INTRODUCTION: Neuroendocrine carcinomas (NECs) of the stomach, although rare, deserve particular attention as they are aggressive and have an extremely poor prognosis [1–6]. In addition, the concept of this disease and its diagnostic criteria have been changed recently. The World Health Organization (WHO) proposed new diagnostic criteria in 2010 that mainly depend on the rate of cellular proliferation [7]. This is a case series of the case admitted in Sree Balaji medical college and hospital for a period of two year and to nd out the incidence of the neuroendocrine tumor and its various modalities of presentation and its management MATERIALS AND METHODOLOGY: Ÿ This is a case series of the case admitted in sree balaji medical college and hospital for a period of two year and to nd out the incidence of the neuroendocrine tumor and i ts various modali t ies of presentat ion and i ts management. Ÿ Inclusion criteria Ÿ All cases of NEC biopsy proven Ÿ Exclusion criteria Ÿ Associated with other malignancy Ÿ After thorough clinical examination patient is examined systematically and necessary investigations sent such as USG abdomen, CECT andomen, MRI contrast A CASE SERIES OF NEUROENDOCRINE TUMOURS OF GI TRACT- A INCREASE INCIDENCE IN RECENT DAYS Original Research Paper Dr. Magesh Kumar. J* Assistant professor, Department of General Surgery, Sree Balaji Medical College & Hospital, Bharath University, Chromepet, Chennai *Corresponding Author General Surgery Gastric neuroendocrine carcinomas are rare and have a poor prognosis and the diagnostic criteria for this disease have recently changed. This is a case series of the case admitted in Sree Balaji medical college and hospital for a period of two year and to nd out the incidence of the neuroendocrine tumor and its various modalities of presentation and its management. ABSTRACT KEYWORDS : Gastric Neuroendocrine Carcinoma, Sporadic Gastric P. Suresh Babu Associate Professor Sree Balaji Medical College & Hospital, Bharath University, Chromepet, Chennai – 600044 K. S. Ravishankar Professor & HOD, Department of General Surgery,Sree Balaji Medical College & Hospital, Bharath University, Chromepet, Chennai – 600044 VOLUME-8, ISSUE-9, SEPTEMBER-2019 • PRINT ISSN No. 2277 - 8160 • DOI : 10.36106/gjra S no Age and sex Clinical presentation Investigations treatment 1 60/F Non- specic abdominal pain UGI scopy – Moderate dysplasia consequent scopy biopsy leading to NEC Total gastrectomy with GJ and JJ 2 50/M Acute Pain Abdomen Features suggestive of gastric perforation CECT – Gastric perforation with perigastric nodes (growth in pylorus) Bilroth II surgery HPE - NEC 3 45/F Dyspepsia for evaluation UGI scopy revealed – polyp in the D1 Biopsy –NEC Endoscopic removal of the polyp. Patient is in observation 4 45/M Presenting with Gastric outlet obstruction CECT, UGI revealed the pathology HPE - antral growth Palliative gastrectomy with GJ and JJ 5 52/M Rectal growth, Bleeding PR for evalauation HPE proven – NEC Pelvic evisceration with prostatectomy as it was inltrating with ileal conduit. CASE SERIES PICTURES: Intra op picture showing tumour Intraop picture showing perforated tumou 72 X GJRA - GLOBAL JOURNAL FOR RESEARCH ANALYSIS Slide showing normal mucosa &tumour site Salt and pepper nuclei appearenace Peripheral palisading of cells For all the mentioned cases immunohistochemistry helped in nalising the diagnosis.the patients are still in followup stusy and doing well.still larger scale of study is in progress. DISCUSSION: Neuroendocrine neoplasm (NEN) is an epithelial neoplasm with predominant neuroendocrine differentiation and is an uncommon tumor with multiple sites of occurrence [5]. NENs are commonly divided by origin as located in the foregut (lung, bronchus, stomach or duodenum), midgut (jejunum, ileum, appendix or proximal colon) and hindgut (distal colon or rectum). The percentage of foregut cases is 34%, midgut 30% and hindgut 36% [6]. Gastric neuroendocrine neoplasms (NENs) embrace a group of tumors that exhibit a spectrum of histopathologic variations, ranging from clearly benign tumors to highly malignant ones. Recently, the concept of this disease and its diagnostic criteria have been changed. In the 2010 WHO criteria, NENs of the stomach are dened as neoplasms with neuroendocrine differentiation, including