Adrenal tumors are masses that grow in the
adrenal gland, the organ responsible for producing hormones in response to
physical and emotional stress.
Adrenal tumors may be functioning or
non-functioning, meaning that some produce hormones and some do not.
Non-functioning tumors don’t make excess hormones. Functioning tumors produce
extra hormones that can cause problems such as early puberty, high blood
pressure, sweating, headaches, and abdominal pain.
Many different types of adrenal tumors
exist including adrenal adenomas, adrenocortical carcinomas (ACCs), and
pheochromocytomas. Adrenal adenomas are relatively common, occurring in some 5%
to 10% of the population, while ACCs are rare. In children, ACCs are most
common in patients under the age of six and in teenagers, and they are more
common in girls than in boys. If an ACC is diagnosed, genetic counseling is
recommended as many occur in the setting of a hereditary cancer syndrome called
Children with adrenal tumors are treated at Dana-Farber/Boston
Children's through our Endocrine-Oncology Program.
Advanced cancers may also be treated through 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.
Continue reading for more information about adrenal tumors or visit the Endocrine-Oncology
Program page to learn more about our expertise or meet our treatment
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.
types of adrenal tumors exist, including adrenal adenomas, adrenocortical
carcinomas, and pheochromocytomas. Adrenal tumors can be either functioning or
non-functioning. Functioning tumors produce an excess of hormones that may include
aldosterone, cortisol, sex hormones, catecholamines, or a combination of these
Adenomas and pheochromocytomas are most often
benign tumors, although pheochromocytomas can occasionally become malignant.
ACCs are malignant tumors and will be further classified by their stage.
As a parent,
you undoubtedly want to know what may have caused your child’s tumor. It is important to understand that tumors often emerge
with no known cause. Many may result from the combined effects of genetic and
Some cancers are caused by inherited conditions.
Disorders associated with pheochromocytomas include neurofibromatosis, von
Hippel-Lindau disease, multiple endocrine neoplasia (MEN) syndromes, tuberous
sclerosis, Sturge-Weber syndrome, and ataxia-telangiectasia.
carcinomas are often linked to genetic
conditions, most often Li-Fraumeni syndrome. About 50% to 80% of pediatric ACC
patients have this syndrome. Other related conditions include MEN1, Lynch syndrome,
Beckwith-Wiedemann syndrome, and hemihypertrophy.
The symptoms of adrenal tumors may vary from child to child
and depend on where the tumor is located and what kind it is. Symptoms might
mimic other, more common ailments. Some common symptoms may include:
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:
your child's adrenal tumor will depend on the type of tumor your child has and whether
it is malignant. 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, she may be eligible for a number of
experimental therapies available through these groups or from one of our
independent clinical investigators.
In general, children treated for adrenal adenomas and
pheochromocytomas enjoy an excellent prognosis, with a full recovery expected
in many cases.
Children with adrenocortical carcinomas have the best
outlook if their tumor is small and can be completely removed surgically. If
not, the child’s prognosis will depend on a number of factors, including age
and whether the cancer has spread. For children with genetic risk factors that
can increase their risk of developing other tumors in the future, long-term
follow up may be needed.
Children who have been treated for adrenal 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.