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.
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.
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.
Many different
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
hormones.
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
environmental factors.
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.
Adrenocortical
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:
- No
symptoms or abdominal pain, fullness, or the presence of an abdominal lump if
the tumor is not producing hormones.
- Weight
changes, high blood pressure, or early onset of pubertal changes (voice
deepening, pubic hair, body odor, acne, or breast tissue developing early) if a
tumor is producing hormones.
- High
blood pressure, rapid pulse, headache, nausea, or sweating from a
pheochromocytoma.
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.
Treatment for
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:
- Surgery, involving biopsy and/or removal of the entire
tumor and nearby tissue and/or adrenal gland. A minimally invasive approach, or
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.
- Sometimes
before removing a pheochromocytoma, your child’s physician may prescribe
medicine to control high blood pressure.
- Medication
to treat excessive hormone production in functioning tumors.
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.
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.