P. Antonodimitrakis, H. Olofsson, L. Grimelius, A. Sundin, C. Wassberg, D. Granberg, B. Skogseid, B. Eriksson
{"title":"Neuroendocrine tumors with syndromic vasoactive intestinal polypeptide hypersecretion: a retrospective study","authors":"P. Antonodimitrakis, H. Olofsson, L. Grimelius, A. Sundin, C. Wassberg, D. Granberg, B. Skogseid, B. Eriksson","doi":"10.2217/IJE-2016-0012","DOIUrl":"https://doi.org/10.2217/IJE-2016-0012","url":null,"abstract":"Aim: Vasoactive intestinal polypeptide producing neuroendocrine tumors are rare and cause severe hormonal symptoms. Patients/methods: Eighteen patients with vasoactive intestinal polypeptide producing neuroendocrine tumors were analyzed with reviews of medical records, radiology and tumor tissue specimens. Results: Twelve patients (67%) had liver metastases at diagnosis. Chemotherapy, somatostatin analogs and interferon were given as medical therapies. Streptozocin/5-fluorouracil produced an objective response in 40% of the evaluable patients. Somatostatin analogs gave a clinical/biochemical response in eight out of nine patients. Transarterial embolization of the liver and peptide receptor radionuclide therapy was given to refractory cases. Sixteen patients died during the observation period. The median overall survival from diagnosis was 102 months. Conclusion: Systemic chemotherapy and somatostatin analogs should be given in cases of advanced disease or for hormonal symptoms.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"4 1","pages":"9-22"},"PeriodicalIF":1.2,"publicationDate":"2017-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0012","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"46522612","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":"Update on microRNA as biomarkers of adrenocortical cancer: perspective on circulating microRNA","authors":"P. Igaz","doi":"10.2217/IJE-2017-0001","DOIUrl":"https://doi.org/10.2217/IJE-2017-0001","url":null,"abstract":"","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"4 1","pages":"1-3"},"PeriodicalIF":1.2,"publicationDate":"2017-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2017-0001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"48479946","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":"Gastro-entero-pancreatic neuroendocrine tumors in multiple endocrine neoplasia type 1: a therapy update","authors":"F. Tonelli, F. Marini, F. Giusti, M. Brandi","doi":"10.2217/IJE-2016-0017","DOIUrl":"https://doi.org/10.2217/IJE-2016-0017","url":null,"abstract":"Gastro-entero-pancreatic neuroendocrine tumors (GEP-NETs) are the second most common tumors in multiple endocrine neoplasia type 1 (MEN1), mainly occurring in pancreatic islets and duodenum, usually as multiple tumors. They can manifest as both nonfunctioning and functioning tumors. Currently, surgical removal of GEP-NETs in MEN1 represents the gold standard curative approach. Conventional medical therapies for sporadic GEP-NETs showed to be effective also in a percentage of MEN1 patients. Innovative medical therapies, that have demonstrated to be effective on sporadic GEP-NETs, still need to be evaluated on MEN1 patients in prospective clinical trials and long-term follow-up. This review resumes current knowledge of MEN1 GEP-NETs, discussing surgical and medical approaches, genetic and molecular bases of tumorigenesis, and presenting novel possible drug therapies.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"4 1","pages":"43-58"},"PeriodicalIF":1.2,"publicationDate":"2017-03-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0017","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41787406","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}
David C Metz, Guillaume Cadiot, Pierre Poitras, Tetsuhide Ito, Robert T Jensen
{"title":"Diagnosis of Zollinger-Ellison syndrome in the era of PPIs, faulty gastrin assays, sensitive imaging and limited access to acid secretory testing.","authors":"David C Metz, Guillaume Cadiot, Pierre Poitras, Tetsuhide Ito, Robert T Jensen","doi":"10.2217/ije-2017-0018","DOIUrl":"https://doi.org/10.2217/ije-2017-0018","url":null,"abstract":"<p><p>In recent years the diagnosis of Zollinger-Ellison syndrome (ZES) has become increasingly controversial with several new approaches and criteria proposed, differing from the classical biochemical criterion of inappropriate hypergastrinemia (i.e., hypergastrinemia in the presence of hyperchlorhydria) (Table 1). These changes have come about because of the difficulty and potential dangers of stopping proton pump inhibitors (PPIs) for gastric acid analysis; the recognition than many of the current assays used to assess gastrin concentrations are unreliable; the development of sensitive imaging modalities that detect neuroendocrine tumors (NETs) including an increasing number of the primary gastrinomas; the increased use of percutaneous or endoscopic ultrasound (EUS)-directed biopsies/cytology and the general lack of availability of acid secretory testing. In this article we will discuss the basis for these controversies, review the proposed changes in diagnostic approaches and make recommendations for supporting the diagnosis of ZES in the modern era.</p>","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"4 4","pages":"167-185"},"PeriodicalIF":1.2,"publicationDate":"2017-01-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije-2017-0018","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"35731178","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}
H. Jedidi, L. Rostomyan, I. Potorac, F. Depierreux, P. Petrossians, A. Beckers
{"title":"Advances in diagnosis and management of familial pituitary adenomas","authors":"H. Jedidi, L. Rostomyan, I. Potorac, F. Depierreux, P. Petrossians, A. Beckers","doi":"10.2217/IJE-2016-0009","DOIUrl":"https://doi.org/10.2217/IJE-2016-0009","url":null,"abstract":"Familial pituitary adenomas account for approximately 5–8% of all pituitary adenomas. Besides the adenomas occurring as part of syndromic entities that group several endocrine or nonendocrine disorders (multiple endocrine neoplasia type 1 or 4, Carney complex and McCune–Albright syndrome), 2–3% of familial pituitary adenomas fit into the familial isolated pituitary adenomas (FIPA) syndrome, an autosomal dominant condition with incomplete penetrance. About 20% of FIPA cases are due to mutations in the AIP gene and have distinct clinical characteristics. Recent findings have isolated a new non-AIP FIPA syndrome called X-linked acrogigantism, resulting from a microduplication that always includes the GPR101 gene. These new advances in the field of pituitary disease are opening up a new challenging domain to both clinicians and researchers. This review will focus on these recent findings and their contribution to the diagnosis and the management of familial pituitary adenomas.