10 Highlights of the Year 2013 for the Carcinoid and Neuroendocrine Tumor Community

As the Carcinoid Cancer Foundation looks back on the year 2013, we reached out to physicians, patients, support group leaders, listserv managers, patient advocates, and business colleagues to see what they thought were among the highlights of the past year.  There were many significant advances and events in the carcinoid and neuroendocrine tumor (NET) community.  Here are 10 of the year’s highlights as shared with us:

1)      PRRT Clinical Trial

For the first time, U.S. patients from the East Coast to the West Coast have the PRRT NETTER-1 Clinical Trial for Neuroendocrine Tumor Patientsopportunity to participate in a PRRT clinical trial, the new 177Lu-DOTA0-Tyr3-Octreotate Phase III clinical trial also known as NETTER-1. This international study is a multicenter randomized clinical trial in patients with inoperable progressive midgut carcinoid. The NETTER-1 study will evaluate the efficacy and safety of Lutathera® compared to high dose Octreotide LAR in patients with inoperable, progressive, somatostatin receptor positive, midgut carcinoid tumors.  The primary objective of the trial is the assessment of Progression-Free Survival (PFS).  Also being studied are safety, Objective Response Rate (ORR), Time to Tumor Progression (TTP), Overall Survival (OS), and Quality of Life (QoL). Lutathera®, Lu-177 Octreotate, is a radiolabeled somatostatin analog that selectively targets somatostatin receptors which are over-expressed in differentiated neuroendocrine tumors. The Lu-177 is delivered directly into the tumor cell, acting like a Trojan horse.  A Trojan horse in cancer therapy is the use of nanotechnology where cancer-fighting particles are targeted only for the cancer cells, leaving healthy cells alone. Lutathera® also releases a gamma ray, which exits the body and enables physicians to image and evaluate the progress of the treatment via a SPECT (Single Photon Emission Computer Tomography) camera.

2)      CLARINET Study and Lanreotide

New findings from the CLARINET (Placebo-Controlled Study of Lanreotide Antiprolierative Response in Patients with GEP-NET) Phase III trial demonstrate that the somatostatin analog lanreotide significantly prolonged progression-free survival in patients with nonfunctioning gastroenteropancreatic (GEP) neuroendocrine tumors (NETS) compared to treatment with placebo.  The CLARINET study builds upon the earlier PROMID prospective trial.   A much larger, multicenter, randomized, double-blind trial, CLARINET involved 204 patients, with a mean age of 63 in the lanreotide arm and 62 in the placebo arm.  Dr. Kjell Oberg, NET cancer specialist from University Hospital Uppsala in Sweden, reported the results at the European Cancer Congress 2013.  Read more here.

3)      Carcinoid Syndrome and TELESTAR Trial

For patients who suffer from carcinoid syndrome – with symptoms such as diarrhea, frequent bowel movements, abdominal pain, and flushing – that is not adequately controlled by their current somatostatin analog therapy, the global Phase III clinical trial of telotristat etiprate is a study of an investigational oral treatment that inhibits the key enzyme responsible for the production of serotonin, tryptophan hydroxylase (TPH).  This unique approach reduces the serotonin produced by functioning carcinoid neuroendocrine tumors (NETs) and helps to control the debilitating effects of carcinoid syndrome. For more details about the clinical trial, including criteria for participation, visit: http://clinicaltrials.gov/ct2/show/NCT01677910?term=Telestar&rank=1.

Phase III Clinical Trial of Teo

4)      Orphan Drug Designation for Gallium-68

The radiopharmaceutical Gallium-68 (DOTA0-hel-Tyr3) octreotide (Ga-68 DOTATOC) has been designated as an orphan drug by the US Food and Drug Administration (FDA) for the management of neuroendocrine tumors. This designation is significant because it may lead to faster approval of the agent, which would greatly benefit carcinoid/NET patients in the United States. Read more from the Society of Nuclear Medicine and Molecular Imaging and from Josh Mailman, chairman of the Society’s Patient Advocacy Advisory Board and president of the NorCal CarciNET support group in California, http://interactive.snm.org/index.cfm?PageID=13165. From the FDA, read more about how drugs are designated as an orphan product.

5)       Genomic Studies and Molecular Profiling

From labs at the Carcinoid and Neuroendocrine Tumor Program, Cedars-Sinai in Los Angeles, California to the Ochsner Neuroendocrine Tumor Program in Kenner, Louisiana ongoing studies of how genes effect growth in neuroendocrine tumors are critical in diagnosing, measuring, and treating NETs.  One of the newest developments is the measurement of genes in simple blood samples for diagnosing or measuring carcinoid and other NETs, The Identification of Gut Neuroendocrine Tumor Disease by Multiple Synchronous Transcript Analysis in Blood.

Molecular profiling and neuroendocrine tumors Molecular profiling of neuroendocrine tumors is becoming more widely available and may be useful in offering personalized treatments for neuroendocrine tumor patients.  This is a method of testing that examines a person’s tumors and studies the genetic characteristics as well as any unique biomarkers. The information gathered is used to identify and create targeted therapies designed specifically for an individual based upon his/her tumors.

