Embryonal rhabdomyosarcoma, NOS
Rhabdomyosarcomas are the most frequent malignant soft tissue tumours in children and adolescents, with embryonal rhabdomyosarcoma as the most common subtype. Typical sites of involvement are the head and neck region and the genitourinary system. Phenotypically and biologically, embryonal rhabdomyosarcomas recapitulate features of embryonal skeletal muscle. Genetically, most cases show loss of chromosomal region 11p15
Alberto S. Pappo (Ed.)
Pediatric Bone and Soft Tissue Sarcomas
Springer Verlag: Berlin Heidelberg New York 2005
Three histological variants of embryonal rhabdomyosarcoma have been identified
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
IARC Press: Lyon 2002
Spindle cell rhabdomyosarcoma
Vulvar embryonal rhabdomyosarcomas are found almost exclusively in girls <10 years of age. The tumours typically arise from the labial or perineal area and form solid masses. Patients present with bleeding and ulceration. The prognosis depends on the clinical stage and the histological pattern. An alveolar histology is associated with a poor prognosis, whereas the survival rate for classic embryonal rhabdomyosarcoma is >90%
Copeland LJ, Gershenson DM, Saul PB, Sneige N, Stringer CA, Edwards CL (1985)
Sarcoma botryoides of the female genital tract.
Obstet Gynecol 66: 262-6
Copeland LJ, Sneige N, Stringer CA, Gershenson DM, Saul PB, Kavanagh JJ (1985)
Alveolar rhabdomyosarcoma of the female genitalia.
Cancer 56: 849-55
Tavassoli FA, Devilee P (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Breast and Female Genital Organs.
IARC Press: Lyon 2003