Sarcoma botryoides is also known as?
Benign tumors of striated muscle are rare. There are three main types of Rhabdomyosarcoma; Embryonal, Pleomorphic and Alveolar.
1. Embryonal Rhabdomyosarcoma (Sarcoma botryoides)
Usually arises in the orbit or genitourinary tract in children. The botryoid type of Embryonal Rhabdomyosarcoma, which frequently originates in mucosa-lined visceral organs such as the vagina and the urinary bladder, generally grows as a polypoid tumor. These tumors may disseminate widely but are responsive to chemotherapy and radiation.
Embryonal Rhabdomyosarcoma occasionally arises in adults. Although regression of tumors in response to pediatric chemotherapy regimens usually occurs, age is an important prognostic factor for survival, with worse outcomes in older patients.
2. Alveolar Rhabdomyosarcoma
Extreme Rhabdomyosarcoma in adolescents and young adults often has alveolar histology. Alveolar Rhabdomyosarcoma is composed of ill-defined aggregates of poorly differentiated round or oval cells that frequently show central loss of cellular cohesion and the formation of irregular alveolar spaces.
These tumors appear to have a worse prognosis than Embryonal Rhabdomyosarcoma in younger children, but not in adults. A specific translocation, t (2, 13) (q37; q14) involving the PAX3 gene on chromosome 2 and the FKHR gene on chromosome 13, is seen in the majority of alveolar Rhabdomyosarcoma.
3. Pleomorphic Rhabdomyosarcoma
In adults, Pleomorphic Rhabdomyosarcoma is the most common form of Rhabdomyosarcoma.