Neuroendocrine tumors, also known as NETs,
are growths that develop from specialized, hormone-producing neuroendocrine
tissue distributed throughout the body.
Neuroendocrine tumors can form in the
gastrointestinal tract, lungs, pancreas, and other tissues. NETs are sometimes
called carcinoid tumors, especially when they grow out of the gastrointestinal
tract or lungs. Pancreatic NETs are sometimes called “islet cell” tumors and
include insulinomas, glucagonomas, gastrinomas, VIPomas, and non-functioning
Some NETs secrete hormones while others do
not. Those that secrete hormones are referred to as functioning and may cause
specific symptoms related to excessive production of that hormone. Most NETs
are slow-growing, but some are more aggressive, growing rapidly and spreading
to other parts of the body.
Children with neuroendocrine tumors are treated at Dana-Farber/Boston
Children's through our Endocrine-Oncology Program.
Our integrated pediatric oncology service offers—in one specialized program—the
combined expertise of a leading cancer center and a premier children’s
hospital. We build a team to treat your child consisting of oncologists,
endocrinologists, genetic counselors, and surgeons. Advanced cancers may also be
treated through our Solid
Continue reading for more information about
neuroendocrine tumors or visit the Endocrine-Oncology
Program page to learn more about our expertise or meet our treatment
is complete, your child’s team will likely determine the tumor’s stage or
classification, a way of describing where the tumor is located and whether it
NETs are sometimes
categorized by their location – for instance, in which part of the
gastrointestinal system they are located. They are also sometimes given more
specific names based on what type of hormone they produce.
As a parent,
you undoubtedly want to know what may have caused your child’s tumor. It is important to understand that often tumors
emerge with no known cause.
Some neuroendocrine tumors result from hereditary
factors. Examples of hereditary or genetic conditions that carry an increased
risk for developing NETs include multiple endocrine neoplasia (MEN), familial
medullary thyroid cancer (FMTC) syndrome, von Hippel-Lindau syndrome,
hereditary paraganglioma-pheochromocytoma syndromes, and Carney triad.
The symptoms of a neuroendocrine tumor may vary from child
to child and can depend on where the tumor is located, what kind of
neuroendocrine tumor it is, and what type of hormone it secretes. Symptoms might
mimic other, more common ailments. Some of the most common symptoms may include:
of these symptoms can also point to other conditions, it’s important to have
your child evaluated by a qualified medical professional right away.
The first step in treating your child is forming an
accurate and complete diagnosis. Your child’s physician may order a number of
different tests including:
There may be other
diagnostic tests that your doctor will discuss with you depending on your child's
individual situation. After we complete all necessary tests, our experts meet
to review and discuss what they have learned about your child's condition. Then
we will meet with you and your family to discuss the results and outline the
best possible treatment options.
your child's neuroendocrine tumor will depend on its type and location. Your
child's doctor may recommend:
Children who are treated through our Endocrine-Oncology Program benefit from
the work of our basic and clinical researchers, who are striving to understand
the scientific causes of endocrine cancers. Their work can result in the
introduction of new treatment options. We are a world leader in translational
research, bringing laboratory advances to the bedside and into doctors’ offices
as quickly as possible.
trials, or research studies evaluating new treatment approaches, are a major
offering at Dana-Farber/Boston Children’s. For many children with rare or
hard-to-treat conditions, clinical trials provide new options.
that your child will be eligible to participate in one of our clinical trials.
In addition to launching our own clinical trials, we also offer trials
available through collaborative groups such as the Children's Oncology Group (COG).
If your child has a progressive or recurrent
tumor, he or she may be eligible for a number of experimental therapies
available through these groups or from one of our independent clinical
Your child’s outlook will likely depend on where his
tumor is and whether it has spread.
Children with a neuroendocrine tumor of the appendix
typically have a good prognosis after surgery. By the time they are diagnosed,
neuroendocrine tumors outside the appendix have often spread or are larger.
Bigger neuroendocrine tumors tend to come back more often, which we would
consider in designing the treatment and follow-up plans.
treated for neuroendocrine tumors should visit a survivorship clinic yearly.
Through the David
B. Perini, Jr. Quality of Life Clinic, our cancer survivorship
clinic, childhood cancer survivors receive a comprehensive follow-up evaluation
from their cancer care team. In addition to meeting with your pediatric
oncologists, your child may see one of our endocrinologists, cardiologists, neurologists,
neuro-psychologists, or alternative/complementary therapy specialists. We also offer patient and family
education, psychosocial assessment, genetic counseling, reproductive counseling, and opportunities to speak with other
childhood cancer survivors.
Lindsay Frazier, MD, explains how a multidisciplinary team of specialists come together to deliver care for solid tumors.