• Journal of Digestive Cancer Research
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• v.3 no.1
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• pp.21-25
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• 2015

Gastric neuroendocrine tumors (GNETs, also known as gastric carcinoids) are rare form of hormone-secreting neoplasms that present with varied clinical syndromes. There are four types of GNETs based on size, proliferation, localization, differentiation, and hormone production. Type I GNET is related to autoimmune atrophic gastritis and hypergastrinemia. Type II GNETs are related to multiple endocrine neoplasia (MEN)-1, Zollinger-Ellison syndrome and hypergastrinemia. Type 3 GNETs are not associated with any background pathology, and type 4 GNETs are poorly differentiated tumors. The most useful diagnostic and prognostic marker for gastrointestinal NETs is plasma chromogranin A (CgA) levels. Endoscopic ultrasound is the method of choice to determine tumor size and depth of infiltration. For optimal management, the type, biology, and stage of the tumor must be considered. Here, we provide a comprehensive and up-to-date review of GNETs.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.5
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• pp.2679-2681
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• 2016

Background: Gastro-entero-pancreatic neuroendocrine neoplasms (GEP-NEN) are relatively rare tumors, not equally distributed in gastro-intestinal system. In 2010, a revised version of the WHO classification of GEP-NENs was published. This study reports for the first time the distribution and characteristics of GEP-NEN in a Lebanese population. Materials and Methods: This descriptive retrospective study concerns all the digestive neuroendocrine tumors with their characteristics diagnosed in $H\hat{o}tel$ Dieu de France in Beirut, Lebanon from 2001 to 2012, all the pathology reports being reanalyzed according to the latest WHO 2010 classification. The characteristics and features of GEP-NEN analyzed in this study were age, gender, grade and site. Results: A total of 89 GEP-NENs were diagnosed, representing 28.2% of all neuroendocrine tumors. The mean age of GEP-NEN patients was 58.7 years and the M/F sex ratio was 1.2. The primary localization was as follows: 21.3%(19) pancreatic, 18% (16) gastric, 15.7% (14) duodenal, 11.2% (10) appendix, 10.1% (9) intestinal, 10.1% (9) colorectal (7.9% colonic and 2.2% rectal), 5.6% (4) hepatic, 2.2% (2) ampulla, 1.1% (1) esophageal and 7.9%(5) NOS digestive (metastatic with unknown primary). Of the 89 patients with GEP-NEN, 56.2% (50) were diagnosed as grade I, 11.2% (10) as grade II, 20.2% (18) as grade III and 12.4% (11) were considered as mixed adeno-neuroendocrine carcinomas (MANEC). Conclusions: This study, one of the rare examples based on the 2010 WHO classification of neuroendocrine tumors in the literature, indicates that in the Lebanese population, all duodenal and appendicular tumors are G1 and the majority of MANEC tumors are gastric and pancreatic tumors. Moreover, more duodenal tumors and fewer rectal tumors were encountered in our study compared to European reports.

• Journal of Gastric Cancer
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• v.11 no.2
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• pp.122-125
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• 2011

We report a rare case of the coexistence of a gastric small cell neuroendocrine carcinoma with a gastric adenocarcinoma. A 62-year-old man presented with epigastric soreness for 1 month. Esophagogastroduodenoscopy revealed a Borrmann type I tumor at the lesser curvature of the lower body of the stomach. The patient underwent a distal gastrectomy with D2 lymph node dissection and the resected specimen exhibited a $3.5{\times}3.5$ cm sized, fungating lesion. Two separated, not intermingling, lesions with non-adenocarcinoma components encircled by well differentiated adenocarcinoma components were identified microscopically. The non-adenocarcinoma component showed neuroendocrine features, such as a solid and trabecular pattern, and the tumor cells showed a high nuclear grade with minimal cytoplasm, indistinct nucleoli, and positive response for synaptophysin, CD56. The final pathological diagnosis was a gastric mixed exocrine-endocrine carcinoma (MEEC) composed of an adenocarcinoma and small cell neuroendocrine carcinoma of the collision type.

