Marcela S Cavalcanti, Mithat Gönen, David S Klimstra
{"title":"The ENETS/WHO grading system for neuroendocrine neoplasms of the gastroenteropancreatic system: a review of the current state, limitations and proposals for modifications.","authors":"Marcela S Cavalcanti, Mithat Gönen, David S Klimstra","doi":"10.2217/ije-2016-0006","DOIUrl":"10.2217/ije-2016-0006","url":null,"abstract":"<p><p>The understanding of neuroendocrine neoplasms has evolved significantly since their initial descriptions in the 1800s to early 1900s. In the gastroenteropancreatic system, this group of malignant tumors is subdivided into well and poorly differentiated neuroendocrine neoplasms based on morphologic, proliferative and biologic differences. However, it has become increasingly apparent that well-differentiated neuroendocrine tumors are not a homogeneous group. Attempting to better predict outcome of these tumors has been the motivation behind numerous proposed classification systems, the evolution of which culminated with the currently used system, the ENETS/WHO classification. Herein, we review the genesis of this classification system and some of its shortcomings. In addition, we discuss some of the most recent proposals that suggest modifications to the current system.</p>","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2016-0006","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"36587134","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Mahvash disease: an autosomal recessive hereditary pancreatic neuroendocrine tumor syndrome","authors":"Jane Rhyu, R. Yu","doi":"10.2217/IJE-2016-0005","DOIUrl":"https://doi.org/10.2217/IJE-2016-0005","url":null,"abstract":"Mahvash disease is a rare autosomal recessive hereditary pancreatic neuroendocrine tumor syndrome characterized by inactivating mutations in the glucagon receptor, hyperglucagonemia without symptoms of the glucagonoma syndrome, pancreatic α-cell hyperplasia and pancreatic neuroendocrine tumors. In Mahvash disease, the lack of negative feedback from glucagon receptor signaling results in compensatory hyperplasia of pancreatic α-cells and secondary tumorigenesis. Over ten clinical cases consistent with Mahvash disease have been reported thus far, most of which have confirmed biallelic mutations in the glucagon receptor. Mahvash disease provides insight into glucagon signaling pathway regulation and pathogenesis of pancreatic neuroendocrine tumors.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0005","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217215","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Cabanillas, N. Busaidy, S. Khan, G. Gunn, R. Dadu, S. Rao, S. Waguespack
{"title":"Molecular diagnostics and anaplastic thyroid carcinoma: the time has come to harvest the high hanging fruit","authors":"M. Cabanillas, N. Busaidy, S. Khan, G. Gunn, R. Dadu, S. Rao, S. Waguespack","doi":"10.2217/IJE-2016-0007","DOIUrl":"https://doi.org/10.2217/IJE-2016-0007","url":null,"abstract":"Targeted therapies have played a major role in cancer therapeutics, starting with the discovery of a drug against BCR–ABL rearrangements in chronic myelogenous leukemia. This led to the first approval of a targeted agent in cancer and since, many others have followed. Anaplastic thyroid cancer (ATC) is an aggressive carcinoma with few curative options. Although previous cytotoxic chemotherapy and kinase inhibitor therapies have not proven efficacious in ATC, some of the newer drugs appear to be promising. A case report and a comprehensive review of the current standard of care, genetics, modern therapeutic drugs and clinical trials are presented, in order to outline where we currently stand and where the future lies in the quest for a cure for ATC.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-07-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0007","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217223","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
R. Grogan, B. Aschebrook-Kilfoy, M. G. White, E. Kaplan, P. Angelos
{"title":"Thyroid incidentalomas and the overdiagnosis conundrum","authors":"R. Grogan, B. Aschebrook-Kilfoy, M. G. White, E. Kaplan, P. Angelos","doi":"10.2217/IJE-2016-0008","DOIUrl":"https://doi.org/10.2217/IJE-2016-0008","url":null,"abstract":"","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0008","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68216757","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Should malnutrition screening be routine for patients with GEP-NET?","authors":"M. Weickert","doi":"10.2217/IJE-2016-0013","DOIUrl":"https://doi.org/10.2217/IJE-2016-0013","url":null,"abstract":"","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0013","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68216821","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
S. Vandeva, A. Elenkova, E. Natchev, S. Zacharieva
