What are paragangliomas/pheochromocytomas?

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 (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.

How we care for PGLs and PCCs

Children with PGLs and PCCs are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center 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.

Our areas of research for PGLs and PCCs

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 for PGLs and PCCs

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.