neuroendocrine tumors (NETs) and NECs arising in the stomach [7]. Synonyms for gastric NETs include carcinoid, well- di f ferent iated endocrine tumor/ carcinomas, and enterochromafn-like cell NETs, and synonyms for NECs include poorly differentiated endocrine carcinomas and small cell and large cell endocrine carcinomas. NENs are classied i n t o N E T G 1 ( c a r c i n o i d ) a n d G 2 , N E C s , m i x e d adenoneuroendocrine carcinomas, enterochromafn cells, serotonin-producing NETs, and gastrin-producing NETs [7]. Gastric NEN is classied into neuroendocrine tumor (NET), n e u r o e n d o c r i n e c a r c i n o m a ( N E C ) , m i x e d adenoneuroendocrine carcinoma, enterochromafn cells, serotonin-producing NETs and gastrinproducing NETs. NETs include NET G1 (carcinoid) and NET G2 (well-differentiated neuroendocrine tumor/carcinoma). NECs include NEC G3 (poorly differentiated neuroendocrine carcinoma small cell type/large cell type) [4]. NEN is positive for synaptophysin and chromogranina [7]. NEN is classied based on the level of cellular proliferation, including the mitotic and Ki-67 indices [4]. In our case, the mitotic index was 16/10 HPF and the Ki-67 labeling index was 26%-50%. Thus, she was diagnosed with NEC. We suspected jumping metastasis from the main lesion to the proximal margin. In the Japanese Classication of Gastric Carcinoma, NENs are classied into carcinoid tumors and endocrine carcinomas (small cell type and large cell type) [8]. Although the prevalence of gastric NENs has recently risen, they are thought to be relatively rare tumors that account for less than 1% of all gastric tumors [9]. In general, the majority of these tumors are NETs, whose courses are indolent and not life threatening. Concerning NETs, more than 100 years have passed since Oberndorfer proposed the term “carcinoid” in 1907 [10]. In 1993, Rindi et al. advocated a classication system with three subtypes of gastric carcinoid tumors according to the clinicopathological features [1], and this classication system is reected in the 2010 WHO criteria. On this background, Gilligan et al. advocated a treatment algorithm for gastric carcinoid tumors, including the above- mentioned subtypes as well as the size and number of tumors [11]. Recently, less invasive therapeutic options, such as endoscopic resection of the tumor, have been reported for small NETs [12]. NECs of the stomach are also rare, representing less than 10% of gastric NENs [2, 13], and such rarity has made it difcult to understand precisely their biological nature and to establish optimal treatment options. The NEC of our case was a difcult diagnosis to establish, and the immunohis tochemis t r y p layed a major ro le . Microscopically, the patient's tumor was uniform in shape and arranged in small microtubular structures (rosette-like arrangement) to form solid nests, with medium-sized, round- to-cuboid-shaped tumor cells on hematoxylin and eosin staining. Onimmunohistochemistry, the tumors are usually positive for synaptophysin and neuronal-specic enolase, but are rarely positive for the chromograninA staining observed in our patient. In the 2010 WHO criteria, NENs are classied into NETs or NECs on the basis of the level of cellular proliferation, including the mitotic and Ki-67 indices. The mitotic index was 46/10HPF and the Ki-67 labeling index was 70–80% in our case, so he was diagnosed as NEC. Lymphatic invasion was widely observed and lymph node involvement was seen in many nodes, suggesting the high-grade malignant nature of this tumor and showing the compatibility of the diagnosis. Aggressive surgery and chemotherapy should be considered for any NEC [3].Gastric NEN is classied into neuroendocrine tumor (NET), neuroendocrine carcinoma (NEC), mixed adenoneuroendocrine carcinoma, enterochromafn cells, serotonin-producing NETs and gastrinproducing NETs. NETs include NET G1 (carcinoid) and NET G2 (well-differentiated neuroendocrine tumor/carcinoma). NECs include NEC G3 (poorly differentiated neuroendocrine carcinoma small cell type/large cell type) [4]. NEN is positive for synaptophysin and chromograninA [7]. NEN is classied based on the level of cellular proliferation, including the mitotic and Ki-67 indices [4]. In our case, the mitotic index was 16/10 HPF and the Ki-67 labeling index was 