Rhabdomyosarcoma

Alternative names:

embryonal cell sarcoma; sarcoma botryoides

Definition:

A highly malignant (cancerous), soft tissue tumor in children. A rhabdomyosarcoma tends to affect 4 major anatomic sites: the head and neck, the genito-urinary tract, the extremities, and the trunk.

Causes, incidence, and risk factors:

The cause of rhabdomyosarcoma is unknown. Increased incidence is seen in families with a history of brain tumors and early breast cancer. In young children, the tumor affects all of the 4 major anatomic areas, while in the adolescent it is most common in the genitourinary system.

The tumor may appear as a swelling or lump in the soft tissue. There may be no pain. The botryoides type, which is more common in the vagina, generally has the appearance of a grape-like cluster and may protrude from the vagina.

Diagnosis of rhabdomyosarcoma is often delayed because of lack of symptoms and its appearance may coincide with a recent injury. Early diagnosis is important because rhabdomyosarcoma is an aggressive tumor that metastasizes (spreads) early.