Vulvovaginal rhabdomyosarcoma

Definition

Vulvovaginal rhabdomyosarcoma is a rare vulvovaginal tumour, a highly malignant soft tissue sarcoma composed of cells with round to oval or spindle-shaped nuclei and eosinophilic cytoplasm that may show differentiation towards striated muscle cells. It usually affects children and presents with a vulvar or vaginal mass that may be polypoid or grape-like (embryonal subtype) and associated with bleeding and ulceration.

Disease data

Classification

Disease

ORPHA code

206492

OMIM code

ICD10 code

C52

ICD11 code

2C71.Y

*Soruce