• Journal of Digestive Cancer Research
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• v.5 no.1
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• pp.50-54
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• 2017

Pancreatic neuroendocrine tumors are rare pancreatic neoplasms comprising 1-2% of all pancreatic tumors and typically present high attenuating mass on arterial and venous phase images, due to their rich capillary network. A 70-year-old South Korean female visited our hospital presenting with jaundice and dark urine color. She had received an operation for treatment of small bowel perforation seven years ago. On physical examination, icteric sclera was observed but otherwise unremarkable. Laboratory tests were abnormal liver function test and suspected obstructive jaundice. Computed tomography revealed 4 cm sized cystic mass lesion with homogeneous low attenuation in the head of pancreas and distal common bile duct was compressed by the mass. During review of past medical records, we found that the mass was observed and measured about 1.7 cm seven years ago. To resolve obstructive jaundice, pylorus preserving pancreaticoduodenectomy was performed and diagnosed with well differentiated pancreatic neuroendocrine carcinoma with intermediate grade.

• Journal of Digestive Cancer Research
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• v.9 no.1
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• pp.19-24
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• 2021

The incidence of small and asymptomatic pancreatic neuroendocrine neoplasms (PNENs) has been increased due to the widespread use of high-resolution imaging techniques and endoscopic procedures in screening programmes. Most of PNENs are indolent neoplasms with slow-growing. However, sometimes, PNENs show local invasion or metastasis with poor prognosis. The management of small, nonfunctioning PNENs remain under debate. The National Comprehensive Cancer Network guidelines recommend observation in selected cases of small PNENs less than 2 cm. Pancreatic surgeons are divided into two factions: "the hawks," who indicate the high risk of malignancy even in small PNENs and, therefore, the need for an aggressive surgical treatment, and the "the doves," who accepts the risk of malignancy in some ≤ 2 cm PNENs, advocate that the risk of overtreating many benign ≤ 2 cm PNENs would be much higher. As the pancreatic surgery remains a high-risk operation with a 28-30% morbidity and 1% mortality, the decision for small PNENs is challenging.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.18
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• pp.7835-7838
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• 2014

Background: Neuroendocrine tumors have widespread and different clinical presentations and prognoses. This study was conducted to assess their survival time and prognostic factors in Iran. Materials and Methods: In a retrospective cohort study, 189 patients diagnosed of having neuroendocrine carcinoma were chosen. The tumor and clinical characteristics of the patients were modeled with a Cox proportional hazard approach. Survival was assessed using Kaplan-Meyer curves. Results: Crude median survival time was 30 months. Women survived longer than men (the median survival time for women was 40 and for men was 24 months). Age (<60 vs >60 years old with hazard ratio (HR) of 2.43, 95% CI 1.3-4.5), primary pathology report (carcinoid vs. others with HR 5.85 cm, 95% CI 2.4-14.3), tumor size cm (for 5-10, HR of 3.1, 95% CI 1.6 and for >10 HR of 8.2, 95% with 95% CI 3.1-21.9), and chemotherapy with single drug (taking vs. not taking with a HR 2.2, 95% CI 1.1-4.8) had significant effects on overall survival of patients. Conclusions: Survival time in patients with neuroendocrine carcinomas is related to demographics, clinical characteristics, tumor histology, and subtype specific treatment.

• Journal of Gastric Cancer
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• v.11 no.4
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• pp.234-238
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• 2011

A 67 year old male at a regular checkup underwent esophagogastroduodenoscopy. On performing esophagogastroduodenoscopy, a lesion about 1.2 cm depressed was noted at the gastric angle. The pathology of the biopsy specimen revealed a well-differentiated adenocarcinoma. On performing an abdominal computed tomography (CT) scan & positron emission tomography-computed tomography (PET-CT) scan, no definite evidence of gastric wall thickening or mass lesion was found. However, lymph node enlargement was found in the left gastric and prepancreatic spaces. This patient underwent laparoscopic assisted distal gastrectomy and D2 lymph node dissection. On final examination, it was found out that the tumor had invaded the mucosal layer. The lymph node was a metastasized large cell neuroendocrine carcinoma with an unknown primary site. The patient refused chemotherapy. He opted to undergo a close followup. At the postoperative month 27, he had a focal hypermetabolic lesion in the left lobe of the liver that suggested metastasis on PET-CT scan. He refused to undergo an operation. He underwent a radiofrequency ablation.

