Diala El-Maouche, James Welch, Sunita K Agarwal, Lee S Weinstein, William F Simonds, Stephen J Marx
{"title":"A patient with MEN1 typical features and MEN2-like features.","authors":"Diala El-Maouche, James Welch, Sunita K Agarwal, Lee S Weinstein, William F Simonds, Stephen J Marx","doi":"10.2217/ije-2015-0008","DOIUrl":"https://doi.org/10.2217/ije-2015-0008","url":null,"abstract":"<p><p>Multiple endocrine neoplasia (MEN) type 1 (MEN1) and 2 (MEN2) rarely co-exist in one case. Here we report a patient with features of both syndromes. The patient presented with typical MEN1 features plus pheochromocytoma and thickened corneal nerves. She had a germline 1132delG frameshift mutation in <i>MEN1</i>, no mutation in <i>CDKN1B</i> (<i>p27</i>) and no <i>RET</i> mutation, but had both <i>RET</i> polymorphisms Gly691Ser and Arg982Cys. This is the first case report of a combination of typical clinical findings of MEN1 harboring a germline <i>MEN1</i> mutation and the MEN2-like phenotype with negative full <i>RET</i> gene analysis of pathogenic variants. Possible explanations include a previously unrecognized phenotype-genotype association or the influence of potential phenotypic modifying <i>RET</i> variants. Furthermore, the combination observed in this patient may point to a single molecular pathway, and supports the possibility of as yet unrecognized connections between the molecular pathways for MEN1/menin protein and MEN2/RET protein.</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-2015-0008","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34363335","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":"Ultrasonographically guided, compartment-oriented lymph node dissection in the management of papillary thyroid cancer","authors":"G. Sakorafas, A. Koureas, D. Nasikas","doi":"10.2217/IJE-2016-0001","DOIUrl":"https://doi.org/10.2217/IJE-2016-0001","url":null,"abstract":"Lymphatic metastases are common in papillary thyroid cancer (PTC) at the time of diagnosis [1]. In the presence of synchronous cervical lymphadenopathy, diagnosed clinically or ultrasonographically, the standard surgical approach has been the modified radical lymph node dissection (MRLND), performed at the time of initial total thyroidectomy. This strategy achieves optimal local control of the disease (thereby minimizing local recurrence rates), improves the efficacy of postoperative radioactive therapy (aiming to eradicate potential residual microscopic disease) and facilitates patient’s monitoring during follow-up by measuring serum thyroglobulin [2]. During recent decades, a modern classification of neck lymph nodes has been widely adopted, based on findings and landmarks from cross-sectional anatomic imaging; this is a useful aid in mapping nodal surgical intervention. This classification system is composed of six major nodal regions (levels or compartments I to VI) and defines a compartment-oriented neck dissection. Sublevel classification is also used when certain zones within the larger levels have independent biological significance [3]. Current evidence suggests specific patterns of lymphatic metastases in PTC. Central (compartment VI) lymph nodes are often the first site for lymphatic metastases from PTC, since this level is considered as the first lymphatic basin draining thyroid parenchyma. The most common lateral lymph node regions harboring metastases are levels III and IV [4,5]. Metastases to levels II and V are more rarely observed [6]. Finally, metastases to level I nodes are extremely rare in PTC [6,7]. Recent advances in imaging allow – with acceptable accuracy – the recognition of the presence of pathological lymph nodes within the different compartments of the neck. Currently, high-resolution ultrasonography (US) is being used extensively in the preoperative assessment of cervical lymph nodes (sensitivity 97%, specificity 93%) [8]. The US characteristics of a suspicious lymph node include increased size, round shape (short axis to long axis ratio ≥0.5), absent echogenic hilum, irregular margins, thickened hypoechoic cortex, intranodal calcifications, cystic necrosis, peripheral or mixed vascularity and increased elasticity score. The accuracy of US in recognizing pathologic neck lymph nodes is higher for the lateral nodal compartments, while US evaluation of central compartment lymph nodes has been proved to be of lower sensitivity in detecting metastatic lymph nodes especially in patients with coexistent chronic","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-04-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217142","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":"New systemic therapies for locally advanced and metastatic thyroid cancer","authors":"C. Herrick, J. Moley","doi":"10.2217/IJE-2015-0002","DOIUrl":"https://doi.org/10.2217/IJE-2015-0002","url":null,"abstract":"Thyroid cancer affects one in 100 people over their lifetime. Differentiated and medullary thyroid cancer, refractory to traditional therapy, respond poorly to chemotherapeutic agents. However, tyrosine kinase inhibitors provide new hope for stabilizing disease in patients with advanced progressive disease. There are multiple tyrosine kinase inhibitors under study for thyroid cancer and currently four drugs that are US FDA approved. Nonetheless, use of these drugs should be selective given a significant adverse event profile and diseases with a typically indolent course. This review will cover molecular mechanisms in thyroid cancer as they are relevant to targeted therapies and review available evidence for the safety and efficacy of therapies currently approved and under study for thyroid cancer.