Pancreatic Neuroendocrine Tumors: A Rare Cancer

From your next door neighbor to Steve Jobs, co-founder of Apple who just announced that he is stepping down as CEO of the company, pancreatic neuroendocrine tumors (pNETs) affect less than 1 person in 100,000 in the United States.  This rare cancer is often confused with the more aggressive pancreatic cancer, the cancer which took the lives of celebrities Patrick Swayze, Luciano Pavarotti, Michael Landon, and Jack Benny.  Pancreatic neuroendocrine tumors grow more slowly than typical pancreatic cancer.

NET cancer survivor Mitchell Berger and his wife, Peg Girshman

NET cancer survivor Mitchell Berger with his wife, Peg Girshman

NET survivor Mitchell Berger has written an excellent NPR editorial explaining the difference between pancreatic neuroendocrine tumors and pancreatic cancer and calling for greater education and understanding about neuroendocrine tumors. Click here to read the article.

Neuroendocrine cancers affect cells throughout the body that secrete hormones.  They are found most commonly in the lung or gastrointestinal system, but they can also originate in other parts of the body such as the pancreas, ovary, and testes, among other sites. Terms associated with neuroendocrine cancers include carcinoid, GEP-NETs (gastroenteropancreatic NET),  and pancreatic NET (islet cell tumor, gastrinoma, glucagonoma, and insulinoma).

(Steve Jobs talks about his cancer diagnosis at the 10:13 mark)

Some Facts about NET Cancers:

  • Over 75% of all carcinoid/NET patients are initially incorrectly diagnosed and treated for the wrong disease. 
  • From the initial onset of symptoms the average time to proper diagnosis can exceed five years.
  • Irritable Bowel Syndrome (IBS) and Crohn’s disease are the two most common misdiagnosed conditions with midgut carcinoid.
  • NET cancers typically spread to the liver and lymph nodes.
  • Abdominal pain, flushing, diarrhea, wheezing, bloating, heart palpitations, weakness, skin rash, heartburn, and weight changes are the most common carcinoid/NET symptoms.
Richard R.P. Warner, MD

Dr. Richard Warner, Medical Director of the Carcinoid Cancer Foundation

According to Richard R.P. Warner, MD, Professor of Medicine at Mount Sinai School of Medicine in New York and Medical Director of the Carcinoid Cancer Foundation,“the prognosis for NET cancer patients  is better the earlier treatment is started. We now know that surgery and other treatments work much better when done early. Early, aggressive treatment of neuroendocrine tumors leads to a much better outcome for patients.”

This past spring, the U.S. Food and Drug Administration (FDA) approved the first new drugs in nearly three decades for pancreatic neuroendocrine tumor patients with tumors that cannot be removed by surgery or that have spread (metastasized) to other parts of the body:  Novartis’ Afinitor (everolimus) and Pfizer’s Sutent (sunitinib).

Another treatment used by pancreatic neuroendocrine tumor patients is PRRT (or PRRNT), peptide receptor radionuclide therapyA clinical trial of Lutetieum-177 (LU-177) with Octreotate began in September 2010.   Ebrahim S. Delpassand, MD, the principle investigator of this clinical trial and medical director of Excel Diagnostics and Nuclear Oncology Center in Houston, said that he and his colleagues “worked extremely hard over many years to bring this treatment to our patients in the United States.  High activity In-111 Octreotide PRRT has also been available at Excel since August of 2005 . We do everything possible to help the patients financially and with accommodations.  We have a dedicated facility for the therapy and patients are treated in a very comfortable, outpatient setting.” Excel Diagnostics and Nuclear Oncology Center in Houston, Texas is the first research facility in the United States to receive authorization to initiate this much needed therapy.

Ebrahim S. Delpassand, MD

Dr. Ebrahim Delpassand

PRRT is also available in Europe, in Germany, Switzerland and the Netherlands, and in Australia and India. During the past 15 years, studies of radio-peptide therapy for various neuroendocrine cancers have shown good clinical and radiological results with minimal side effects.

The 1st World Congress on Gallium-68 and PRRNT, co-organized by the Department of Nuclear Medicine/PET Center, Zentralklinik Bad Berka and the Institute of Nuclear Chemistry, Johannes Gutenberg University, Mainz was held from June 23 – 26, 2011 in Bad Berka, Germany.

Liver transplantation in selected patients with pNETs can increase a patient’s overall survival by 5 years in 45% to 66% of patients.

To learn more about pancreatic neuroendocrine tumors,  visit the Carcinoid Cancer Foundation’s website where you can download a PowerPoint presentation by Michael J. Demeure, MD, MBA, Senior Investigator at TGen (The Translational Genomics Research Institute) and Director of the Rare Tumors Center at Scottsdale Healthcare in Arizona.

Michael J. Demeure, MD

Dr. Michael Demeure

To read more about Steve Jobs here are three recent articles:

  • The Wall Street Journal, August 25, 2011

Jobs Struggled with Health Problems for Years by Nick Wingfield

  • The New York Times, August 24, 2011

Jobs Steps Down at Apple, Saying He Can’t Meet Duties by David Streitfeld

  • CNN Health, August 25, 2011

Piercing Together Details of Jobs’ Health History by Elizabeth Landau (with quotes from NET specialist Dr. James Yao)

For additional previous press coverage, see a list of articles in our blog:

Carcinoid Cancer Foundation Thanks Steve Jobs for Bringing About Greater Awareness of Neuroendocrine Cancers

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