Jennifer Chan, MD is the Clinical Director of the Gastrointestinal Cancer Center and the Director Carcinoid and Neuroendocrine Tumors Institute, as well as an Associate Professor of Medicine at Harvard Medical School.

National Youth Cancer Awareness Week

"[A younger diagnosis is] more common in [patients with] pancreas neuroendocrine tumors who are in their teenage years and on the verge of adulthood. I've taken care of several over the years, [and] its [prevelance] is certainly something to be aware of."

Jennifer Chan, MD, MPH, an associate professor of medicine at Harvard Medical School and clinical director of the Gastrointestinal Cancer Center at Dana-Farber Cancer Institute, discussed the occurrence of neuroendocrine tumors (NETs) in pediatric and younger adult populations in recognition of Adolescent and Young Adult Cancer Awareness Week.

Although NETs are most commonly diagnosed in middle-aged adult patients, these tumors can also occur in younger patients, including adolescents and, more rarely, children, Chan began. In pediatric cases, advanced or metastatic NETs are uncommon. When NETs are identified in this population, they are often appendiceal tumors discovered incidentally during appendectomy procedures. These tumors are typically localized and carry an excellent prognosis. In most cases, surgical resection is curative, and patients do not require further treatment.

In young adults and adolescents, pancreatic NETs may occasionally present. Chan noted that she has treated several patients who were teenagers or young adultswith pancreatic NETs, although such cases remain rare. Unlike appendiceal tumors, pancreatic NETs may require more complex management strategies depending on disease extent and clinical presentation.

Despite the rarity of NETs in younger individuals, it is essential for clinicians to remain aware of them as a diagnostic consideration in patients across all age groups, Chan continued. Although the majority of pediatric and adolescent cases are cured with local therapy, a subset may present with more advanced disease that necessitates ongoing monitoring or systemic treatment. Furthermore, although not all NETs in younger patients are hereditary, clinicians should remain vigilant in evaluating potential underlying genetic syndromes in cases with suggestive clinical or family histories, Chan said.

Videos

Jennifer Chan, MD, MPH, discusses the prevalence of neuroendocrine tumors during Adolescent and Young Adult Cancer Awareness Week.

Dr Chan on the Prevalence of Neuroendocrine Tumors in Young-Adult Patients

Dr Chan on the FDA Approval of Cabozantinib for Pancreatic and Extra-Pancreatic Neuroendocrine Tumors

"This approval really means that we have a new standard option to offer to patients with neuroendocrine tumors who've had at least 1 other line of therapy and whose disease is progressing and needs additional therapy to control growth."

Jennifer Chan, MD, MPH, an associate professor of medicine at Harvard Medical School and clinical director of the Gastrointestinal Cancer Center at Dana-Farber Cancer Institute, discussed the clinical relevance of the 

 for the treatment of adult patients with previously treated, unresectable, locally advanced or metastatic, well-differentiated pancreatic neuroendocrine tumors (pNETs) and extra-pancreatic neuroendocrine tumors (pNETS).

The regulatory decision on March 26, 2025, was supported by data from the phase 3 CABINET trial (NCT03375320), which showed that among patients in the pNET cohort (n = 99), those treated with cabozantinib experienced a median progression-free survival (PFS_ of 13.8 months (95% CI, 8.9-17.0) vs 3.3 months (95% CI, 2.8-5.7) for those given placebo (HR, 0.22; 95% CI, 0.12,-0.41; 

 < .0001). In the epNET cohort, the median PFS was 8.5 months (95% CI, 6.8- 12.5) in the cabozantinib arm vs 4.2 months (95% CI, 3.0-5.7) in the placebo arm (HR, 0.40; 95% CI, 0.26- 0.61; 

Chan noted that the approval of cabozantinib represents a key advancement in the treatment landscape for patients with pNETs and epNETs. Chan highlighted the importance of this milestone, particularly given the limited treatment options available for this patient population. The broad label indication is notable, encompassing patients with both functional and nonfunctional tumors, as well as those with somatostatin receptor–positive and –negative disease.

Chan concluded by noting that cabozantinib offers a new, broadly applicable therapeutic option for patients with progressive NETs, addressing a longstanding unmet need in this field. As treatment options for NETs continue to expand, the integration of cabozantinib into clinical practice will provide oncologists with an effective tool for disease management in a heterogeneous and challenging patient population, she said.

Jennifer Chan, MD, MPH, discusses the FDA approval of cabozantinib for previously treated, advanced well-differentiated (extra) pancreatic neuroendocrine tumors.

Jennifer Chan, MD, MPH, senior physician, clinical director, Program in Carcinoid and Neuroendocrine Tumors, Dana-Farber Cancer Institute, assistant professor of medicine, Harvard Medical School, discusses a trial of cabozantinib (Cabometyx) in neuroendocrine tumors (NETs).

A phase II trial in patients with carcinoid and pancreatic NETs showed that treatment with cabozantinib was associated with objective tumor responses, as well as encouraging progression-free survival.

Chan says there is ongoing work to evaluate cabozantinib in a larger trial.

Jennifer Chan, MD, MPH

Articles

Dr Chan on the Prevalence of Neuroendocrine Tumors in Young-Adult Patients

Dr Chan on the FDA Approval of Cabozantinib for Pancreatic and Extra-Pancreatic Neuroendocrine Tumors

Dr. Chan on Trial of Cabozantinib in Neuroendocrine Tumors