26%-50%. Thus, she was diagnosed with NEC. We suspected jumping metastasis from the main lesion to the proximal margin. Gastric NEN has different prognoses VOLUME-8, ISSUE-9, SEPTEMBER-2019 • PRINT ISSN No. 2277 - 8160 • DOI : 10.36106/gjra X 73GJRA - GLOBAL JOURNAL FOR RESEARCH ANALYSIS VOLUME-8, ISSUE-9, SEPTEMBER-2019 • PRINT ISSN No. 2277 - 8160 • DOI : 10.36106/gjra and treatments depending on type. The prognosis of NET G1 is good and the 5 year survival rate is high. NET G2 has a favorable prognosis but is aggressive. NEC has the highest malignant potential but the 5 year survival rate is 75%-80%; however, the prognosis is poor. NET can be removed by endoscopic resection, whereas NEC requires surgical resection and lymph node dissection [8]. The best choice adjuvant chemotherapy for NEC is cisplatinum-based chemotherapy [9]. However, in this case we used a 5FU oral agent because of the patient's nancial status and compliance. In conclusion, a neuroendocrine tumor can be removed by endoscopic resection but it must be a radical surgical resection in accordance with a malignant tumor, due to its aggressive tendency and high malignant potential. DIAGNOSIS PROTOCOL Ÿ 24 hr urinary measurement of 5HIAA Ÿ Serum chromogranin A Ÿ NT – proBNP ( N-terminal pro-brain natriuretic peptide)- Ÿ used in surveillance & as a prognostic marker. Ÿ CECT scan – investigation of choice – solid mass with spiculated borders & radiating surrounding strands that is associated with linear strands within the mesenteric fat & kinking of the bowel Ÿ MRI – useful in liver metastasis Ÿ Nuclear imaging - somatostatin receptor positivity – identication of extra abdominal metastatic disease or in cases where primary cannot be identied by CT scan. Ÿ Ga-DOTATATE PET/CT – localize primary tumor in patients with neuroendocrine metastasis of unknown origin & to dene the extent of metastatic disease . Ÿ PET scan using F-DOPA(dihydroxyphenylalanine) - used in identing NETs MEDICAL AND SURGICAL TREATMENT: Ÿ Multidisciplinary Ÿ Surgical debulking, hepatic artery embolization, chemoembolization, or radioembolization and medical therapy Ÿ Treatment of patients with small bowel NET – based on tumor size, site & presence or absence of metastatic disease. Ÿ Lesion <1cm with no evidence of regional LN metastasis – segmental intestinal resection. Ÿ Lesion >1cm, multiple tumors or with regional LN metastasis , regardless of size of primary tumor – wide excision of bowel & mesentery needed. Ÿ Lesion of terminal ileum – right hemicolectomy. Ÿ Small duodenal tumors- locally excised , whereas more extensive tumors needs pancreatoduodenectomy. Ÿ Liver metastasis – trans arterial chemoembolization or arterial embolization. Ÿ OCTREOTIDE & LANREOTIDE & PASIREOTIDE – relieves symptoms Ÿ PEPTIDE RECEPTOR RADIONUCLIDE THERAPY YTTRIUM & LEUTICIUM ISOTOPES conjugated with somatostatin analogues – advanced NETs Ÿ INTERFERON ALPHA &PEGYLATED INTERFERON ALPHA-2b – ce l l cyc le inh ib i t ion a t G1/S & antiangiogenesis effects through downregulation of VEGF. Ÿ CYTOTOXIC CHEMOTHERAPY- STREPTOZOTOCIN, 5FU & CYCLOPHOSPHAMIDE – well differentiated & metastatic NETs. Ÿ KETANSERIN & CYPROHEPTADINE – controls symptoms. Ÿ TELOTRISTAT ETIPRATE – inhibits serotonin synthesis. Ÿ BEVACIZUMAB – inhibits angiogenesis. Ÿ Targeting of multiple signaling pathways is a treatment strategy that may provide better tumor control & overcome resistance mechanisms involved with targeting of a single pathway. CONCLUSION we described a case series of sporadic NEC. An adequate description of NECs should be globally and historically discussed in relation to the real manifestation of this tumor group, considering the evaluation of the Consensus Conference. The diagnosis and treatment of these tumors should be evaluated in large clinical studies. REFERENCES 1. G. Rindi, O. Luinetti, M. Cornaggia, C. Capella, and E. Solcia ; Three subtypes of gastric argyrophil carcinoid and the gastric neuroendocrine carcinoma: a clinicopathologicstudy, Gastroenterology,1993,104 [4], 994–1006. 2. G. Rindi, C. Bordi, S. Rappel, S. la Rosa, M. Stolte, and E. Solcia : Gastric carcinoids and neuroendocrine carcinomas: pathogenesis, pathology, and behavior : World Journal of Surgery, 1996, 20 [2],168–172. 3. G. 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