Hope for Neuroendocrine Tumor and Carcinoid Syndrome Patients
For most people, the word "cancer" brings up a variety of intense feelings and emotions associated with the disease. For Maryanne Wahmann, that too-real experience began in her early thirties. For about seven years, neither she nor her doctors knew what was causing the wracking gastrointestinal (GI) symptoms, including explosive diarrhea and abdominal pain, that some days prevented her from leaving the bathroom. During those years she was a frequent visitor to emergency rooms and physicians’ offices.
As Maryanne explained during a recent interview, the nature of her symptoms lead her doctors to diagnose her with Crohn’s disease, ulcerative colitis, and irritable bowel syndrome. Several clinicians told her she was crazy, and one even recommended she see a marriage counselor.
Neuroendocrine tumor and carcinoid cancer
Little did they know Maryanne actually had a neuroendocrine tumor (NET) that had advanced into carcinoid syndrome, a relatively rare type of cancer. It wasn’t until one doctor used a pediatric scope, which is longer and more flexible than those typically used in adults for colonoscopies, that the reason for her symptoms was identified: a midgut tumor, located between the small and large intestines. “We’re 99.9% sure that tumor was there the whole time,” noted Maryanne.
Yet no one had found it. This is not uncommon, according to clinical oncologist Hal Gerstein, MD, since unlike typical GI malignancies, where the primary tumor can be “quite large” and the metastatic cancer (the one that spreads) is small, the situation with carcinoid cancer is the opposite, which makes it difficult to find the tumor.
Gerstein noted there are about 5,000 new cases of NETs and carcinoid cancer diagnosed per year, while the number of people being treated for the disease can range from about 100,000 to 200,000. For these individuals, like Maryanne, the road to identifying the disease can be a long and arduous one.
Once Maryanne received an accurate diagnosis and treatment (thanks to use of the pediatric scope), she was inspired to start a nonprofit organization dedicated to helping others who are walking the road of frustration, questions, and concerns she encountered during her journey, as well as educating the public and medical community about this elusive disease.
The disease is known as carcinoid cancer and related neuroendocrine tumors (NETs). Carcinoid tumors and other neuroendocrine tumors usually arise from cells that make hormones. The problem is they produce an excessive amount of hormone-like substances such as histamine, serotonin, prostaglandins, tachykinin, and bradykinin. When levels of these substances are abnormally high, they can result in various symptoms referred to as carcinoid syndrome. Similarly, other neuroendocrine tumors can produce other hormone-like factors that cause various other syndromes. Gerstein pointed out that about 20 percent of people with NETs will go on to develop carcinoid syndrome.
Tumors are typically located in the gastrointestinal system, especially in the midgut as Maryanne experienced, but they also can be found in the lungs, testes, pancreas, and ovaries. According to the Carcinoid Cancer Foundation, “these rare types of ‘midway’ growths” are called “cancers in slow motion” because they “mostly tend to grow so slowly” that individuals with the tumors usually don’t have symptoms for years.
Along with GI symptoms (e.g., diarrhea, abdominal pain, heartburn, bloating), others can include facial flushing, rash, weakness, and wheezing. Because these symptoms can be associated with a wide range of other diseases and conditions, reaching a diagnosis is a challenge. Dr. Gerstein noted that in some cases, unchecked carcinoid cancer can cause fibrosis on the heart valves.
Hope for NET and carcinoid cancer patients
Maryanne and Dr. Gerstein are hopeful for patients with NETs and carcinoid syndrome. One reason is the introduction of new treatments with “more treating coming in the future.” Currently the primary treatment options include somatostatin analogues, (SSAs), which prevent the body from producing too many hormones and can reduce symptoms of carcinoid syndrome and slow tumor growth.
The Food and Drug Administration also recently (February 28, 2017) approved Xermelo (telotristat ethyl), which inhibits the tryptophan hydroxylase enzyme to reduce the overproduction of serotonin within NET cells, which causes diarrhea. When used along with SSA therapy, it has significantly reduced carcinoid syndrome’s debilitating diarrhea. Additional treatments for carcinoid syndrome include other targeted therapies, embolization of liver metastases, and surgery.
Patients, their families, and caregivers also have access to the Neuroendocrine Cancer Awareness Network, which Maryanne launched in 2003. Along with offering information packets to patients and their families on NET and carcinoid syndrome and doctor referrals, NCAN has also provided 60 educational programs around the country. “We are trying to bring awareness to patients and caregivers and educate more doctors,” explains Maryanne. Anyone can call the hotline 9AM to 9PM, seven days a week, at 866-850-9555 and speak with a patient or caregiver for more details, physician referrals, and support group information.