New Tools for Neuroendocrine Tumors

Monday, March 13, 2017

Rush first in state to offer novel diagnostic, treatment options

Rush University Medical Center soon will be the first hospital in Illinois to offer a sophisticated functional imaging test for neuroendocrine tumors. Because neuroendocrine neoplasms (cancerous growths) or their sites can be tiny, even as small as a centimeter in length, they are difficult to detect on with traditional imaging techniques such as CT, MRI.

That’s why Xavier M. Keutgen, MD, director of surgical services with the Rush Neuroendocrine Tumor Program, is excited about Rush obtaining the 68 Gallium DOTATATE PET/CT scanner. On March 20, Rush will begin offering this test, which was recently approved by the Food and Drug Administration.

Neuroendocrine tumors can be difficult to diagnose and treat. One reason is the inherent complexity of the neuroendocrine system, which is not an organ or a gland but a network of cells scattered all around the body. Neuroendocrine cells can release various hormones that help regulate various biological functions. 

Because these cells are found throughout the body, neuroendocrine tumors can grow in many different places, including the pancreas, adrenal glands, gastrointestinal tract, lungs and brain. Prognosis and treatment recommendations tend to vary depending on the tumor’s origin, stage and grade.

When caught at an early stage, neuroendocrine tumors can often be cured by surgically removing the tumor or tumors. “Unfortunately, in about half of the patients, these tumors have metastasized at the time of diagnosis, meaning they have spread out of their primary location where they originated,” Keutgen says. 

Surgically removing all neuroendocrine tumors requires knowing exactly where the cancer is hiding. That’s where the DOTATATE test comes in. 

Zeroing in on tumors 

For a DOTATATE test, patients are injected with a radioactive dye designed to bind to neuroendocrine tumors in the body, no matter how small, thus shining a spotlight on them. Studies have shown DOTATATE to be vastly superior in identifying neuroendocrine tumors compared to a similar but older test called the Ostreoscan. 

DOTATATE results can be critical to developing a successful treatment plan. “Let’s say a patient comes to me with a CT scan that shows a neuroendocrine tumor in the small intestine and something questionable on the liver or lungs,” Keutgen says. “In this case, we don’t know if the cancer has metastasized or not. 

“By getting a DOTOTATE scan, we will know whether that questionable liver or lung finding is a neuroendocrine tumor deposit. If it is, we can often remove all tumors when we operate and potentially cure the patient. If it isn’t, then we avoid an unnecessary surgery and focus on other non-surgical ways to help the patient.” 

‘Patients need a place to go’

While the disease is relatively rare, a large metropolitan area like Chicago has approximately 200 to 350 new cases a year, Keutgen estimates. “The neuroendocrine tumor world is evolving rapidly, and patients need a place to go that can provide the entire diagnostic and treatment spectrum,” he says. “They need a team of experts who know all the available options and can determine the best approach for a particular patient with a particular type of tumor.”   

Over the past 10 to 15 years, medical knowledge about how to fight neuroendocrine tumors has grown by leaps and bounds. Nonetheless, when Keutgen joined Rush’s medical staff last summer, he was surprised to learn that the Chicago area did not have a dedicated center devoted to serving patients with these tumors. 

Keutgen organized a group of Rush surgeons, interventional radiologists, medical oncologists, endocrinologists, gastroenterologists, pathologists and other subspecialists to launch Rush’s neuroendocrine tumor program, the only program of its kind in the Chicago area, in 2016.

Personalizing treatment 

When neuroendocrine tumors have spread to various places in the body, physicians may prescribe chemotherapy, or drugs to slow tumor growth. “Right now, oncologists assign one drug or another just randomly,” Keutgen said. “It’s like flipping a coin. If it doesn’t work after three or six months, then they’ll try another.” 

To improve on this approach, Rush physicians are partnering with the National Cancer Institute (one of the National Institutes of Health) using genetic tests of tumors, which provide details of mutations inside cancer cells. “You figure out what the mutation is, and you assign a drug that targets that mutation,” Keutgen says.

In late spring, Rush will be starting a clinical trial in which patients will have their neuroendocrine tumors tested for several genetic mutations. Then patients with those mutations will be prescribed a drug, either Sunitinib or Everolimus, depending on which mutation their cancer has.   

In addition, all cancer patients with aggressive or hard-to-treat cancers may benefit from a partnership that Rush University Cancer Center has with Tempus, a Chicago biotech company that sequences a person’s genome to look for more than 1,700 genetic mutations linked to different cancers. 

This may turn up a potential novel drug therapy to consider. “A patient with neuroendocrine cancer may have a mutation usually only found in breast cancer for example,” Keutgen said. “If there’s a drug that’s been approved to treat that mutation in breast cancer, we can try the drug which we would not use traditionally in this scenario on our neuroendocrine patient.”

Working with community physicians

Keutgen expects additional discoveries in neuroendocrine tumor treatment and diagnosis in coming years. In addition to being a surgeon, he is a researcher interested in discovering more about why neuroendocrine tumors develop and how to treat them based on their molecular profile. 

Because neuroendocrine tumor knowledge is continuing to advance, Keutgen believes it’s vital for subspecialists like himself and his colleagues at Rush’s Neuroendocrine Tumor Program to work closely with a patient’s oncologist or primary care physician. 

“We’re here to assist and support physicians in the community treating these patients,” he said. “A lot of patients with neuroendocrine tumors require several different types of treatments in their lifetime. We can give local physicians insights into what drugs and other treatments will work best and then work together to follow the patient regularly to make sure the treatment is working.”