Loss of ATRX or DAXX expression and concomitant acquisition of the alternative lengthening of telomeres phenotype are late events in a small subset of MEN-1 syndrome pancreatic neuroendocrine tumors.

Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc
Roeland F de WildeKaren E Matsukuma

Abstract

Approximately 45% of sporadic well-differentiated pancreatic neuroendocrine tumors harbor mutations in either ATRX (alpha thalassemia/mental retardation X-linked) or DAXX (death domain-associated protein). These novel tumor suppressor genes encode nuclear proteins that interact with one another and function in chromatin remodeling at telomeric and peri-centromeric regions. Mutations in these genes are associated with loss of their protein expression and correlate with the alternative lengthening of telomeres phenotype. Patients with multiple endocrine neoplasia-1 (MEN-1) syndrome, genetically defined by a germ line mutation in the MEN1 gene, are predisposed to developing pancreatic neuroendocrine tumors and thus represent a unique model for studying the timing of ATRX and DAXX inactivation in pancreatic neuroendocrine tumor development. We characterized ATRX and DAXX protein expression by immunohistochemistry and telomere status by telomere-specific fluorescence in situ hybridization in 109 well-differentiated pancreatic neuroendocrine lesions from 28 MEN-1 syndrome patients. The study consisted of 47 neuroendocrine microadenomas (<0.5 cm), 50 pancreatic neuroendocrine tumors (≥0.5 cm), and 12 pancreatic neuroendocrine tumor ly...Continue Reading

References

Feb 15, 1997·Cancer·I M Modlin, A Sandor
Sep 11, 2001·Genes, Chromosomes & Cancer·P S MooreA Scarpa
Apr 30, 2002·Human Pathology·Tiziana D'addaCesare Bordi
Jun 1, 2002·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Steven N HochwaldDavid S Klimstra
Sep 4, 2003·Proceedings of the National Academy of Sciences of the United States of America·Yutong XueWeidong Wang
Apr 5, 2005·Cancer Research·Robert A MarciniakF Brad Johnson
May 16, 2006·The American Journal of Surgical Pathology·Martin AnlaufGünter Klöppel
Jun 21, 2008·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·James C YaoDouglas B Evans
Jan 13, 2010·Europace : European Pacing, Arrhythmias, and Cardiac Electrophysiology : Journal of the Working Groups on Cardiac Pacing, Arrhythmias, and Cardiac Cellular Electrophysiology of the European Society of Cardiology·Dimpi PatelAndrea Natale
Jul 24, 2010·Proceedings of the National Academy of Sciences of the United States of America·Peter W LewisC David Allis
Jul 2, 2011·Science·Christopher M HeaphyAlan K Meeker
Sep 6, 2011·The American Journal of Pathology·Christopher M HeaphyAlan K Meeker

