- Botryoid rhabdomyosarcoma
Infobox_Disease
Name = PAGENAME
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DiseasesDB = 1557
ICD10 =
ICD9 =
ICDO = 8910/3
OMIM =
MedlinePlus =
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eMedicineTopic =
MeshID = D018233Sarcoma botryoides or botryoid rhabdomyosarcoma is a subtype of
embryonal rhabdomyosarcoma , that can be observed in theurinary bladder of infants and young children or thevagina in females, typically younger than age 8. The name comes from the gross appearance of "grape bunches" ("botryoid" in Greek).Histology
Under the
microscope one can seerhabdomyoblast s that may contain cross-striations.Tumor cells are crowded in a distinct layer beneath the vaginalepithelium (cambium layer ).Clinical characteristics
For botryoid rhabdomyosarcoma of the vagina, the most common clinical finding is vaginal bleeding [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.] but vaginal bleeding is not specific for sarcoma botryoides: other
vaginal cancer s are possible.Epidemiology
Sarcoma botryoides normally is found in children under 8 years of age. Onset of
symptom s occurs at age 3 years (38.3 months) on average. [cite journal |author=Hilgers R |title=Pelvic exenteration for vaginal embryonal rhabdomyosarcoma: a review |journal=Obstet Gynecol |volume=45 |issue=2 |pages=175–80 |year=1975 |pmid=1090863] Cases of older women with this condition have also been reported. [cite journal |author=Reynolds E, Logani S, Moller K, Horowitz I |title=Embryonal rhabdomyosarcoma of the uterus in a postmenopausal woman. Case report and review of the literature |journal=Gynecol Oncol |volume=103 |issue=2 |pages=736–9 |year=2006 |pmid=16684558 |doi=10.1016/j.ygyno.2006.03.033]Treatment and prognosis
The disease used to be uniformly fatal, with a 5-year survival rate between 10 to 35%. [cite journal |author=Piver M, Rose P |title=Long-term follow-up and complications of infants with vulvovaginal embryonal rhabdomyosarcoma treated with surgery, radiation therapy, and chemotherapy |journal=Obstet Gynecol |volume=71 |issue=3 Pt 2 |pages=435–7 |year=1988 |pmid=3347430] As a result, treatment was
radical surgery . New multidrugchemotherapy regimens with or withoutradiation therapy are now used in combination with less radical surgery with good results, although outcome data are not yet available. [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.]External links
* [http://www.humpath.com/spip.php?page=article&id_article=12639 humpath.com #12369]
References
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