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-04-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2015-0002","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217029","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":"Value-based healthcare: implications for thyroid cancer","authors":"A. Ying, T. Feeley, M. Porter","doi":"10.2217/IJE-2015-0005","DOIUrl":"https://doi.org/10.2217/IJE-2015-0005","url":null,"abstract":"Today's delivery of care to thyroid cancer patients is complex, and costly, with uneven outcomes that can be improved. The incidence of thyroid cancer is rising and requires coordinated, multidisciplinary care with high volume centers that is not always available in our current fragmented healthcare system. To address the needs of patients, providers and payers, we believe that thyroid cancer care needs to be reexamined from the perspective of value for the patient, which is defined as the outcomes that matter to patients relative to the cost of delivering them. This paper provides recommendations based on the key principles of the value-based approach to transform the delivery of thyroid cancer care.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2015-0005","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217038","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}
Claudio M Pacella, Sara Nasoni, F. Grimaldi, E. Stasio, I. Misischi, S. Bianchetti, E. Papini
{"title":"Laser ablation with or without chemoembolization for unresectable neuroendocrine liver metastases: a pilot study","authors":"Claudio M Pacella, Sara Nasoni, F. Grimaldi, E. Stasio, I. Misischi, S. Bianchetti, E. Papini","doi":"10.2217/IJE.15.34","DOIUrl":"https://doi.org/10.2217/IJE.15.34","url":null,"abstract":"Aim: To evaluate the effectiveness of laser ablation (LA) with or without selective transarterial chemoembolization in patients with large, isolated or oligonodular unresectable neuroendocrine liver matastases. Materials & methods: Ten patients (mean age: 53.6 years ± 14.1; range: 24–79) with neuroendocrine tumors (NETs) and 13 liver matastases (mean diameter: 4.3 ± 2.8 cm; range: 1.5–12) underwent LA alone (n = 9) or LA followed by selective transarterial chemoembolization (n = 3). Results: Complete response was obtained in six patients with LA alone and in two patients with combined treatment. The 5-year overall survival rates from the initial diagnosis and post-treatment were 80 and 50%, respectively. Conclusion: This treatment modality may provide effective control of tumor burden and general symptoms improvement in patients with limited but unresectable disease.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE.15.34","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68216866","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}
T. Pandian, M. Khatib, Jad M. Abdelsattar, D. Farley
{"title":"Simulation for endocrine surgery training: a call to action","authors":"T. Pandian, M. Khatib, Jad M. Abdelsattar, D. Farley","doi":"10.2217/IJE-2016-0003","DOIUrl":"https://doi.org/10.2217/IJE-2016-0003","url":null,"abstract":"The approach to surgical education has undergone metamorphosis in the last two decades. The mantra, ‘see one, do one, teach one’ has been replaced colloquially with ‘see one, simulate many deliberately, do one’. Simulation has become an integral part of surgical training across many specialties. A large amount of resources, time and expertise have been devoted to the development and study of simulation's role for surgical mastery and improved patient outcomes. Despite significant advances, there is still much to be learned. Many specialties, such as endocrine surgery, are yet to contribute to this effort. Herein, we assess the state of simulation in endocrine surgery and call for experts in the field to unite and join the simulation movement.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2016-0003","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217168","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. Rosenthal, P. Angelos, K. Bible, C. Fassler, S. Finder, L. Greene, M. Tulchinsky
