[Congenital botryoid rhabdomyosarcoma of the vulva].
Rhabdomyosarcoma is the most frequent malignant soft tissue tumor in pediatric patients; however, the vulvar location and congenital appearance are exceptional. We present the case of a newborn girl with botryoid rhabdomyosarcoma of the vulva, treated with chemotherapy, conservative surgery and autologous transplant. Botryoid rhabdomyosarcoma is a variation of embryonal rhabdomyosarcoma that typically grows in mucosa-lined hollow organs, from where it can spread to the body surface. The treatment of botryoid rhabdomyosarcoma in the genito-urinary area is based on polychemotherapy, and it can be complemented with radiotherapy and conservative surgery if necessary, thus resulting in an excellent prognosis and few long-term functional sequelae.
Servicio de Anatomía Patológica, Hospital Infantil Universitario Niño Jesús, Avda. Menéndez Pelayo 65, 28009 Madrid, Spain. icolmenero.hnjs@madrid.salud.org
Ana
A
Fábregues
Isabel
I
Colmenero
Antonio
A
Torrelo
Daniel
D
Azorín
Inmaculada
I
de Prada
Trinidad
T
Contra
Imelda
I
González-Mediero
Antonio
A
Zambrano
D005260:Female; D006801:Humans; D007231:Infant, Newborn; D012208:Rhabdomyosarcoma / Q000151:congenital* / Q000473:pathology; D014846:Vulvar Neoplasms / Q000151:congenital* / Q000473:pathology