• Investigative Magnetic Resonance Imaging
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• 제14권2호
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• pp.145-150
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• 2010

소아에서 췌장의 악성 종양은 매우 드물게 발생하고 있으며, 특히 악성 신경내분비 종양은 더더욱 드물다. 저자들은 16세 소아 환자에서 발생한 비기능성 악성 신경내분비 종양의 증례를 경험하여 CT와 MRI 소견을 보고하고자 한다. 췌장 두부에서 발생한 고형 종양으로 조영증강 MRI의 문맥기에서 지연기로 갈수록 조영증강이 잘 되고, 주변의 혈관 침습, 총담관 폐색, 림프절병증 등 악성 소견을 동반할 때 췌장의 비기능성 악성 신경내분비 종양을 감별 진단에 포함하여야 한다.

• Journal of Digestive Cancer Research
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• 제5권1호
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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.

• 대한영상의학회지
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• 제82권5호
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• pp.1297-1303
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• 2021

췌장의 충돌 종양은 매우 드문 종양으로서 췌장선암종과 신경내분비 종양, 췌관내유두상 점액 종양과 신경내분비 종양, 그리고 췌장 고형성 가유두상 종양으로 이루어진 증례들이 보고된 바 있다. 우리는 30세 임신한 여성에서 빠르게 자란, 데스모이드 섬유종증과 점액성 낭성종양으로 이루어진 췌장의 충돌 종양의 증례를 보고하고자 한다. 저자들이 아는 한, 섬유종증과 점액성 낭성 종양으로 이루어진 췌장의 충돌 종양을 최초로 보고하는 증례이다.

• Journal of Digestive Cancer Research
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• 제4권2호
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• pp.51-57
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• 2016

The epidemiology of pancreatic neuroendocrine neoplasms (PNENs) in Asia has been clarified through epidemiological studies, including one conducted in Japan, and subsequently another in South Korea. As endoscopic ultrasonography (EUS) has become more widely accessible, endoscopic ultrasound-fine needle aspiration (EUS-FNA) has been performed in pancreatic tumors for which the clinical course was only monitored previously. This has enabled accurate diagnosis of pancreatic tumors based on the 2010 WHO classification; as a result, the number of patients with an accurate diagnosis has increased. Although surgery has been the standard therapy for PNENs, new treatment options have become available in Japan for the treatment of advanced or inoperable PNENs; of particular note is the recent introduction of molecular target drugs (such as everolimus and sunitinib) and streptozocin. Treatment for progressive PNENs needs to be selected for each patient with consideration of the performance status, degree of tumor differentiation, tumor mass, and proliferation rate. Somatostatin receptor (SSTR)-2 is expressed in many patients with neuroendocrine tumor. Somatostatin receptor scintigraphy (SRS), which can visualize SSTR-2 expression, has been approved in Japan. The SRS will be a useful diagnostic tool for locating neuroendocrine neoplasms, detecting distant metastasis, and evaluating therapy outcomes. In this manuscript, we review the latest diagnostic methods and treatments for PNENs.

• Advances in pediatric surgery
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• 제15권2호
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• pp.180-185
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• 2009

We report a case of nonfunctioning neuroendocrine tumor of the pancreas in a 15-year-old girl who presented with back pain. On physical examination, there was mild tenderness in the left upper quadrant of the abdomen. The patient had no pancreatic hormone-associated symptoms. An abdominal ultrasonography showed a well-demarcated hypervascular solid mass with calcification in the tail of the pancreas. An abdominal computed tomography scan showed a $6{\times}5cm$ sized well-encapsulated enhancing solid mass with cystic component in the tail of the pancreas. Distal pancreatectomy was performed. Pathology revealed awelldifferentiated nonfunctioning low grade malignant neuroendocrine tumor of the pancreas. The postoperative course was uneventful.