{"title":"Epidemiological variations of aggressive growth hormone-secreting adenomas","authors":"S. Vandeva, A. Elenkova, E. Natchev, S. Zacharieva","doi":"10.2217/IJE-2015-0011","DOIUrl":"https://doi.org/10.2217/IJE-2015-0011","url":null,"abstract":"Acromegaly is a chronic disorder characterized by increased morbidity and mortality in uncontrolled patients. Growth hormone-secreting pituitary adenoma is the hallmark in the majority of cases, generally considered as benign due to lack of distant metastases. However, clinical behavior in a certain proportion of these adenomas could be quite aggressive, causing difficulties in their management. Aggressive pituitary adenomas have some clinical, radiological, ultrastructural and molecular features in common and they are usually resistant to the standard treatment. In the recent years, efforts have been made to define the most appropriate markers of such adenomas that would allow an early detection and efficient individualized therapeutic strategy. The aim of this review is to give an update on epidemiology and certain markers predicting aggressive behavior of somatotropinomas.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2015-0011","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217108","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Management of pancreatic neuroendocrine tumors","authors":"X. Keutgen, Bruna Babic, N. Nilubol","doi":"10.2217/IJE-2016-0002","DOIUrl":"https://doi.org/10.2217/IJE-2016-0002","url":null,"abstract":"Pancreatic neuroendocrine tumors (pNETs) are rare tumors that have a better prognosis than their exocrine counterpart, but frequently present with advanced disease. Management of pNETs has evolved considerably over the past decade. Surgical resection remains the only potentially curative option for patients with pNETs. Patients who have locoregionally advanced and/or metastatic pNETs require additional treatments. These include liver-directed (transarterial (chemo)-embolization, selective intraarterial radio therapy) and systemic therapies (somatostatin analogs, targeted therapy such as tyrosine-kinase inhibitors and mammalian target of rapamycin inhibitor, peptide receptor radionuclide therapy and cytotoxic chemotherapy). The aim of this article is to review the current treatment options as well as potential future therapeutic perspectives for patients with pNETs.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217156","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Initial surgical management of medullary thyroid cancer","authors":"Kathryn E. Coan, Tracy S. Wang","doi":"10.2217/IJE-2015-0009","DOIUrl":"https://doi.org/10.2217/IJE-2015-0009","url":null,"abstract":"Medullary thyroid cancer (MTC) accounts for 5–10% of thyroid cancer. The majority, 75–80%, of MTC are sporadic with the remainder being hereditary secondary to a mutation in the RET proto-oncogene. Hereditary MTC may be isolated as in familial medullary thyroid cancer or associated with multiple endocrine neoplasia syndrome types 2A and 2B. The primary treatment modality for sporadic MTC is total thyroidectomy and central compartment neck dissection; consideration of lateral neck dissection should be based on preoperative imaging findings. The timing of prophylactic thyroidectomy is dependent on the specific RET codon mutation.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-07-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2015-0009","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217047","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Amir Mehrvarz Sarshekeh, Daniel M Halperin, Arvind Dasari
{"title":"Update on management of midgut neuroendocrine tumors.","authors":"Amir Mehrvarz Sarshekeh, Daniel M Halperin, Arvind Dasari","doi":"10.2217/ije-2015-0004","DOIUrl":"10.2217/ije-2015-0004","url":null,"abstract":"<p><p>Midgut neuroendocrine tumors are typically indolent but can be fatal when advanced. They can also cause significant morbidity due to the characteristic carcinoid syndrome. Somatostatin analogs continue to be the mainstay of treatment given their antiproliferative properties, as well as inhibitory effects on hormones that cause carcinoid syndrome. There have been several recent advances in the systemic therapy of these tumors including consolidation of somatostatin analogs as the cornerstone of therapy, completion of pivotal trials with mTOR inhibitors, and the establishment of novel approaches including peptide receptor radionuclide therapy and oral inhibitors of peripheral tryptophan hydroxylase in tumor and symptom control, respectively. In this review article, the recent advances are summarized and an updated approach to management is proposed.</p>","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2015-0004","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34613949","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Adrenocortical carcinoma: modern management and evolving treatment strategies.","authors":"Lucas A McDuffie, Rachel D Aufforth","doi":"10.2217/ije-2015-0003","DOIUrl":"https://doi.org/10.2217/ije-2015-0003","url":null,"abstract":"Adrenocortical carcinoma (ACC) is a rare cancer with a poor prognosis. Unlike many other cancers, there has been little improvement in patient outcome over the past several decades. However, as scientific advancements are made and our understanding of the molecular genetics involved in ACC improve then progress may be achieved in this devastating disease. This review focuses on recent literature published in the field of ACC from 2010 to 2015 with an emphasis on improving diagnosis, staging and treatment for ACC.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2015-0003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34573563","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}