NET Oncogene Patterns Don’t Fit the Classic Mold

Excerpt:

“Matthew H. Kulke, MD, MMSc, has been a leader in the development of new therapies and clinical management strategies for patients with neuroendocrine tumors (NETs).

“In a recent milestone, Kulke presented phase III clinical trial data at the 2015 European Cancer Congress indicating that telotristat etiprate, a novel tryptophan hydroxylase inhibitor, improves diarrhea control in combination with a somatostatin analog for patients with metastatic NETs and inadequately controlled carcinoid syndrome. In the clinical arena, Kulke serves as co-chair for the National Comprehensive Cancer Network guidelines panel on NETs and also is an active member of medical society advisory boards and task forces related to NETs.”

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New Treatments for Gastrointestinal Neuroendocrine Tumors


Neuroendocrine tumors of the digestive system (GI-NETs) can arise in different parts of the digestive tract. GI-NETs originating in the ileum, duodenum, and appendix are known as midgut NETs, and tumors of the stomach, colon, and rectum are non-midgut NETs. Many of these tumors are functional; that is, they produce a variety of hormonal substances that cause serious, debilitating symptoms. Continue reading…


Lu-Dotatate Shows Unprecedented PFS in Midgut NETs

“The radiopharmaceutical Lu-Dotatate (177Lutetium DOTATATE; Lutathera) demonstrated an unprecedented 79% reduction in the risk of progression or death compared with high-dose octreotide LAR (60 mg) in patients with progressive, metastatic midgut neuroendocrine tumors (NETs), according to results from NETTER-1 trial presented by Jonathan Strosberg, MD, at the 2015 NANETS Symposium.”

” ‘The findings were, in my opinion, extraordinarily impressive, the median progression-free survival improved by nearly 80%, which is fairly unprecedented in oncologic studies,’ said Strosberg, a medical oncologist and researcher at the Moffitt Cancer Center. ‘The finding is important because limited therapeutic options exist for such patients, who comprise 20% to 45% of neuroendocrine tumor cases.’ ”

“The NETTER-1 trial is the first prospective, randomized, phase III study for patients with midgut NETs, specifically those in the ileum and cecum. Patients in the trial had progressed on prior therapy with octreotide at 30 mg and had inoperable, somatostatin receptor positive tumors.”


Lexicon's Telotristat Etiprate Shows Clinical Benefit In Treating Carcinoid Syndrome In Cancer Patients When Added To Standard Of Care

“Lexicon Pharmaceuticals, Inc.’s (Nasdaq: LXRX) telotristat etiprate was shown to have clinical benefit in treating carcinoid syndrome in cancer patients not adequately controlled by long-acting somatostatin analog (SSA) therapy, the current standard of care, according to data from the Phase 3 TELESTAR study presented today at the European Cancer Congress in Vienna, Austria.

“Telotristat etiprate, Lexicon’s most advanced product candidate, met the study’s primary endpoint with clinically meaningful reductions in bowel movement frequency in patients whose condition was not adequately controlled by SSA therapy. Carcinoid syndrome is characterized by frequent and debilitating diarrhea that often prevents patients from leading active, predictable lives, as well as by facial flushing, abdominal pain, heart valve damage and other serious consequences.

” ‘We are pleased with the efficacy and safety results of telotristat etiprate and also with the durability of the response shown in this study,’ said Lexicon Executive Vice President and Chief Medical Officer Pablo Lapuerta, M.D. ‘The data also support that the compound is acting directly on the cause of carcinoid syndrome, by reducing serotonin production within tumor cells.’ “


Pancreatic Neuroendocrine Tumors: A Lesser Threat than Adenocarcinomas, but Still Hard to Treat


Pancreatic neuroendocrine tumors (PNETs) constitute only about 3% to 5% of all pancreatic cancers. Compared to the most common pancreatic cancer—adenocarcinoma (aka exocrine tumors), PNETs have a longer disease course and better prognosis; the 5-year survival rate is 42% for PNETs, but only about 5% to 6% for adenocarcinomas. When PNETs are localized, they can usually be removed by surgery. However, PNETs tend to metastasize, most often to the liver, and present a formidable treatment challenge at this stage. Continue reading…


Lung NETs and Their Treatment


Cancers that arise in the lung are mostly of the type known as NSCLC (non-small cell lung carcinoma). A much smaller proportion of lung tumors arise from neuroendocrine cells in the lungs. These cells (which are also found in most other organs) secrete a variety of hormones that are necessary for normal organ function, as well as for healing after injury or infection. Like other lung cells, neuroendocrine cells may transform to become cancers. Lung cancers that arise from neuroendocrine cells are called pulmonary neuroendocrine tumors (NETs), or lung NETs. Continue reading…


Hormone-Mimicking Drugs Are Used To Treat Gastroenteropancreatic Neuroendocrine Tumors (GEP-NETs)


Neuroendocrine tumors (NETs) can arise wherever neuroendocrine (hormone-producing) cells are found—which is in most organs. Most NETs (65%-70%) are gastroenteropancreatic, or GEP, arising in different gastrointestinal organs. GEP-NETs are most commonly found in the small bowel (including the appendix), stomach, and rectum. Still, NETs in general are rare, which complicates the development of new treatments and identification of the genetic drivers of these cancers. Treatment of GEP-NETs is clearly an unmet medical need, and is now even more urgent because their incidence has been on the rise in the last 20 years. Continue reading…


FDA Grants Priority Review to Lanreotide Injection for Gastroenteropancreatic Neuroendocrine Tumors

Editor’s note: In the U.S., a drug must be approved by the U.S. Food and Drug Administration (FDA) in order for it to be prescribed to patients with specific diseases. Particularly promising drugs might be granted Priority Review, meaning that the FDA agrees to work with the drug manufacturer to accelerate the approval process. The FDA recently granted priority review to a drug meant to treat a subset of pancreatic cancer tumors known as gastroenteropancreatic neuroendocrine tumors. The drug is called lanreotide (aka Somatuline Depot). The FDA’s decision was based on promising results for the lanreotide in a clinical trial that tested it in volunteer patients.

“The U.S. Food and Drug Administration (FDA) has accepted and granted priority review to Ipsen’s supplemental New Drug Application (sNDA) for the somatostatin analog lanreotide (Somatuline Depot) 120 mg injection in the treatment of gastroenteropancreatic neuroendocrine tumors. The FDA designates priority review status to drug candidates that have the potential to offer a significant improvement in treatment compared to currently approved options. A decision is expected in early 2015.

“In the United States, lanreotide is indicated for the long-term treatment of patients with acromegaly who have had an inadequate response to or cannot be treated with surgery and/or radiotherapy. The active substance in the drug is lanreotide acetate, a somatostatin analog that inhibits the secretion of several endocrine, exocrine, and paracrine amines and peptides.

“ ‘[Lanreotide] is the first and only somatostatin analog to demonstrate a statistically significant improvement in progression-free survival in patients with gastroenteropancreatic neuroendocrine tumors in a large, multinational clinical trial,’ said Cynthia Schwalm, President and CEO of Ipsen North America.”