6)      NET Cancer Day, November 10, 2013

Now in its 4th year, Worldwide NET Cancer Awareness Day focuses on raising awareness and education of carcinoid and other rare neuroendocrine cancers, including pancreatic neuroendocrine tumors, paraganglioma, pheochromocytoma, and multiple endocrine neoplasia (MEN).  This global effort is sponsored by the International Neuroendocrine Cancer Alliance (INCA), comprised of patient advocate/patient organizations from Australia to the United States.  The 2013 Name Your NET Hero campaign spotlighted doctors, nurses, spouses, friends, partners, colleagues, and loved ones considered heroes — people whose support, faith and positive energy are crucial in one’s journey through life – in the NET community. In the United States 42 US Governors issued proclamations or letters of support for carcinoid and NET cancer awareness, a record in 4-year history of NET Cancer Day!

42 US Governors Supported NET Cancer Awareness in 2013

7)      NET Conferences

2013 saw a record number of conferences for NET patients and medical professionals.  In California Carcinoid and Neuroendocrine Tumor Support Group Hosts Conferencethe United States, conferences kicked off in January when the Neuroendocrine Carcinoid Cancer Education Association (NCCEA), in conjunction with the Colorado Carcinoid Support Group, presented a mid-winter conference at the University of Colorado, Anschutz Medical Campus in Denver and NorCal CarciNETs Support Group and the UCSF Helen Diller Family Comprehensive Cancer Center presented an educational day of discussion on the latest developments in NETs.

Carcinoid Cancer Awareness Network logoThe Carcinoid Cancer Awareness Network (CCAN) presented regional conferences in New York City, New York (April); in Lexington, Kentucky (April); Rochester, Michigan (June); Orlando, Florida (September); and Los Angeles, California (December).

A highlight of the year was the first conference to focus on lung carcinoid, sponsored by CCAN in conjunction with Dr. Eric Liu and Lovable Lungnoids, an online Facebook group for lung carcinoid patients and their loved ones.  The conference, held at Vanderbilt-Ingram Cancer Center in Nashville, Tennessee, featured presentations by Dr. Dan Granberg, one of the world’s leading experts in bronchial carcinoid, and Dr. Eric Liu, Director of the Neuroendocrine Center at Vanderbilt University.

Eric H. Liu, MD, Director of the Neuroendocrine Center at Vanderbilt University

Eric H. Liu, MD, Director of the Neuroendocrine Center at Vanderbilt University

The Caring for Carcinoid Foundation sponsored patient education conferences in Los Angeles, California (April), Philadelphia (September) in conjunction with the Caring for Carcinoid Foundation logoUniversity of Pennsylvania Abramson Cancer Center; Stanford, California (October) in conjunction with Stanford Cancer Center; and Houston, Texas (November), co-sponsored by The Methodist Cancer Center, in conjunction with The University of Texas MD Anderson Cancer Center.

NANETS, the North American Neuroendocrine Tumor Society, the organization North American NeuroEndocrine Tumor Society (NANETS)of carcinoid/NET cancer medical/healthcare professionals, held its annual conference in October in South Carolina.  Additionally, NANETS presented regional conferences in Lexington, Kentucky, in conjunction with the University of Kentucky College of Medicine in April and in conjunction with Roswell Park Institute in Buffalo, New York in June.

The Second Annual International Patient Symposium on Pheochromocytoma and Paraganglioma was held in June on the National Institutes of Health (NIH) campus in Bethesda, Maryland.  Internationally recognized pheochromocytoma and paraganglioma specialist, Dr. Karel Pacak, talked about “Pheo  Para Research — Recent Achievements and Future Visions.”

The New Jersey Carcinoid Cancer NETwork presented conferences in June and November, in conjunction with Worldwide NET Cancer Awareness Day.

New Jersey Carcinoid Cancer NETwork

Multiple Endocrine Neoplasia (MEN) was the focus of conferences in June (at the Mayo Clinic in Rochester, Minnesota) and September (at Vanderbilt in Nashville, Tennessee), sponsored by American Multiple Endocrine Neoplasia Support.

American Multiple Endocrine Neoplasia Support logo

Internationally, the European Neuroendocrine Tumour Society (ENETS) held its annual conference in Barcelona, Spain in March; The UKI NETS  (UK and Ireland Neuroendocrine Tumour Society) 11th National Conference was in London in November; and the inaugural conference of the Asia Pacific NeuroEndocrine Tumour Society (APNETS) was held in Kuala Lumpur, Malaysia in October.

8)   Warner Advocacy Award

Jim Weiveris was selected as the winner of the 2013 Warner Advocacy Award. Jim Weiveris Winner of the 2013 Warner Advocacy AwardPresented by Novartis Oncology through Patient Advocacy and The NET AllianceTM, the award is given to the individual who best embodies the spirit, passion, and dedication of Monica Warner, a driving force behind the Carcinoid Cancer Foundation for two decades and an extraordinary advocate for carcinoid and neuroendocrine tumor survivors. Jim’s journey into advocacy for people with neuroendocrine cancer began in the late 1990s. His first wife, Susan, was experiencing vague symptoms, like debilitating stomach pain, which the doctors couldn’t diagnose. Scans finally revealed a gastrointestinal NET in 2000.  Jim and Susan helped cofound the NY Metro Carcinoid Support Group for patients and caregivers in the New Jersey/New York metropolitan area. After Susan passed away in 2003, Jim continued to stay active in the carcinoid/NET community and helped form a separate New Jersey Carcinoid Cancer Network, which he continues to oversee today.

