They are a malignant gastrointestinal tumor, but are slow-growing and can be treated with surgery and chemotherapy.
THE SPECIALIST: Dr. Celia Divino
The chief of the division of general surgery at Mount Sinai, Dr. Celia Divino specializes in performing surgeries on the gastrointestinal tract. She has been working in the field for 25 years.
WHO’S AT RISK
There are 11,000-15,000 new cases of carcinoid diagnosed every year in the U.S., and an estimated 115,000 Americans are currently living with the disease. “Carcinoid is a malignant gastrointestinal (GI) tumor that secretes hormones that can affect every organ from the eyes to the liver to the heart,” says Divino. “In the past, carcinoid tumors were assumed to be benign, but they’re not — they’re a slow-growing cancer that is a type of neuroendocrine tumors.” The pancreatic cancer that killed Steve Jobs began as a type of neuroendocrine tumor (NET).
Carcinoid tumors predominantly affect the GI system. “What distinguishes carcinoid tumors is that they secrete a lot of substances that cause a diverse range of effects, including diarrhea, abdominal pain, heartburn and rashes,” says Divino. “Because the symptoms are so diverse and nonspecific, it’s often misdiagnosed. Unfortunately, because the symptoms are so misleading, many patients don’t get diagnosed until too late.”
The underlying cause of carcinoid tumors is a transformation of neuroendocrine cells. “Usually, the transformed cells develop in the stomach, small intestine, appendix or colon, and then spread elsewhere through the blood, like any cancer,” says Divino. “When we can diagnose carcinoid early, we can really help people.”
Advocacy groups have adopted the zebra as a logo of sorts for carcinoid. “We compare carcinoid to a zebra because it’s so heterogeneous or mixed, like a zebra with its stripes,” says Divino. “A zebra looks like a horse, but it’s not quite a horse — you’re not quite sure what it is. This happens all the time in carcinoid patients because their symptoms can resemble anxiety attacks or irritable bowel syndrome or appendicitis.”
As yet there are no identified risk factors associated with carcinoid. “To date, there is no genetic defect, and no known environmental exposures,” says Divino. “Carcinoid affects men and women at any point in life. With more testing and awareness, we can do a much better job of treating these patients.”
SIGNS AND SYMPTOMS
Although carcinoid can cause many diverse symptoms, there are a few red flags. “The classic triad of symptoms is flushing (redness), diarrhea and palpitations — nonspecific symptoms, but when they cluster together, it’s suspicious,” says Divino. “Stress can be a trigger for many patients, which is one of the reasons carcinoid is often misdiagnosed as an anxiety attack or PTSD.” Another common symptom is wheezing.
The diagnosis of carcinoid is made through a urine test and blood tests. “When these tests are positive, that calls for more intensive tests: CT scans, other radioactive scans and endoscopy,” says Divino. “This is a disease where early diagnosis makes all the difference.”
Once patients are correctly diagnosed with carcinoid, doctors have a battery of treatments to keep it in check. “The good thing about this disease is that there’s so much we can do, medically and surgically,” says Divino. “These tumors aren’t benign, but they are slow-growing, so they’re compatible with a decent survival and good quality of life.”
Treatment involves a multispecialty approach including gastroenterologists, surgeons, oncologists, cardiologists and radiologists.
The pancreatic cancer that killed Steve Jobs began as a type of neuroendocrine tumor.One of the goals of early detection is to identify tumors in time to remove them. “The mainstay of treatment is to remove the primary tumor — anywhere we can remove the tumors, we take them out,” says Divino. “The trick is to tell which tissue has the tumor — it can be hard to tell the difference between the actual tumor and fibrous tissue that surrounds it.” The prognosis and recovery from surgery depend on how far along the carcinoid is, and how much is being removed.
Medications are the primary tool for symptom management. “There’s a shot we can give patients once a month to control symptoms like diarrhea, flushing and palpitations — on their own, these symptoms can become so debilitating, that patients really can’t survive without treatment,” says Divino. “For tumors that we can’t remove surgically, there are chemotherapeutic treatments that can be given to specifically target the tumor.”
New technologies are revolutionizing the way doctors treat carcinoid. “Our ability to use blood tests to check for tumor markers has advanced tremendously, and there’s a new scan called the gallium scan that is very specific and sensitive,” says Divino. “New agents are being developed, so there’s a lot we can do to circumvent bad outcomes.”
QUESTIONS FOR YOUR DOCTOR
If you have unexplained symptoms that fit the triad of flushing, diarrhea and palpitations, be proactive about asking, “Should I be tested for carcinoid?” And don’t hesitate to ask, “Can you refer me to a carcinoid center?” “You need to go to a place that takes care of many carcinoid patients and has a multidisciplinary team familiar with the disease,” says Divino.
“The fact is that when correctly treated, carcinoid patients have a decent prognosis and quality of life — and we’re developing more and more improved treatments, so the prognosis is only getting better.”
WHAT YOU CAN DO
Get informed. Because carcinoid is a disease that remains underdiagnosed, it’s all the more important for patients to seek out the latest information. Two reliable websites are hosted by the Carcinoid Cancer Foundation (carcinoid.org) and Mount Sinai’s Center for Carcinoid and Neuroendocrine Tumors (mountsinai.org/patient-care/service-areas/digestive-disease/areas-of-care/neuroendocrine-tumors).
Get support. There’s a robust community of carcinoid patients who share their experiences through chat rooms and support groups.
Don’t ignore symptoms. “Carcinoid has a characteristic clustering of symptoms: flushing, diarrhea and palpitations,” says Divino. “Since carcinoid still isn’t on the radar screen for many doctors, it’s essential that patients be their own advocates.”
Address nutrition. Because carcinoid tumors burn through amino acids, patients can develop protein deficiencies — which require specialized diets.
New York Daily News
Sunday, April 27, 2014