❮ Previous
Next ❯

Citations

Jul 4, 2013·Diagnostic Pathology·Antonio ZizziRodolfo Montironi
Feb 23, 2013·Endocrine-related Cancer·A KarpathakisC Thirlwell
Jan 15, 2014·Nature Structural & Molecular Biology·Roderick J O'SullivanJan Karlseder
Jan 1, 2014·Cancer Metastasis Reviews·J CapdevilaA Scarpa
Oct 8, 2014·Annual Review of Cell and Developmental Biology·Dan FilipescuGeneviève Almouzni
Feb 26, 2016·Pancreatology : Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]·Atsuko HadanoTetsuya Mine
Sep 29, 2015·Expert Review of Gastroenterology & Hepatology·Antonio PeaLaura D Wood
Mar 1, 2014·Surgical Pathology Clinics·Michelle D Reid, Barbara A Centeno
Dec 9, 2015·Trends in Cancer·Robert L Dilley, Roger A Greenberg
Mar 30, 2016·Critical Reviews in Oncology/hematology·A ViúdezN Ramírez
Feb 27, 2016·Future Oncology·Alberto ManganoGianlorenzo Dionigi
Sep 15, 2015·Pancreatology : Official Journal of the International Association of Pancreatology (IAP) ... [et Al.]·Irene EspositoGünter Klöppel
Sep 19, 2014·The Journal of Pathology·Brooke E HowittBradley J Quade
Mar 5, 2013·Current Opinion in Genetics & Development·David Clynes, Richard J Gibbons
Mar 31, 2015·Journal of Pathology and Translational Medicine·Laura D Wood, Ralph H Hruban
Jul 15, 2015·The Lancet. Diabetes & Endocrinology·Christopher J YatesRajesh V Thakker
Aug 3, 2013·Molecular and Cellular Endocrinology·Alan Meeker, Christopher Heaphy
Dec 3, 2014·Human Molecular Genetics·Michael A LevyNathalie G Bérubé
Mar 3, 2015·Seminars in Oncology·Arvind RishiRalph H Hruban
Dec 3, 2014·Seminars in Diagnostic Pathology·Chanjuan Shi, David S Klimstra
May 12, 2016·European Journal of Endocrinology·Joakim Crona, Britt Skogseid
May 12, 2016·Journal of Internal Medicine·P BjörklundJ Crona
Oct 19, 2016·Nature Reviews. Clinical Oncology·Mark KiddKjell Öberg
Jun 9, 2016·Diagnostic Pathology·Wenzel M HackengLodewijk A A Brosens
Jun 28, 2016·Human Pathology·Wenzel M HackengLaura D Wood
Jan 8, 2016·Histopathology·Masako IkemuraMasashi Fukayama
Apr 8, 2017·Molecular and Cellular Endocrinology·Justin S GundaraTom Kurzawinski
Oct 6, 2017·World Journal of Gastrointestinal Oncology·Brendan M FinnertyThomas J Fahey Iii
Jun 2, 2018·The Korean Journal of Thoracic and Cardiovascular Surgery·Taek Yong KoSung Dal Park
Jan 13, 2018·The Journal of Endocrinology·Andrea Mafficini, Aldo Scarpa
Jul 16, 2017·Endocrine-related Cancer·Annunziata Di DomenicoAurel Perren
Dec 12, 2018·Pancreas·Bhavina D O Batukbhai, Ana De Jesus-Acosta
Jan 19, 2019·Endocrine Reviews·Andrea Mafficini, Aldo Scarpa

❮ Previous
Next ❯

Related Concepts

Related Feeds

Adenoma, Islet Cell

Islet Cell Adenoma arises in the islet cells, which are insulin producing cells of the pancreas. These tumors can be either malignant or benign. Discover the latest research on Islet Cell Adenoma here.

Cancer Epigenetics Chromatin Complexes (Keystone)

Epigenetic changes are present and dysregulated in many cancers, including DNA methylation, non-coding RNA segments and post-translational protein modifications. The epigenetic changes may or may not provide advantages for the cancer cells. This feed focuses on chromatin complexes and their role in cancer epigenetics.

Carcinoma, Islet Cell (Keystone)

Islet Cell Carcinoma or Pancreatic Neuroendocrine Tumours arise in the hormone producing cells of the pancreas. These tumors can be either malignant or benign. Discover the latest research on islet cell carcinoma in this feed.

Autoimmune Polyendocrine Syndromes

This feed focuses on a rare genetic condition called Autoimmune Polyendocrine Syndromes, which are characterized by autoantibodies against multiple endocrine organs. This can lead to Type I Diabetes.

Cancer Epigenetics and Chromatin (Keystone)

Epigenetic changes are present and dysregulated in many cancers, including DNA methylation, non-coding RNA segments and post-translational protein modifications. The epigenetic changes may or may not provide advantages for the cancer cells. This feed focuses on chromatin and its role in cancer epigenetics please follow this feed to learn more.

Carcinoma, Islet Cell

Islet Cell Carcinoma or Pancreatic Neuroendocrine Tumours arises in the hormone producing cells of the pancreas. These tumors can be either malignant or benign. Discover the latest research on Islet Cell Carcinoma here.