Sarcoma botryoides

Sarcoma botryoides, also known as embryonal rhabdomyosarcoma, is a rare vaginal cancer. It occurs in the vagina of female infants and children, typically younger than age 8. The name comes from the gross appearance of "grape bunches" (botryoid in Greek).

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Histology

Under the microscope one can see rhabdomyoblasts that may contain cross-striations. Tumor cells are crowded in a distinct layer beneath the vaginal epithelium ( cambium layer).

Clinical characteristics

The most common clinical finding is vaginal bleeding^[1] but vaginal bleeding is not specific for sarcoma botryoides: other vaginal cancers are possible.

Epidemiology

Sarcoma botryoides normally is found in children under 8 years of age. Onset of symptoms occurs at age 3 years (38.3 months) on average.^[2] Cases of older women with this condition have also been reported.^[3]

Treatment and prognosis

The disease used to be uniformly fatal, with a 5-year survival rate between 10 to 35%.^[4] As a result, treatment was radical surgery. New multidrug chemotherapy regimens with or without radiation therapy are now used in combination with less radical surgery with good results, although outcome data are not yet available.^[5]

References