• Asian Pacific Journal of Cancer Prevention
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• v.16 no.6
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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.

• Nuclear Medicine and Molecular Imaging
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• v.40 no.2
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• pp.90-95
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• 2006

Neuroendocrine tumors (NETs) consist of a heterogeneous group of tumors that are able to uptake neuroamine and/or specific receptors, such as somatostatin receptors, which can play important roles of the localization and treatment of these tumors. When considering therapy with radionuclides, the best radioligand should be carefully investigated. $^{131}I$-MIBG and beta-particle emitter labeled somatostatin analogs are well established radionuclide therapy modalities for NETs. $^{111}In,\;^{90}Y\;and\;^{177}Lu$ radiolabeled somatostatin analogues have been used for treatment of NETs. Further, radionuclide therapy modalities, for example, radioimmunotherapy, radiolabeled peptides such as minigastrin are currently under development and in different phases of clinical investigation. for all radionuclides used for therapy, long-term and survival statistics are not yet available and only partial tumour responses have been obtained using $^{131}I$-MIBG and $^{111}In$-octreotide. Experimental results using $^{90}Y$-DOTA-lanreotide as well as $^{90}Y-DOTA-D-Phe1-Tyr^3-octreotide$ and/or $^{177}Lu-DOTA-Tyr^3-octreotate$ have indicated the possible clinical potential of radionuclides receptor-targeted radiotherapy it may be hoped that the efficacy of radionuclide therapy will be improved by co-administration of chemotherapeutic drugs whose antitumoral properties may be synergistic with that of irradiation.

• The Korean Journal of Cytopathology
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• v.7 no.2
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• pp.213-217
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• 1996

Medullary carcinoma of the thyroid gland is a malignant neuroendocrine tumor arising from calcitonin producing-parafollicular cells. The tumor is clinically divided into sporadic and familial form, constituting about 80% and 20%, respectively. Recently, we experienced a case of unilateral and solitary sporadic medullary carcinoma of the left thyroid gland. The patient was a 9 year-old female, who presented with a palpable mass on the anterior lateral neck of 8 months duration without any familial and personal history of neuroendocrine disease. The cytopathologic findings showed spindle cells and plasmacytoid cells in the background of colloid-like materal. The nuclei were eccentrically located, mildly hyperchromatic and pleomorphic, showing speckled chromatin pattern without nuclear inclusion or prominent nucleoli. The cytoplasm was abundant and had a pale granular cyanophilic appearance. No amyloid materal could be identified.

• Korean Journal of Head & Neck Oncology
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• v.28 no.2
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• pp.149-152
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• 2012

Paraganglioma of the thyroid is a neuroendocrine tumor originating from the neural crest. To our knowledge, only 32 cases of thyroid paraganglioma(TP) have been reported to date. TP is difficult to distinguish from medullary thyroid cancer(MTC), hence correct diagnosis is seldom obtained preoperatively. Although immunohistochemistry is helpful for confirmation of TP, these markers are not specific for differential diagnosis between TP and MTC. Therefore, an index of suspicion is important when encountered with MTC with unusual features, or given pathology with neuroendocrine features and unusual immunohistochemical findings. The authors report a case of TP misdiagnosed as MTC at preoperative work-up and intraoperative frozen section analysis, with a review of the literature. The case depicts various immunohistochemical characteristics of the tumor.

• Radiation Oncology Journal
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• v.15 no.4
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• pp.369-377
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• 1997

Purpose : This study was Performed to identify the histopathologic feature by the reevaluation of the Pathologic specimen of the cervical tumors and to evaluate the clinical findings and the treatment results of the patients with small cell carcinoma of the cervix treated by radiotherapy. Materials and Methods : 2890 patients with cervical carcinoma received radiotherapy at the Department of Radiation Oncology. Yonsei Cancer Center, Yonsei University College of Medicine between October 1981 and April 1995. Of the 2890 patients in this data base, sixty were found to have small cell carcinomas $(2.08\%)$. Among them thirty six patients were transferred from other hospitals. the biopsy specimens of those Patients were not available. So we could review the slides of the other twenty four patients who were diagnosed at our hospital. Twenty four patients with small cell carcinoma of the cervix were analyzed retrospectively based on the assessment of H & E staining and other four immunohistochemical stains for neuroendocrine differentiation (neuron specific enolase, chromogranin. synaptophysin and Grimelius stain). And we also evaluate the Patients and tumor characteristics. response to radiation. patterns of failures, 5 year overall and disease free survival rates. Results : Thirteen tumors were neuroendocrine carcinomas(13/24 = $54.2\%$) and eleven tumors were squamous carcinomas, small cell type (11/24 = $47.8\%$) based on the assessment of H & E staining and other four neuroendocrine marker studies. So we classified the Patients two groups as neuroendocrine carcinoma and small cell type of squamous carcinoma, Among the 13 neuroendocrine carcinomas, five were well to moderately differentiated tumors and the other eight were Poorly differentiated or undifferentiated ones. The median age was 54 years old (range 23-79 years). Eight Patients had FIGO stage IB disease, 12 had stage 11, 3 had stage III and one had stage IV disease, Pelvic lymph node metastases were found in five Patients $(20.8\%)$. three of them were diagnosed by surgical histologic examination and the other two were diagnosed by CT scan. There was no difference between two histopathologic groups in terms of patients and tumor characteristics. response to radiation. 5 year overall and disease free survival rates. However the distant metastases rate was higher in neuroendocrine carcinoma Patients (6/13:$46.2\%$) than in small cell type of squamous carcinoma Patients (2/11:$18.2\%$), but there was no statistically significant difference because of the small number of patients (P>0.05). Conclusion : More than half of the small cell carcinoma of the cervix patients were neuroendocrine carcinoma (13/24 : $54.1\%$) by reevaluation of the biopsy specimen of the cervical tumors. The tendency of distant metastases of the neurolndocrine carcinoma was greater than those of the small cell type of squamous carcinoma $(46.2\%\;vs.\;18.2\%)$. But there were no differences in the patients and tumor characteristics and other clinical treatment results in both groups. These data suggest that radical local treatment such as radiotherapy or radical surgery combined with combination systemic cytotoxic chemotherapy might provide these patients with the best chance for cure.