9)       Books by Carcinoid/NET Patients  

Three books were published by patients in the carcinoid/NET community in 2013:  Becoming Sunny Susan by “Sunny Susan” Anderson; But You Look So Good . . . Stories by Carcinoid Cancer Survivors by Maria Gonzalez and I Have Cancer. And I’ve Never Felt Better! by Tracy Krulik.

“At the heart of the story is the idea of LIVING with cancer.  It took eight years to correctly Susan Anderson's Book, Becoming Sunny Susandiagnose my rare disease, carcinoid/neuroendocrine tumors. Then, they said I would die in just a few more years.  That was eighteen years ago, and here I am!  How have I fared?  Very well”, says Susan Anderson, who created the first patient website in the carcinoid/NET community in 1997. ”Read the book (Becoming Sunny Susan) to find out how I have lived a happy life, even with cancer.”

But You Look So Good . . . Stories by Carcinoid Cancer SurvivorsThe intimate stories of those living with a poorly understood and rare cancer are shared in Maria Gonzalez’s book, But You Look So Good . . . . Stories by Carcinoid Cancer Survivors. Over 20 patient and caregiver stories explore how carcinoid and neuroendocrine tumor (NET) patients came to be diagnosed, many enduring years of symptoms and lack of a proper diagnosis or no diagnosis at all; surgical experiences; the challenges of living with carcinoid syndrome; the value of in-person and online support groups; seeking information about these rare diseases; what it’s like for the families of patients; and hopes for the future.

Imagine nine years of debilitating gastrointestinal illness; misdiagnoses from doctors that include gastroparesis, pancreatitis, functional dyspepsia, Irritable Bowel Syndrome (IBS), and being told to reduce the stress in your life; a battery of tests such as endoscopy, colonoscopy, MRI, and an octreoscan; and a variety of medications – only to still feel horribly ill.  Tracy Krulik, author of  I Have Cancer. And I’ve Never Felt Better! overcame these challenges to lead a healthier, happier, and more balanced life.

I Have Cancer, Tracy Krulik book  10)      Education and Awareness  

Awareness and education are critical for the carcinoid/NET community — with greater knowledge of these rare diseases more patients will be correctly diagnosed and get earlier treatment, which can both improve and extend their lives.  Awareness and education lead to support, to research, to new treatment options and one day, to a cure – awareness and education lead to hope.  In addition to conferences, awareness and education are made possible by websites, online e-groups, and the continued availability and usage of the carcinoid-doctor e-group.  The Carcinoid Cancer Foundation saw the 1,500,000th visitor to its website in 2013.  People from over 150 countries on 6 continents regularly visit CCF’s website.  E-groups continue to grow with the carcinoid ACOR group, for example, serving more than 1,000 members.  New this year is a Pancreatic Neuroendocrine Tumor support group through Inspire, which connects patients, families, friends, caregivers and health professionals for health and wellness support.  A number of Facebook groups have also been created by members of the carcinoid/NET community.  Created in 2006 by the Association of Cancer Online Resources (ACOR)  the Carcinoid-Dr listserv (for healthcare professionals only) offers physicians and other healthcare professionals the opportunity to communicate with carcinoid/NET specialists. These specialists are available to discuss and share their experiences with diagnostic, treatment and surveillance options and procedures for rare neuroendocrine cancers. Many of these specialists have up to forty years’ experience in managing these orphan diseases.

Inspire Pancreatic Neuroendocrine Tumor Support Community

Also shared as significant for the carcinoid/NET community in the year 2013 are the following:

  • A $1 million challenge grant from the Goldhirsh-Yellin Foundation to the Caring for Carcinoid Foundation to expand NET cancer research
  • The retirement of carcinoid/NET specialist Dr. Larry Kvols of the Moffitt Cancer Center in Tampa, Florida
  • The affirming of Obamacare; no longer will patients be denied insurance for a pre-existing condition
  • The formal incorporation and inaugural conference of APNETs, the Asia Pacific Neuroendocrine Tumor Society
  • Sadly, the passing of several key support group and patient/patient advocate organization leaders.  In the United States, the community lost the leadership, vision and friendship of Dianna Boomershine, Heartland Carcinoids & NETs Support Group; Corie Dean, Pacific Northwest Carcinoid/NET Support Group, Washington Chapter; and Bea Lehming, Capital Area Carcinoid Survivors (CACS); in Canada, Maureen Coleman, founder of CNETS Canada and a member of the Worldwide NET Cancer Awareness Day Alliance; and in Sweden, Agneta H-Franzen of Carpa and the International Neuroendocrine Cancer Alliance.In Memoriam 2013, Carcinoid and Neuroendocrine Cancer Leaders
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