Jamir Temjen Sunup, Badhe Bhawana Ashok, Stephen Norton, Srinivas Bheemanathi Hanuman, Pottakkat Biju
North Eastern Indira Gandhi Regional Institute of Health and Medical Sciences Shillong, Meghalaya, India.
Department of Pathology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER) Puducherry, India.
Int J Clin Exp Pathol. 2022 May 15;15(5):225-232. eCollection 2022.
The neuroendocrine system of the gastroenteropancreatic (GEP) region gives rise to unique, heterogeneous malignancies that need a high index of suspicion to make a diagnosis owing to their indolent course.
The present study aimed to find the incidence and the differences in the morphologic and immunohistochemical profile of gastroenteropancreatic neuroendocrine tumors (GEPNET) in a referral center of South India, JIPMER, Puducherry, India.
There were 55 gastroenteropancreatic region neuroendocrine neoplasms (NEN) assessed for demographic, clinical and radiological features. Gross morphological features, histopathological features, mitotic index, Ki67 proliferation index, and immunohistochemical positivity for synaptophysin, chromogranin-A, CD-56, NSE (Neuron Specific Enolase) and pan-cytokeratin (Pan-CK) were also assessed.
The majority were nonfunctional tumors presenting with abdominal pain, gastrointestinal bleed, vomiting, jaundice, and loss of weight and appetite. The sites of involvement according to the order of frequency were duodenum, stomach, rectum, pancreas, ileum, appendix and jejunum. The endoscopic appearance of duodenal and jejunal tumors showed polypoidal, nodular and ulceroproliferative growth. These tumors were diagnosed by preoperative biopsy; 54% of them were grade-1 neuroendocrine tumors exhibiting nesting, trabecular, cord, and solid sheet patterns. All 55 cases were synaptophysin-positive with variable positivity for chromogranin, neuron-specific enolase, CD56, and Pan-CK. Mixed adenoneuroendocrine carcinomas (MANECs) involving the duodenum and stomach comprised 7.3% of all GEPNETs. Pancreatic neuroendocrine tumors constituted 9% of all tumors; one was multifocal. Lymph node metastasis was seen in 12/55 tumors; 6/12 showed liver metastasis also. All metastasizing tumors measured less than 4 cm in size. Statistical correlation of the tumor grade, mitotic count and Ki67 index as analysed by Spearman's correlation between the paired data denoted by r in 55 tumors showed a strong correlation between mitotic count and Ki67 index; a moderate correlation was noted between the tumor grade and Ki67 index.
The clinicopathologic profile of 55 GEPNET revealed a majority to be sporadic Grade 1 tumor. Tumors that showed lymph node and liver metastasis were less than 4 cm in size. MANECs were found in the duodenum and stomach.
胃肠胰（GEP）区域的神经内分泌系统会引发独特的、异质性的恶性肿瘤，由于其病程进展缓慢，需要高度怀疑才能做出诊断。
本研究旨在确定印度南部转诊中心贾瓦哈拉尔·尼赫鲁医学院（JIPMER）位于印度本地治里的胃肠胰神经内分泌肿瘤（GEPNET）的发病率以及形态学和免疫组化特征的差异。
对55例胃肠胰区域神经内分泌肿瘤（NEN）进行了人口统计学、临床和放射学特征评估。还评估了大体形态特征、组织病理学特征、有丝分裂指数、Ki67增殖指数以及突触素、嗜铬粒蛋白A、CD-56、神经元特异性烯醇化酶（NSE）和泛细胞角蛋白（Pan-CK）的免疫组化阳性情况。
大多数为无功能肿瘤，表现为腹痛、胃肠道出血、呕吐、黄疸以及体重和食欲下降。按发生频率排序，受累部位依次为十二指肠、胃、直肠、胰腺小肠、阑尾和空肠。十二指肠和空肠肿瘤的内镜表现为息肉样、结节样和溃疡增殖性生长。这些肿瘤通过术前活检确诊；其中54%为一级神经内分泌肿瘤，呈现巢状、小梁状、条索状和实性片状结构。所有55例病例突触素均呈阳性，嗜铬粒蛋白、神经元特异性烯醇化酶、CD56和Pan-CK呈不同程度阳性。累及十二指肠和胃的混合性腺神经内分泌癌（MANEC）占所有GEPNET的7.3%。胰腺神经内分泌肿瘤占所有肿瘤的9%；其中1例为多灶性。55例肿瘤中有12例出现淋巴结转移；12例中有6例同时伴有肝转移。所有发生转移的肿瘤大小均小于4 cm。通过Spearman相关性分析55例肿瘤配对数据中r表示的肿瘤分级、有丝分裂计数和Ki67指数之间的统计相关性，结果显示有丝分裂计数与Ki67指数之间存在强相关性；肿瘤分级与Ki67指数之间存在中度相关性。
55例GEPNET的临床病理特征显示大多数为散发性一级肿瘤。出现淋巴结和肝转移的肿瘤大小小于4 cm。在十二指肠和胃中发现了MANEC。