{"title":"Informed consent for low-risk thyroid cancer","authors":"M. Rosenthal, P. Angelos, K. Bible, C. Fassler, S. Finder, L. Greene, M. Tulchinsky","doi":"10.2217/IJE-2015-0010","DOIUrl":"https://doi.org/10.2217/IJE-2015-0010","url":null,"abstract":"Significant barriers to informed consent surround the clinical management of adult patients with well-differentiated thyroid cancer. The literature reveals lack of disclosure surrounding clinical equipoise; confusing and conflicting terminology; and an insufficient number of prospective trials with proper ethical oversight. We provide guidance for valid consent to treatment in this population, and propose stipulative definitions for a variety of terms used in this context. Three critical areas are addressed: surgical management, radioactive iodine management and nonvalidated practice. Sound ethical frameworks for valid consent in patients with low-risk thyroid cancer include consent to observational (or ‘active surveillance’) research protocols, consent to nonvalidated practice and consent when there are opposing standards of care due to insufficient data and disagreement among the community of experts.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-04-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE-2015-0010","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217091","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}
Kirby Tobin, Rachel R Ayers, Mohammad Rajaei, Rebecca S Sippel, Courtney J Balentine, Dawn Elfenbein, Herb Chen, David F Schneider
{"title":"Use of the gamma probe to identify multigland disease in primary hyperparathyroidism.","authors":"Kirby Tobin, Rachel R Ayers, Mohammad Rajaei, Rebecca S Sippel, Courtney J Balentine, Dawn Elfenbein, Herb Chen, David F Schneider","doi":"10.2217/ije.15.27","DOIUrl":"https://doi.org/10.2217/ije.15.27","url":null,"abstract":"<p><strong>Aim: </strong>The purpose of this study was to determine threshold gamma probe counts to distinguish single adenoma (SA) from multigland disease (MGD) during radioguided parathyroidectomy.</p><p><strong>Methods: </strong>A retrospective analysis of 1656 patients was performed. <i>Ex vivo</i> counts of the first excised gland were taken and recorded as a percentage of background counts.</p><p><strong>Results: </strong>69.4% of MGD patients had counts below the 50% threshold. The 50% threshold correctly grouped 72.8% of our cohort. Counts of more than 100% were accurate for grouping SA, with only 6.8% of patients with counts more than 100% having MGD.</p><p><strong>Conclusions: </strong>The gamma probe can aid surgeons in deciding to continue neck exploration if MGD is suspected or wait for labs to confirm cure if SA is suspected.</p>","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/ije.15.27","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"34355922","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":"Surgical management of adrenocorticotropic hormone-secreting pituitary adenomas","authors":"E. Kulubya, D. Donoho, J. Carmichael, G. Zada","doi":"10.2217/IJE.15.26","DOIUrl":"https://doi.org/10.2217/IJE.15.26","url":null,"abstract":"Cushing's disease is defined as hypercortisolemia caused by secretion of adrenocorticotropic hormone from a pituitary adenoma. The diagnosis of Cushing's disease is based on clinical symptoms, laboratory and imaging findings. Once an adrenocorticotropic-hormone-secreting adenoma is found, the standard of care is transsphenoidal adenomectomy. MRI negative microadenomas and large, invasive macroadenomas provide a surgical and clinical challenge and are best handled at high volume centers. Multiple options exist for refractory disease or recurrence, including repeat surgery, radiosurgery and radiotherapy, medical management and bilateral adrenalectomy.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE.15.26","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68217171","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":"Ovarian cancer: is chemotherapy before surgery the way forward?","authors":"S. Kehoe, Rami Fares, A. Phillips","doi":"10.2217/IJE.15.30","DOIUrl":"https://doi.org/10.2217/IJE.15.30","url":null,"abstract":"The treatment of advanced ovarian cancer is a combination of surgery and platin-based chemotherapy, with surgery traditionally the first intervention. Neoadjuvant therapy was only used in women who were deemed unfit for an operation. However, in recent years, two prospective randomized studies have evaluated the concept of neoadjuvant chemotherapy, and in both studies the results revealed that neoadjuvant chemotherapy with delayed surgery afforded similar survival rates compared with primary surgery but with reduced treatment-related morbidity. Thus, a novel evidence-based alternative approach to advanced ovarian cancer is now available.","PeriodicalId":42691,"journal":{"name":"International Journal of Endocrine Oncology","volume":null,"pages":null},"PeriodicalIF":1.2,"publicationDate":"2016-01-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/IJE.15.30","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"68216749","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}