• Journal of Digestive Cancer Research
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• v.7 no.2
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• pp.45-50
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• 2019

Incidence of rectal neuroendocrine neoplasms (NEN) has increased tremendously over the decades due to disease awareness and widespread use of screening colonoscopy. Although NEN has been recognized as malignant disease, most rectal NENs are initially found as small mass confined to the submucosa, which can be removed completely through various endoscopic treatments with good prognosis. In this review, we summarize the treatment options focusing on localized T1 rectal NEN by comparing representative international guidelines and discuss current controversies on the management. We also discuss various resection techniques focusing on endoscopic resection.

• Journal of Gastric Cancer
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• v.11 no.4
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• pp.195-199
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• 2011

Purpose: Composite neuroendocrine-exocrine carcinomas are malignancies that have two distinct components residing within the same tumor: an adenocarcinomatous portion and a neuroendocrine portion. This is rare in gastric cancers; however, poorly differentiated adenocarcinomas can sometimes reveal evidence of neuroendocrine features (NEF) or be 'mixed endocrine and exocrine carcinomas'. This study aimed to review NEF in gastric adenocarcinoma and to evaluate its prognostic significance. Materials and Methods: We selected 29 patients who were diagnosed with gastric adenocarcinoma with NEF and received gastrectomies at the Department of Surgery, Ajou University Hospital between January 2001 and December 2009. We analyzed the clinicopathologic features of gastric cancer with NEF and the prognosis associated with such tumors. Results: The pathologic result with respect to TNM staging of the gastric cancers with NEF were as follows: 5 cases of T1, 5 cases of T2, 10 cases of T3, and 9 cases of T4. There were 7 cases of N0, 7 cases of N1, 8 cases of N2 and 7 cases of N3. The staging of patients with NEF was higher than that of patients without NEF. Especially tumor lymphovascular invasion rate was 82.8%. The overall survival of patients with gastric cancer characterized by NEF was 73.8 months. Conclusions: Positive NEF status might be correlated with clinicopathologic parameters such as a high stage and high frequency of regional lymph node metastasis.

• The Korean Journal of Gastroenterology
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• v.72 no.6
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• pp.318-321
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• 2018

Primary neuroendocrine tumors originating from the extrahepatic bile duct are rare. Among these tumors, large cell neuroendocrine carcinomas (NECs) are extremely rare. A 59-year-old man was admitted to Sanggye Paik Hospital with jaundice that started 10 days previously. He had a history of laparoscopic cholecystectomy, which he had undergone 12 years previously due to chronic calculous cholecystitis. Laboratory data showed abnormally elevated levels of total bilirubin 15.3 mg/dL (normal 0.2-1.2 mg/dL), AST 200 IU (normal 0-40 IU), ALT 390 IU (normal 0-40 IU), and gamma-glutamyl transferase 1,288 U/L (normal 0-60 U/L). Serum CEA was normal, but CA 19-9 was elevated 5,863 U/mL (normal 0-37 U/mL). Abdominal CT revealed a 4.5 cm sized mass involving the common bile duct and liver hilum and dilatation of both intrahepatic ducts. Percutaneous transhepatic drainage in the left hepatic duct was performed for preoperative biliary drainage. The patient underwent radical common bile duct and Roux-en-Y hepaticojejunostomy for histopathological diagnosis and surgical excision. On histopathological examination, the tumor exhibited large cell NEC (mitotic index >20/10 high-power field, Ki-67 index >20%, CD56 [+], synaptophysin [+], chromogranin [+]). Adjuvant concurrent chemotherapy and radiotherapy were started because the tumor had invaded the proximal resection margin. No recurrence was detected at 10 months by follow-up CT.