The pancreas is an organ responsible for helping
the body digest food and regulate sugar. Tumors of the pancreas can develop
from the organ’s exocrine cells, which make enzymes to aid in digestion, or
endocrine cells, which produce hormones such as insulin and glucagon that
control blood sugar levels.
Several different types of pancreatic
tumors can develop in children, such as solid pseudopapillary tumors (SPTs), pancreatoblastomas,
and neuroendocrine tumors (NETs). NETs in the pancreas, also known as islet
cell tumors, include insulinomas, glucagonomas, gastrinomas, VIPomas,
somatostatinomas, and nonfunctioning tumors. Although pancreatic tumors are
rare in children, some kinds, such as NETs, occur more commonly in children
with certain inherited or genetic conditions. SPTs are most often seen in girls
and young women.
Children with tumors of the pancreas 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.
Once diagnosis
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
has spread.
The type of
pancreatic tumor your child has will help determine his prognosis and
treatment. Types of tumors of the pancreas include:
- Insulinomas
- Glucagonomas
- Gastrinomas
- VIPomas
- Somatostatinomas
- Nonfunctioning tumors
- Solid pseudopapillary tumors
- Pancreatoblastomas
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. Many result from a mingling of genetic and
environmental factors. In some cases, inherited conditions are linked to the
development of pancreatic tumors, as is the case with von Hippel Lindau
syndrome and multiple endocrine neoplasia syndromes.
The symptoms of pancreatic tumors may vary from child to
child and depend on where the tumor is located and whether it produces hormones.
Symptoms might mimic other, more common ailments. Some of the most
common symptoms include:
- No
symptoms if a tumor is not producing hormones or feelings of pain and fullness
if a non-functioning tumor grows large, pressing on nearby tissues and organs.
- Low
blood sugar, changes in behavior, seizures, or coma from tumors that secrete
insulin (insulinomas).
- Stomach
ulcers, vomiting blood, abdominal and back pain, reflux, and diarrhea from tumors
that produce gastrin (gastrinomas). This is sometimes called Zollinger-Ellison
syndrome.
- Diarrhea,
dehydration, low sodium, weight changes, or high blood pressure from tumors that
make other hormones.
- Jaundice
(yellowing of the skin or eyes), itching, or light-colored stool from a tumor
located in the head of the pancreas that is disrupting the bile duct.
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 pancreatic tumor will depend on the type and location of the
tumor. Your child's doctor may recommend:
- Surgery, involving biopsy and removal of the entire
tumor and nearby tissue. A minimally invasive approach, such as laparoscopy, is
possible for many tumors.
- 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 prognosis will depend on the type of
tumor he or she has, the specific location of the tumor in the pancreas,
whether it is benign or malignant, and whether it has spread.
Children treated
for tumors of the pancreas 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.