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"3 1","pages":"313-323"},"PeriodicalIF":1.2,"publicationDate":"2016-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0009","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68216767","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":"Signaling pathways in medullary thyroid carcinoma: therapeutic implications","authors":"Zahra Nozhat, M. Hedayati, Hoda Pourhassan","doi":"10.2217/IJE-2016-0014","DOIUrl":"https://doi.org/10.2217/IJE-2016-0014","url":null,"abstract":"Medullary thyroid cancer (MTC) is the third most frequent thyroid cancer arising from thyroid parafollicular cells. Surgery is the first-line strategy in treatment of MTC but disease relapse and patient's death have been observed in approximately two out of three of MTC cases. Identification of molecular mechanisms and different signaling pathways has offered new insights for disease treatment. The development of tyrosine kinase inhibitors targeting these pathways has provided a promising landscape for prevention of progression in patients with advanced metastatic MTC. In this review article different altered molecular pathways implicated in the development of MTC and the therapeutic strategies based on targeting the identified signaling pathways have been summarized.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"3 1","pages":"299-312"},"PeriodicalIF":1.2,"publicationDate":"2016-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0014","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217335","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":"Durable response to lenvatinib in progressive, therapy-refractory, metastatic paraganglioma","authors":"S. Jasim, A. Chintakuntlawar, K. Bible","doi":"10.2217/IJE-2016-0015","DOIUrl":"https://doi.org/10.2217/IJE-2016-0015","url":null,"abstract":"Paragangliomas are rare, often vascular, endocrine tumors that are sometimes malignant. Antiangiogenic agents may play a role in treating advanced disease. We report a case of a 49-year-old woman with metastatic and rapidly progressive secretory paraganglioma (with germ line SDHB p.V140F–c.418G>T mutation) who responded rapidly and durably to the VEGFR- and multi-targeted kinase inhibitor, lenvatinib, despite progression through prior cytotoxic and kinase inhibitor therapy. She incurred dramatic response within 48 h of lenvatinib initiation, but developed parallel transient mild tumor lysis syndrome and, later, localized radiation ‘recall’ pneumonitis. Follow-up assessments demonstrated a confirmed response evaluation criteria in solid tumors response as well as symptomatic and biochemical improvement. Lenvatinib may represent a promising therapeutic for further development in treating metastatic paraganglioma.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"3 1","pages":"285-289"},"PeriodicalIF":1.2,"publicationDate":"2016-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0015","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217344","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":"Resection of primary gastroenteropancreatic neuroendocrine tumors in the presence of irresectable liver metastases","authors":"Philippa K Wadsworth, Robert P. Jones, G. Poston","doi":"10.2217/IJE-2016-0010","DOIUrl":"https://doi.org/10.2217/IJE-2016-0010","url":null,"abstract":"Gastroenteropancreatic neuroendocrine tumors often present at an advanced stage and carry a variable prognosis, with many patients living long periods even with metastatic disease. Resection of the primary tumor when liver metastases are unresectable is a contentious subject with no consensus between existing guidelines. A number of recent studies have suggested a potential survival benefit as well as improved symptom control with resection. With variable results from nonoperative management strategies, the risk:benefit ratio of resection of the primary tumor may be favorable for carefully selected patients.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"12 1","pages":"291-297"},"PeriodicalIF":1.2,"publicationDate":"2016-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0010","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68216777","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}
A. Violante, T. Ortiga-Carvalho, M. H. Costa, M. Vaisman
{"title":"Treatment of pheochromocytomas and paragangliomas: genetic approach?","authors":"A. Violante, T. Ortiga-Carvalho, M. H. Costa, M. Vaisman","doi":"10.2217/IJE-2016-0016","DOIUrl":"https://doi.org/10.2217/IJE-2016-0016","url":null,"abstract":"Pheochromocytomas (PHEOs) and paragangliomas (PGLs) are neuroendocrine tumors derived from the chromaffin tissue of the adrenal medulla or from extra-adrenal sympathetic and parasympathetic paraganglia. These tumors affect one in 2500–6500 people, with 500–1600 patients diagnosed annually in the USA. Its real incidence is likely to be much higher, and many patients may die undiagnosed. It is a rare cause of secondary hypertension, with an incidence of 0.1–0.6%. PHEOs are more common than PGLs, and 5–7% is related to hormonal secretion. The average age of diagnosis is 43 years, but 10–20% of PHEO/PGL patients are children, and the tumors are generally associated with genetic conditions. The worldwide consensus is that people with PHEOs/PGLs should undergo a mandatory genetic investigation, especially with multiple injuries and those who are younger. Although many PHEOs are sporadic and benign, approximately 30% of familial tumor cases have been identified. Multiple endocrine neoplasia type 2, von Hippel–Li...","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"3 1","pages":"325-331"},"PeriodicalIF":1.2,"publicationDate":"2016-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0016","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217355","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":"Akt as a potential prognostic marker in neuroendocrine tumors: a possibility?","authors":"A. Hague, Helen L Robbins","doi":"10.2217/IJE-2016-0019","DOIUrl":"https://doi.org/10.2217/IJE-2016-0019","url":null,"abstract":"","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":"110 9 1","pages":"281-284"},"PeriodicalIF":1.2,"publicationDate":"2016-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0019","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217391","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}