• 한국간담췌외과학회지
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• 제27권1호
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• pp.107-113
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• 2023

Mixed adenoneuroendocrine carcinoma is defined as a tumor with a mixture of adenocarcinoma components and neuroendocrine neoplasm components. Each of these two components of mixed adenoneuroendocrine carcinoma accounts for at least 30% of all tumors. Mixed adenoneuroendocrine carcinoma might be located in the ampulla of Vater, a very rare location compared to other organs. Thus, its treatment and prognosis plans have not been established yet. We report three cases of mixed adenoneuroendocrine carcinoma occurring in the ampulla of Vater. Each patient had a different clinical course. In general, difficulty in preoperative diagnosis, risk of early recurrence, and poor disease course were main hallmarks of mixed adenoneuroendocrine carcinoma arising from the ampulla of Vater. However, one patient in this case report survived although she did not receive adjuvant chemotherapy due to her old age. Therefore, it is important to establish a careful treatment strategy for mixed adenoneuroendocrine carcinoma arising from the ampulla of Vater.

• Asian Pacific Journal of Cancer Prevention
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• 제17권5호
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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.

• Asian Pacific Journal of Cancer Prevention
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• 제16권6호
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• pp.2151-2159
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• 2015

Pancreatic neuroendocrine tumors (pNETs) are rare and heterogenous tumors and surgery to remove the primary tumor is the mainstay of treatment for resectable disease. However, curative surgery is often not feasible, because half of patients with pNET have metastases at the time of diagnosis. Palliative dubulking surgery and liver-directed therapies are appropriate options for these patients. Streptozocin-based regimens are standard, although temozolamide-based treatments are rapidly gaining wide clinical application. Somatostatin analogs are mainly indicated in hormonally active tumors to ameliorate symptoms. In addition, anti-tumoral activity has been proven in well-differentiated NETs. Recently, there has been tremendous progress in the molecular biology of pNETs; thereby, the efficacy of sunitinib and everolimus in the treatment of patients with metastatic pNETs has been proven by large placebo-controlled phase III trials. Currently, there are no definitively proven predictive biomarkers to evaluate response to medical therapies in patients with pNET. Therefore, further studies are needed to individualize and optimize their management. This article reviews systemic chemotherapy, targeted therapies, and anti-secretory treatments for the management of patients with unresectable or metastatic pNETs, summarized in the light of recent advances.

• Clinical Endoscopy
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• 제57권3호
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• pp.393-401
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• 2024

Background/Aims: Endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) is a highly accurate method for diagnosing pancreatic neuroendocrine tumors (PNETs); however, some PNETs are difficult to diagnose. Recently, the efficacy of needle-based confocal laser endomicroscopy (nCLE) in diagnosing solid pancreatic masses has been reported. However, the efficacy of nCLE in the diagnosis of PNETs remains unknown and only a small number of cases have been reported. Hence, this study aimed to evaluate the efficacy of nCLE in the diagnosis of PNETs. Methods: This single-center retrospective study evaluated 30 consecutive patients with suspected PNETs on contrast-enhanced computed tomography, who consented to nCLE combined with EUS-FNA and were diagnosed using EUS-FNA or surgical resection. The diagnostic criteria for PNETs using nCLE were based on the nesting and trabecular and glandular arrangement of tumor cell clusters surrounded by capillary vessels and fibrosis, as reported in previous studies. Results: The diagnosis using nCLE was classified into three categories: misdiagnosis in three cases (10%), non-diagnostic in six cases (20%), and diagnostic in 21 cases (70%). nCLE was able to diagnose PNET in one of the two cases with inconclusive EUS-FNA. Conclusions: Although further development of the resolution and optimization of the diagnostic criteria are required, nCLE may constitute a useful diagnostic option in cases of inconclusive EUS-FNA for PNETs.

• Korean Journal of Radiology
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• 제20권1호
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• pp.5-17
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• 2019

The diagnosis and management of pancreatic neuroendocrine neoplasms (NENs) have evolved significantly in recent years. There are several diagnostic and therapeutic challenges and controversies regarding the management of these lesions. In this review, we focus on the recent significant changes and controversial issues regarding the diagnosis and management of NENs and discuss the role of imaging in the multidisciplinary team approach.