Paragangliomas and pheochromocytomas are
tumors that develop out of the neuroendocrine tissue responsible for making epinephrine.
This hormone, also known as adrenaline, and other related hormones help
regulate heart rate and blood pressure in response to stress.
Paragangliomas, known as PGLs,
can develop anywhere in the body from the pelvis to the skull. Some release
hormones that are similar to adrenaline and others do not.
Pheochromocytomas, known as PCCs, are
found specifically in the adrenal gland where adrenaline is produced. PCCs
secrete epinephrine and norepinephrine hormones.
PCCs and PGLs are rare and often
slow growing. Most are benign but can cause problems such as high blood
pressure, sweating, and headaches when they produce hormones. Many are
associated with genetic or inherited conditions. Hereditary
paraganglioma-pheochromocytoma is an inherited condition that occurs when
benign tumors grow in the paraganglia.
Children with PGLs and PCCs 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.
Continue reading for more information about
paragangliomas and pheochromocytomas or visit the Endocrine-Oncology
Program page to learn more about our expertise or meet our treatment
team.
As a parent,
you undoubtedly want to know what may have caused your child’s tumor. Sometimes, tumors emerge with no known cause. Some result
from a mix of genetic and environmental factors, while others are linked to
inherited conditions.
Examples of conditions
associated with PGLs and PCCs include hereditary paraganglioma-pheochromocytoma
syndrome, neurofibromatosis, von Hippel-Lindau disease, multiple endocrine
neoplasia syndromes, tuberous sclerosis, Sturge-Weber syndrome, and
ataxia-telangiectasia.
The symptoms of PCCs or PGLs 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.
For PCCs/PGLs,
the most common symptoms are:
- high
blood pressure
- rapid
pulse
- heart
palpitations
- headache
- dizziness
- poor
weight gain despite good appetite
- abdominal
pain
- nausea
- vomiting
- pale
skin
- clammy
skin
- sweating
- growth
failure
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 paraganglioma or pheochromocytoma will depend on the location and
type of your child’s 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 or thoracoscopy is possible for many tumors.
- Sometimes
before removing a pheochromocytoma, your child’s physician may prescribe
medicine to control high blood pressure.
There are also some newer agents that are
being investigated to reduce the need for big surgeries for advanced
paragangliomas.
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 a number of
factors. These include whether the tumor is benign or malignant, whether it has
spread, whether it is producing hormones, or whether it has recurred. If your
child’s PCC or PGL is due to an inherited or genetic condition, it is likely
that your doctor will recommend screening to detect new tumors that could grow
in the future.