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.
at Dana-Farber/Boston Children's
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
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
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
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.
the most common symptoms are:
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:
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 paraganglioma or pheochromocytoma will depend on the location and
type of your child’s tumor. Your child's doctor may recommend:
There are also some newer agents that are
being investigated to reduce the need for big surgeries for advanced
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, 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.
Lindsay Frazier, MD, explains how a multidisciplinary team of specialists come together to deliver care for solid tumors.