Adrenal Tumors

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.

There are different types of adrenal tumors, including adrenal adenomas, adrenocortical carcinomas (ACCs), and pheochromocytomas. Adrenal adenomas are relatively common, occurring in some 5-to-10 percent 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 Li-Fraumeni syndrome.

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:

• Abdominal pain, fullness, or the presence of an abdominal lump
• Weight changes
• Early onset pubertal changes
• High blood pressure

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.

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 (ACCs) are often linked to genetic conditions, most often Li-Fraumeni syndrome. About 50 to 80 percent of pediatric ACC patients have this syndrome. Other related conditions include MEN1, Lynch syndrome, Beckwith-Wiedemann syndrome, and hemihypertrophy.

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:

• Physical exam
• Blood and urine tests
• Biopsy
• Magnetic resonance imaging (MRI)
• Computerized tomography scan (CT/CAT scan)
• Molecular testing

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, radiation, or chemotherapy.

Chemotherapy

A drug treatment that aims to destroy or shrink cancer cells, may be given before or after surgery. 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 with adrenal tumors are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Endocrine-Oncology Program. Advanced cancers may also be treated through our Solid Tumor Center. This 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.

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.

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.