Adrenal Tumors

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.

• Get answers to common questions about clinical trials for cancer and blood disorders.
• Contact us: If you’re not sure which clinical trials might be right for your child, email us at clinicaltrials@danafarberbostonchildrens.org. We can help you navigate your options.

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.