Carcinoid tumors are a type of
neuroendocrine tumor that can develop in the appendix, gastrointestinal tract,
or lungs. These tumors are occasionally found growing in the appendix after it
is removed during an episode of appendicitis.
The majority of carcinoid tumors in
children are small, slow-growing, and benign. In rare cases, these tumors can
grow more quickly and spread to other sites in the body.
Children with carcinoid tumors are treated at Dana-Farber/Boston Children's
through our Endocrine-Oncology Program in
conjunction with our Solid
Tumor Center. 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.
As a parent,
you undoubtedly want to know what may have caused your child’s tumor. Some carcinoid tumors are linked to inherited
conditions, such as MEN1. However, most carcinoid tumors emerge with no known
cause.
The symptoms of a carcinoid tumor may vary from child to
child and depend on where the tumor is located and what kind of tumor it is.
Symptoms might mimic other, more common ailments. Some of the most
common symptoms include:
- Abdominal
pain due to appendicitis. Most cases of appendicitis, however, are not
related to carcinoid tumors.
- In rare
cases, children may develop carcinoid syndrome, with symptoms such as
redness in the face and neck, rapid heartbeat, difficulty breathing,
sudden drop in blood pressure, and diarrhea. These symptoms are caused by
hormones produced by the tumor and rarely occur except in advanced cases.
Because many
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:
- A
physical exam and complete medical history.
- Blood
and urine tests.
- A
biopsy, a tissue sample taken from the tumor. The tumor's appearance under a
microscope helps doctors to make a diagnosis so the appropriate treatments can
be recommended.
- Magnetic
resonance imaging (MRI), a diagnostic
procedure that produces detailed images of the area where the tumor is located.
An MRI uses a combination of large magnets, radiofrequencies, and a computer to
analyze organs and structures within the body.
- A
computerized tomography scan (CT/CAT scan), an imaging technique that provides
more detailed pictures than X-rays.
- Molecular
testing to determine whether the tumor is linked to specific genes.
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.
Treatment for
your child's carcinoid tumor will depend on its location and whether it has
spread. Your child's doctor may recommend:
- Surgery, involving biopsy and removal of the entire
tumor and nearby tissue.
- Radiation,
the use of high-energy rays from a specialized machine to damage or kill cancer
cells and shrink tumors. This is often used together with surgery, either
before or after removal of the tumor.
- Chemotherapy, a drug treatment
that aims to destroy or shrink cancer cells, may be given before or after
surgery.
- Different groups
of chemotherapy drugs work in different ways. Your child may receive
chemotherapy orally, as a pill to swallow; intramuscularly, as an injection
into the muscle or fat tissue; intravenously, as a direct injection into the
bloodstream or IV; or intrathecally, as a direct injection into the spinal
column through a needle. Often, a combination of chemotherapy drugs is used.
- While
chemotherapy can be quite effective in treating certain cancers, the drugs
cannot differentiate normal healthy cells from cancer cells. As a result, there
can be adverse side effects during treatment. Being able to anticipate these
side effects can help the care team, child, and family prepare and, in some
cases, prevent these complications from occurring, if at all possible.
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.
Clinical Trials
Clinical
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.
It’s possible
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, she may be eligible for a number of
experimental therapies available through these groups or from one of our
independent clinical investigators.
Your child’s outlook will likely depend on where the
tumor is and whether it has spread.
For tumors growing in the appendix, often an
appendectomy is the only thing needed to completely remove the tumor. In this
situation, a child’s prognosis is excellent. Larger tumors in other locations
or those that have spread throughout the body are more difficult to treat and
may require surgery, chemotherapy, radiotherapy, or other therapies or
interventions.
Children
treated for carcinoid 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.