的2例GNET病例以及文献中的其余94例病例，以确定临床病理预后因素。
患者的平均年龄为36岁，肿瘤中位大小为4.5厘米。总共67.0%的肿瘤位于小肠，76.4%的患者出现复发或转移。性别和破骨细胞样细胞的存在有显著差异（<0.01）。显微镜下，大多数细胞呈圆形或短梭形，胞质嗜酸性弱或清亮。肿瘤细胞总是排列成实性片状、巢状和假腺泡状。免疫组化显示S100和SOX10呈强弥漫性表达，HMB45和Melan - A表达完全缺失。总共72.9%的病例显示有EWSR1基因重排，包括我们的2例病例。中位死亡时间和首次转移时间分别为61个月和12个月。K - M分析显示，根据淋巴结侵犯或远处转移（M + N）、独立淋巴结转移（N）、较低的组织学分级（G2）以及积极的放化疗，生存率有很大差异（=0.026、=0.027、=0.039和=0.037）。然而，独立的T、独立的M以及术后常规辅助治疗对总生存期或无病生存期无统计学影响。
GNET是一种在临床、形态、免疫化学和基因特征上都不同的新实体。根治性切除、密切随访和辅助治疗可能对延长生存期有效。