• The Korean Journal of Nuclear Medicine
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• v.33 no.1
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• pp.11-27
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• 1999

Peptide imaging is a new diagnostic modality in nuclear medicine. $^{111}In$-pentetreotide ($Octreoscan^R$) is the first commercially available peptide radiopharmaceutical. This review article presents the results of previous studies using $^{111}In$-pentetreotide for several disease states, including neuroendocrine tumors, breast cancer and malignant lymphoma. The use of hand-held probe during surgery and the preliminary results of radiotherapy using radiolabeled somatostatin analogues are also reviewed. It can be concluded that somatostatin receptor scintigraphy is a promising diagnostic tool for localizing primary tumors that express receptors for somatostatin, staging secondary spread of tumor tissue, following up after therapy and identifying patients who may benefit from therapy with unlabelled or radiolabeled octreotide. The somatostatin receptor imaging will stimulate the development of new radiopharmaceuticals for other receptors and enhance the therapeutic use of radiolabeled peptides.

• Journal of Digestive Cancer Reports
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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 Yeungnam Medical Science
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• v.27 no.2
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• pp.98-104
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• 2010

Primary anterior mediastinal neoplasms comprise a diverse group of tumors and account for 50% of all mediastinal masses. Thymic epithelial neoplasm are most common and classified into thymoma, invasive thymoma, and thymic carcinoma. Neuroendocrine differentiation of thymic epithelial neoplasm are rare malignancies. Germ cell tumor (GCT) is second most common anterior mediastinal tumor and most of them are mature cystic teratoma. Malignant mediastinal GCT are rare than benign. Primary thoracic lymphoma is rare than thoracic involvement of systemic lymphoma and most common location of primary thoracic lymphoma is anterior mediastinum. The clinical and radiologic appearance of the most common masses are reviewed.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.6
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• pp.2541-2546
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• 2014

Background: Primary hepatic neuroendocrine carcinoma (PHNEC) is rarer than extrahepatic gastrointestinal neuroendocrine carcinoma (NEC). It is difficult to make a correct diagnosis and poses a challenge for management. Materials and Methods: Ten PHNEC patients were admitted to our hospital from June 2006 to June 2011. Laboratory tests and imaging scans were performed for diagnosis and exclusion of extrahepatic NEC. All patients were AFP - and CA199-. Seven patients had solid tumors with cystic changes on ultrasonography, CT and/or MRI. For the initial treatment, four patients received combined-therapy and six monotherapy. Considering overall treatment, six patients received combined-therapy and four patients monotherapy. Staging criteria of primary hepatocellular carcinoma (PHC, AJCC 7th edition) were used to differentiate the stage of all patients: 3 patients were stage I, 2 stageII, 4 patients stageIII and 1 stageIV. All patients were followed up and clinical data were gathered. Results: The median follow-up duration was 38.5 months. The 1-year, 2-year, 3-year and 6-year disease-free survival was 80.0%, 46.2% and 46.2% and 0% respectively. The overall survival rates were 100%, 67.1%, 67.1% and 33.6% respectively. Patients in early-stages (I/II) had similar disease-free and overall survival as those in advanced-stages (III/IV). Patients with monotherapy had significant shorter disease-free and overall survival than the patients with combination-therapy. Conclusions: PHNEC has a unique specificity during its occurrence and development. The staging criteria of PHC might not be suitable for the PHENT. More convenient and effective features need to be found in imaging and laboratory detection. Surgical resection, TACE, chemotherapy and radiofrequency ablation should be performed in combination and actively for patients with PHNEC or recurrence to get the best effectiveness; they might extend the disease-free and overall survival.

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.11
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• pp.6687-6692
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• 2013

Background: This study aimed to determine the demographical distribution, survival and prognostic factors for neuroendocrine tumors monitored in our clinic. Materials and Methods: Data for 52 patients who were admitted to Cumhuriyet University Medical Faculty Training Research and Practice Hospital Oncology Center between 2006 and 2012 and were diagnosed and treated for neuroendocrine tumors were investigated. Results: Of the total, 30 (58%) were females and 22 (42%) were males. The localization of the disease was gastroenteropancreatic in 29 (56%) patients and other sites in 23 (44%). The most frequently involved organ in the gastroenteropancreatic system was the stomach (n=10, 19%) and the most frequently involved organ in other regions was the lungs (n=10, 19%). No correlation was found between immunohistochemical staining for proteins such as chromogranin A, synaptophysin, and NSE and the grade of the tumor. The patients were followed-up at a median of 24 months (1-90 months). The three-year overall survival rate was 71%: 100% in stage I, 88% in stage II, 80% in stage III, and 40% in stage IV. The three-year survival rate was 78% in tumors localized in the gastroenteropancreatic region, and 54% in tumors localized in other organs. In the univariate analysis, gender, age, performance status of the patients, grade, localization, surgical treatment, and neutrophil/lymphocyte ratio (${\leq}5$ versus >5) affected the prognosis of the patients. Conclusions: Most of the tumors were localized in the gastroenteropancreatic region, and the three-year survival rate in tumors localized in this region was better than the tumors localized in other sites. Surgical treatment was a positive independent prognostic factor, whereas Grade 3 and a neutrophil/lymphocyte ratio of >5 were negative independent prognostic factors.