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Cancer Health Center

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Unusual Cancers of Childhood (PDQ®): Treatment - Patient Information [NCI] - Unusual Cancers of the Reproductive and Urinary Systems


See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for more information on testicular germ cell tumors.

Ovarian Cancer

Ovarian cancer is a disease in which malignant (cancer) cells form in the ovary. The ovaries are a pair of organs in the female reproductive system. They are located in the pelvis, one on each side of the uterus (the hollow, pear-shaped organ where a fetus grows). Each ovary is about the size and shape of an almond. The ovaries produce eggs and female hormones (chemicals that control the way certain cells or organs function).

Anatomy of the female reproductive system. The organs in the female reproductive system include the uterus, ovaries, fallopian tubes, cervix, and vagina. The uterus has a muscular outer layer called the myometrium and an inner lining called the endometrium.

Most ovarian tumors in children are benign (not cancer). They occur most often in females aged 15 to 19 years.

There are several common types of malignant ovarian tumors:

  • Germ cell tumors: Tumors that start in egg cells in females. These are the most common ovarian tumors in girls. (See the PDQ summary on Childhood Extracranial Germ Cell Tumors Treatment for more information on ovarian germ cell tumors.)
  • Epithelial tumors: Tumors that start in the tissue covering the ovary. These are the second most common ovarian tumors in girls.
  • Stromal tumors: Tumors that begin in stromal cells, which make up tissues that surround and support the ovaries.
  • Other tumors, such as Burkitt lymphoma and small cell carcinoma of the ovary (a very rare tumor).

Risk Factors, Signs and Symptoms, and Diagnostic and Staging Tests

The risk of ovarian cancer is increased by having one of the following conditions:

  • Ollier disease (a disorder that causes abnormal growth of cartilage at the end of long bones).
  • Maffucci syndrome (a disorder that causes abnormal growth of cartilage at the end of long bones and of blood vessels in the skin).
  • Peutz-Jeghers syndrome.

Ovarian cancer may cause any of the following signs and symptoms. Check with your child's doctor if your child has any of the following:

  • Painful menstrual periods.
  • A lump in the abdomen.
  • Pain or swelling in the abdomen.
  • Having male sex traits, such as body hair or a deep voice.
  • Early signs of puberty.
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