Sarcoma botryoides
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Overview
Sarcoma botryoides, also known as embryonal rhabdomyosarcoma, is a type of rhabdomyosarcoma in the vagina of young girls, typically younger than 8. The name comes from the gross appearance of "grape bunches" (botryoid in Greek).
Pathophysiology
Under the microscope one can see rhabdomyoblasts that may contain cross-striations. Tumor cells are crowded in a distinct layer beneath the vaginal epithelium, named the cambium layer.
History & Symptoms
The most common clinical finding is vaginal bleeding (Rahaman and Cohen, 2003).
Epidemiology
Sarcoma botryoides is found in children under 8 years of age with an average age of onset of symptoms of about 3 years (38.3 months) (Hilgers, 1975). Cases of older women with this condition have also been reported (Reynolds et al 2006)
Treatment and prognosis
The disease used to be uniformly fatal, with a 5-year survival rate between 10 to 35% (Piver and Rose, 1988). 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 (Rotmensch and Yamada, 2003).
References
- Hilgers R (1975). "Pelvic exenteration for vaginal embryonal rhabdomyosarcoma: a review". Obstet Gynecol. 45 (2): 175–80. PMID 1090863.
- Piver M, Rose P (1988). "Long-term follow-up and complications of infants with vulvovaginal embryonal rhabdomyosarcoma treated with surgery, radiation therapy, and chemotherapy". Obstet Gynecol. 71 (3 Pt 2): 435–7. PMID 3347430.
- Rahaman, J and Cohen, CJ. Gynecologic Sarcomas. in Holland-Frei Cancer Medicine - 6th Ed. Kufe, DW et al editors. BC Decker Inc, Hamilton, Ontario, 2003.
- Reynolds E, Logani S, Moller K, Horowitz I (2006). "Embryonal rhabdomyosarcoma of the uterus in a postmenopausal woman. Case report and review of the literature". Gynecol Oncol. 103 (2): 736–9. PMID 16684558.
- Rotmensch, J and Yamada, SD. Neoplasms of the Vulva and Vagina. in Holland-Frei Cancer Medicine - 6th Ed. Kufe, DW et al editors. BC Decker Inc, Hamilton, Ontario, 2003.