Unmasking Rare Cancers on Rare Disease Day

Neuroendocrine tumors are tricky to diagnose but treatable

/ Author:  / Reviewed by: Joseph V. Madia, MD

(RxWiki News) In the United States, more than 600 patient organizations, government agencies, educational institutions, clinical centers, and companies have partnered to participate in the 5th Annual Rare Disease Day February 29, 2012. And on this day, dailyRx is taking a look at rare cancers known as neuroendocrine tumors, or NETs.

NETs were spotlighted last year with the death of Steve Jobs. He had a pancreatic neuroendocrine tumor, one of various cancers that can develop in the endocrine organs and nervous system, which work to keep the body functioning normally.

"If you have difficult to explain symptoms that don't go away, visit your doctor."

These are rare cancers affecting between 12,000-15,000 people in the United States each year. Most often, NETs appear in the gastrointestinal system, but can also occur in the lungs and in the major neuroendocrine glands, such as the pancreas, thyroid and parathyroid, and adrenal glands.

They are tricky to diagnose because often NETs cause no symptoms, or the symptoms that do appear could be caused by a number of other conditions.

dailyRx spoke with NET specialist, Steven Libutti, M.D., vice chairman of surgery and director of the Montefiore Einstein Center for Cancer Care, and asked him to describe some of the symptoms. 

"A NET in the GI tract can present as a small bowel obstruction, which most people won't ignore," Dr. Libutti said. But other tumors are more difficult to distinguish. 

"If the cancer has spread to the liver, for example, a person might have flushing [turning red] in the face. If it shows up in the adrenal gland, high blood pressure is a common symptom," Dr. Libutti told dailyRx.

"These are not distinct symptoms that patients can usually distinguish from other conditions," he said.

So how are NETs diagnosed? "Most are found incidentally. A physician may work up difficult to explain symptoms. Or, imaging studies may pick up the tumors," explained Dr. Libutti.

The good news is that if these tumors are detected early, according to Dr. Libutti, "Surgery can be effective to treat and cure these tumors"

For more advanced disease, surgery that's combined with either regional or systemic therapy can also be helpful.

"We have a lot more weapons against NETs than we had even a few years ago," Dr. Libutti said.

Dr. Libutti will be hosting a Twitter chat at 2:00 p.m., EST on February 29, 2012 to discuss the signs and symptoms, diagnosis and treatment of neuroendocrine tumors.

To participate, use hashtag #Monte_NETs to be part of the conversation, which will be moderated by the Carcinoid Cancer Awareness Network, the Carcinoid Cancer Foundation, and VHL Family Alliance.

Reviewed by: 
Review Date: 
February 28, 2012
Last Updated:
March 1, 2013