Endocrine Tumors

Also called: neuroendocrine tumors

What are endocrine tumors?

Endocrine tumors affect the organs that make hormones. Hormones are chemicals that travel through your bloodstream and send signals that control body functions such as growth and development, metabolism, mood and reproduction.

Most endocrine tumors in children are either:

  • Noncancerous (benign) or
  • Low-grade cancers that grow and spread slowly.

A small percentage of endocrine tumors are high-grade (fast-growing) cancers. These can include some of the following types of tumors:

  • Gonadal tumors (affecting the testes or ovaries)
  • Germ cell tumors
  • Thyroid cancers
  • Adrenocortical tumors

Where endocrine tumors begin determines the body functions they affect. These endocrine tumors occur in children and adolescents:

  • Tumors in the gonads. These include testicular and ovarian tumors as well as germ cell tumors. Germ cell tumors start from the cells in an unborn baby that develop into the child’s reproductive system.
  • Childhood cancers of the parathyroid glands, adrenal medulla and pancreas are very rare.
  • Rarely, some childhood endocrine cancers (such as thyroid tumors) occur after treatment (such as neck radiation) for another cancer.
  • Some endocrine tumors (including some types of thyroid or adrenal cancers) occur as part of hereditary syndromes (passed down in families). These include:
    • Multiple endocrine neoplasia (MEN) syndrome, in which cancers can grow in more than one endocrine gland
    • Von Hippel-Lindau syndrome, in which tumors such as pheochromocytomas may grow. Pheochromocytomas are rare, usually noncancerous tumors that develop in the adrenal glands. These tumors can raise heart rate and blood pressure.

What are the symptoms of endocrine tumors?

Symptoms of endocrine tumors vary according to tumor type. If your child has an endocrine tumor, you may see some of these general symptoms:

  • A thickening or lump in any part of the body
  • Ongoing pain in a specific area
  • Anxiety or panic attacks
  • Feeling nervous
  • Feeling irritable
  • Confusion
  • Depression
  • Fatigue
  • Fever
  • Sweating
  • Headaches
  • Nausea or vomiting
  • Diarrhea or other changes in bowel or bladder habits
  • Unexplained weight gain or loss
  • Intestinal bleeding
  • Jaundice (yellowing of the skin and the whites of the eyes)
  • Facial flushing (red cheeks that feel warm)

How are endocrine tumors treated?

Multiple treatments may be used.

  • Surgery — is considered the best way to treat most endocrine tumors. The goal is to remove the entire tumor or as much as possible.

Chemotherapy, radiation and/or hormone replacement therapy may be given along with or after surgery. The goal is to lessen the chance that the tumor will return by killing or reducing the number of cells that may remain.

  • Chemotherapy (“chemo”) — uses powerful medicines to kill cancer cells or stop them from growing (dividing) and making more cancer cells. Chemotherapy is especially effective in germ cell tumors of the ovaries and testes.
    • Chemo may be injected into the bloodstream, so that it can travel throughout the body.
    • Some chemo may be given by mouth.
    • Combination therapy uses more than one type of chemo at a time.
  • Radiation therapy — uses high-energy X-rays or other types of radiation to kill cancer cells or stop them from growing. Radiation therapy may lessen the chance of the cancer returning, even if the tumor is resistant to radiation or has been completely removed.
    • External radiation uses machines outside the body to deliver the X-ray dose.
    • Internal radiation uses needles, seeds, wires or catheters (tubes) to deliver the radiation directly into or close to the cancer.
    • Some types of thyroid cancer use radioactive iodine to find where the cancer may have spread and to help treat cancer that is left after surgery.
  • Hormone therapy — can lower hormone levels in the body to help stop the tumor from growing or relieve tumor symptoms
    • Sometimes the patient’s thyroid gland will be removed because of thyroid cancer. Hormone therapy is typically used to replace hormones needed for normal body functioning. For some types of thyroid cancer, this is also a key part of the treatment to decrease the chance that cancer would grow or come back.

What are the survival rates for endocrine tumors?

Survival rates for treated patients for endocrine tumors depend on the tumor location:

  • For most patients with pituitary tumors and thyroid tumors and ovarian or testicular germ cell tumors, the prognosis is very good.
  • For patients with adrenal tumors, five-year survival rates range from below 40 percent to greater than 85 percent, depending on how much the disease has spread when it is diagnosed.

Why choose St. Jude for your child’s endocrine tumor treatment?

  • St. Jude is the only National Cancer Institute-designated Comprehensive Cancer Center devoted solely to children.
  • St. Jude has created more clinical trials for cancer than any other children’s hospital in the United States.
  • The nurse-to-patient ratio at St. Jude is unmatched— averaging 1:3 in hematology and oncology, and 1:1 in the Intensive Care Unit.
  • Complete, quality surgery is an important part of treating endocrine tumors. Cancer surgery in children requires expertise, because of the risks of not completely removing the tumor, not completely assessing disease spread, and injuring blood vessels or nerves. For example, adrenal glands can be fragile, and tumors may have clots in the large veins. The expert skills and experience of St. Jude specialty surgeons can help improve a child’s chances for the best outcome. Due to hormonal changes for some types of endocrine tumors, expert care before, during and after surgery is also key.
  • For germ cell tumors, scientists focus on reducing the amount of chemo while maintaining excellent cure rates. Because these tumors are so rare, clinical trials are nationwide with many institutions working together.
  • Scientists at St. Jude found specific mutations (changes) in the p53 gene that increase the likelihood that a person will develop adrenocortical tumors.
  • St. Jude brings the minds and efforts of doctors and scientists interested in adrenocortical tumors together. For example, St. Jude scientists created an adrenal tumor model in the lab that researchers use to test drugs for ACT. St. Jude doctors and scientists from the clinic and the lab regularly meet together, in person and online with partners elsewhere, to discuss the best new ideas for adrenocortical tumors.
  • Partly as a result of research conducted at St. Jude, the Children’s Oncology Group created a multi-institutional treatment program that studies the biology of the tumors and the incidence of the different types of p53 mutations.
  • St. Jude takes parts in international studies, including work with the Children’s Oncology Group to study how to provide the safest and most effective treatments for patients with both small tumors and tumors that have spread.
  • St. Jude offers a dedicated team of specialists to meet the needs of children with endocrine tumors, including: surgeons; doctors and nurses who treat this cancer; doctors who specialize in radiation therapy, in hormones (endocrinologists), and in diagnosing these tumors (pathologists); experts in diagnostic imaging and nuclear medicine; genetic counselors; dietitians; child life specialists; psychologists; Quality of Life team members; experts who help manage and minimize the long-term or late effects of treatment; researchers; scientists; and many others.

The St. Jude Web site is designed for educational purposes only and is not engaged in rendering medical advice or professional services. The information provided through this site should not be used for diagnosing or treating a health problem or a disease. It is not a substitute for professional care. If you have or suspect you may have a health problem, you should consult your health care provider.