AYA Cancer Awareness Week: Patient Support, Multidisciplinary Care Are Key for Management of NETs

Jennifer Chan, MD, MPH

In recognition of Adolescent and Young Adult (AYA) Cancer Awareness Week, Jennifer Chan, MD, MPH, emphasized the importance of highlighting the incidence of neuroendocrine tumors (NETs) in this patient population. Although NETs are more frequently diagnosed in middle-aged adults, Chan noted that they can also present in younger individuals, including adolescents and those in early adulthood, and increasing clinical vigilance in this group may help facilitate earlier diagnosis and improved outcomes.

“It's so important to recognize that we need to care for these patients. They have special needs and may also benefit from going to places where there are [treatment and support] programs in place,” Chan said. “Patients definitely benefit from not feeling alone and knowing that they're supported, [surrounded] by other people going through the same issues and journey as they are.”

In an interview with OncLive®, Chan addressed the rarity of advanced NETs in pediatric and young adult populations, noting that early-stage presentations—such as appendiceal NETs discovered incidentally—can be curable. She also highlighted that pancreatic NETs may have a higher prevalence in adolescents or young adults, and she emphasized the need to consider NETs in the differential diagnosis, particularly when symptoms such as diarrhea or flushing persist without a clear explanation.

Additionally, Chan outlined key considerations for diagnosis, stressing the importance of maintaining a high index of suspicion and employing tools such as imaging, endoscopy, and biopsy when warranted.

Chan is an institute physician, clinical director of the Gastrointestinal Cancer Center, and director of the Program in Carcinoid and Neuroendocrine Tumors at Dana-Farber Cancer Institute, as well as an associate professor of medicine at Harvard Medical School, both in Boston, Massachusetts.

OncLive: How common are NETs within pediatric and young adult populations, and why is it important to raise awareness about them?

Chan: We do encounter patients who are younger, some of [whom are] pediatric patients [that] may have a NET, and we also have younger adults with NETs. In general, within we see most patients [with NETs] are more middle-aged. [However], we also see a whole range of ages, including younger patients.

[The prevalence] of advanced or metastatic NETs in the pediatric and [young adult] populations is pretty rare. If you were to think about [the diagnosis] of NETs in younger patients, oftentimes it may be [found] incidentally [during] an appendectomy, and those patients we expect are going to be cured.

It's uncommon to have metastatic or advanced NETs in pediatric patients, but we do see them [on occasion]. There are patients—and I'd say that this is more common in pancreas NETs—who are in their teenage years and on the verge of adulthood [who have advanced NETs], and I've taken care of several over the years, [and] it is certainly something to be aware of.

Fortunately, when we do encounter NETs in younger and pediatric patients, they can be cured and often are cured.

From a diagnostic standpoint, what key information should primary care physicians be aware of when evaluating patients who may have a NET?

The most important thing to keep in mind about NETs is to think about them as a possibility for symptoms that people might be experiencing.

One thing that has been a challenge in the past is that because NETs have symptoms that overlap with some other common gastrointestinal conditions, someone's years of, for instance, diarrhea or flushing may be related to more common gastrointestinal disorders like irritable bowel syndrome. That is important to keep it in mind.

Once you start to think about the diagnosis, there are things that you can do to try to establish the diagnosis. Some of it may involve endoscopy, and some of it may involve imaging and then, ultimately, biopsies. However, the most important thing to keep to do is to think about [NETs as a possible diagnosis].

When it comes to referrals, oftentimes, this is a disease where there are multiple disciplines that are involved. As oncologists, we often are some of the first specialists to know about a diagnosis because—especially in a less-common cancer diagnosis—the call often comes to oncologists. However, we do involve a lot of other disciplines, [including] surgeons, advanced endoscopists, and endocrinologists. When it comes to the referrals, patients often will be seeing a number of different specialists to try to come up with an [individualized] treatment plan.

How does a younger patient’s age influence treatment decisions or the selection of treatment modalities in this population?

Right now, this is a really important question, and especially as we start to see [younger] patients across the board in terms of cancer diagnoses, it's important to keep in mind that there may be differences in how the cancer behaves in younger patients vs older patients, and there are also different needs for patients.

Some younger patients are at a very different stage of their life [compared with] older patients in terms of their responsibilities at work and to family, and they also have very different financial concerns.

There are medical needs that patients who are younger have that may not be as much of a concern for older patients. Some of these [needs] may, for instance, include questions and needs regarding whether there is going to be an impact of any of the therapies on fertility. There also is a really important need to support younger patients from a psychosocial standpoint by helping them address some of the challenges of living as a young person with cancer and navigating other medical, financial, and logistical concerns that many of their friends and colleagues don't have.

There has been an increasing need across the board to support our young patients, and this is cutting across many different cancer diagnoses. At Dana-Farber Cancer Institute, we have programs set up to help support our young adult patients, and some of them even lie within certain disease centers as we start to see more [younger] patients with, for example, gastrointestinal and digestive cancers.