Molecular and Cellular Endocrinology 386 (2014) 1

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Molecular and Cellular Endocrinology journal homepage: www.elsevier.com/locate/mce

Editorial

Genetics and Epigenetics of Endocrine Neoplasia Given the increasing frequency with which nodules in the endocrine systems glands are being detected by a variety of imaging modalities, the distinction of clinically-relevant disease from incidental findings is becoming more relevant. In this issue of Molecular and Cellular Endocrinology, we have gathered experts from around the world who share their perspectives on the latest advancements in our understanding of the different forms of Endocrine Neoplasia, from their genesis to their management. Pituitary tumors represent a prototypical example of a common endocrine neoplasm with wide-ranging clinico-pathological manifestations. Nevertheless, the majority of these sporadic adenomas show a paucity of intragenic activating events. In this regard, Klibanski and co-workers review the epigenetic events in pituitary tumorigenesis. Kopchick and co-workers build on their seminal observations in mouse growth hormone receptor knock-out models to develop rational pharmacotherapeutic strategies for pituitary tumors. Thyroid cancer is the most common malignancy arising from hormone-producing glands. It is currently the 3rd most common malignancy in young women; the vast majority are derived from follicular epithelial cells that represent a model of malignant transformation. Koenig and Colleagues review our current understanding of molecular genetic and epigenetic events in thyroid carcinogenesis. Importantly, they reconcile these findings with well-established histopathologic classifications of thyroid carcinomas and what is emerging in the rapidly developing area of targeted therapeutics for refractory thyroid cancer. Parathyroid tumors also span the spectrum of hyperplasia to adenoma to carcinoma. In particular, the histopathologic distinction in sporadic and familial disorders can often be challenging, leaving major diagnostic questions unanswered. Thakker and colleagues review the latest advances in our understanding of multiple endocrine neoplasia syndromes that involve the parathyroids. Additionally, Arnold and co-workers discuss the latest developments in recognized and novel fusion genes implicated in sporadic forms of these distinct endocrine neoplasms.

0303-7207/$ - see front matter Ó 2013 Elsevier Ireland Ltd. All rights reserved. http://dx.doi.org/10.1016/j.mce.2013.10.026

While adrenal adenomas are common, the almost universally lethal adrenal carcinomas continue to be a therapeutic challenge. Hammer and colleagues review our current understanding of this unique endocrine malignancy with promising new approaches to their classification and therapeutic approaches. Exciting new developments in the succindate dehydrogenase (SDH) isoenzyme family and mitochondrial functions have ushered a new era in our understanding of pheochromocytomas and the closely-related paragangliomas. Pacak and colleagues present a comprehensive new genetic classification system of these endocrine tumor syndromes with increasingly important prognostic and pharmacotherapeutic implications. Neuroendocrine tumors (NETs) of the dispersed endocrine system including pancreatic and intestinal neuroendocrine tumors have recently received unprecedented attention. This has been fueled by advances in their clinic-pathological classifications and peptide imaging and radio-therapeutic strategies. Moreover, successful genome wide sequencing efforts have highlighted the diverse molecular landscape of these neoplastic entities. In this regard, Meeker and co-workers review the implications of these exciting findings for the classification and pharmaco-therapeutic targeting of NETs. We trust that this collection of up-to-date reviews brings seemingly distinct fields of endocrine neoplasia closer together and within clinical and investigative reach. Guest Editors Shereen Ezzat Sylvia L. Asa Ontario Cancer Institute, Princess Margaret Cancer Centre, Canada E-mail address: [email protected] (S. Ezzat) Available online 1 November 2013

Genetics and epigenetics of endocrine neoplasia.

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