Neuroendocrine News

April 2014

In the April 2014 issue of the Journal of the American College of Surgeons, Dr. Choti and colleagues reported that the genetic change of "alternative lengthening of telomeres (ALT+)," can be used to predict the organ of origin in patients who have a neuroendocrine tumor in their liver. In some patients it can be hard to determine if a neuroendocrine tumor in the liver originated in the pancreas, or, for example, in the small bowel. Choti and colleagues used a molecular test for ALT and found that those tumors that originated in the pancreas and spread to the liver were more likely to have ALT than were tumors that arose in the small bowel and spread to the liver. Although a research test, it is hoped that one day this approach will help patients with a metastatic neuroendocrine tumor of unknown origin. PMID: 24655849

April 2014

Collaborators from Johns Hopkins and Memorial Sloan-Kettering Cancer Center reported in the April 2014 issue of the American Journal of Surgical Pathology their experience treating patients with aggressive ("poorly differentiated") neuroendocrine tumors of the pancreas. The authors studied 44 patients with poorly differentiated neuroendocrine tumors and found that they did significantly worse than did patients with well-differentiated (low grade) neuroendocrine tumors. Eighty-eight percent of the patients with poorly differentiated neuroendocrine tumors metastatic disease at presentation, and an additional 7% later developed metastases. Follow-up information was available for 43 patients; 33 died of disease, with an average survival of only 11. The 5-year survival rate was 16.1%. This study highlights the need to accurately grade neuroendocrine tumors, and suggests that patients with poorly differentiated neuroendocrine tumors need to be treated aggressively. PMID: 24503751

November 2013

In the November 2013 issue of the American Journal of Surgical Pathology, Dr. Ralph Hruban and colleagues from the team at Johns Hopkins reviewed the microscopic grading of 297 pancreatic neuroendocrine tumors. Grading of these tumors is performed microscopically and is determined by how fast the tumor cells are dividing. The team found that the combination of two methods for determining how fast the tumor cells are dividing (the "mitotic rate" and the "Ki67 labeling index") was better than either method alone. This study highlights the importance of good pathological review in the evaluation of neuroendocrine tumors. PMID: 24121170

October 2013

Dr. Joanna Law and colleagues at Johns Hopkins report a new way to localize small neuroendocrine tumors of the pancreas in the October issue of Surgical Endoscopy. In this paper Dr. Law and colleagues show that small “fiducials” placed at the time of endoscopic ultrasound can help guide surgeons to the precise location of small neuroendocrine tumors. This more precise localization has the potential for increasing the precision of surgery and reducing the amount of pancreatic tissue that needs to be resected. PMID: 23636530

May 2013

Dr. Trevor Ellison and colleagues reported in the Annals of Surgery the 26-year experience at Johns Hopkins on the surgical treatment of pancreatic neuroendocrine tumors. In this tour de force, Dr. Ellison and colleagues found that tumor grade (how fast the tumor cells are dividing) is the most important predictor of long-term outcome for patients with a neuroendocrine tumor of the pancreas. Importantly, they propose a simple prognostic tool that integrates grade and patient sex. This study reinforces the importance of tumor grade, and highlights the extraordinary experience the Hopkins team has in treating neuroendocrine tumors. PMID: 23673766

February 2013

The team at Johns Hopkins has successfully sequenced all known human genes in a series of medullary carcinomas of the thyroid. As reported in the Journal of clinical endocrinology, Nishant Agrawal and colleagues at Johns Hopkins, in a real tour de force, sequenced the exomes (all 21,000 known human genes) of a series of 17 clinically well-characterized medullary cancers of the thyroid. They then validated their findings in a series of 40 additional cancers. The authors found that the genes RET, HRAS and KRAS are frequently mutated (the DNA is altered abnormally) in these cancers. This study is important because it defines the genetic abnormalities driving the formation of medullary cancers of the thyroid. J Clin Endocrinol Metab. 2013 Feb;98(2):E364-9. PMID: 23264394

February 2013

In the February 2013 issue of the journal, The American Journal of Surgical Pathology, Dr. Chad McCall and colleagues from the Multidisciplinary Neuroendocrine Tumor Team at Johns Hopkins reported that the correct microscopic grading of pancreatic neuroendocrine tumors plays an important role in establishing the prognosis for patients. Dr. McCall and his colleagues found that it is important that pathologists use “Ki-67 stain” to assess tumor grade, in addition to the “old-fashioned” method of simply counting dividing cells (mitoses). PMID: 24121170

August 2012

Dr. Siva Raman and colleagues in Radiology at Johns Hopkins report in the August 2012 issue of the American Journal of Radiology on the imaging appearance of pancreatic neuroendocrine tumors, as well as a number of mimics on computerized tomography (CT). They demonstrate that pancreatic neuroendocrine tumors have a distinct appearance on imaging, and emphasize that experience is needed because a number of other entities can mimic the appearance of a neuroendocrine tumor radiologically. PMID: 22826391

March 2011

In an exciting breakthrough, Dr. Y. Jiao and colleagues at Johns Hopkins reported in the March 2011 issue of Science that they successfully sequenced the exomes (all 21,000 known human genes) in a series of pancreatic neuroendocrine tumors. This represents a major advance in our understanding of the gene changes that drive these tumors. Dr. Jiao and colleagues discovered a new cancer pathway (ATRX and DAXX mutations leading to the alternative lengthening of telomeres) and several gene changes (mutations) that may be targetable (treatable) with currently available drugs. PMID: 21252315