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14-September-2008 11:27:25 - Botryoid rhabdomyosarcoma Botryoid rhabdomyosarcoma Classification and external resources ICD-O: 8910/3 DiseasesDB 1557 MeSH D018233 Sarcoma botryoides or botryoid rhabdomyosarcoma is a subtype of embryonal rhabdomyosarcoma, that can be observed in the urinary bladder of infants and young children or the vagina in females, typically younger than age 8. The name comes from the gross appearance of grape bunches botryoid in Greek. Contents 1 Histology 2 Clinical characteristics 3 Epidemiology 4 Treatment and prognosis 5 External links 6 References 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 For botryoid rhabdomyosarcoma of the vagina, the most common clinical finding is vaginal bleeding1 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 External links humpath.com #12369 References ^ Rahaman, J and Cohen, CJ. Gynecologic Sarcomas. in Holland-Frei Cancer Medicine - 6th Ed. Kufe, DW et al ors. BC Decker Inc, Hamilton, Ontario, 2003. ^ Hilgers R 1975. Pelvic exenteration for vaginal embryonal rhabdomyosarcoma: a review. Obstet Gynecol 45 2: 175-80. PMID 1090863. ^ 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. doi:10.1016/j.ygyno.2006.03.033. PMID 16684558. ^ 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. ^ Rotmensch, J and Yamada, SD. Neoplasms of the Vulva and Vagina. in Holland-Frei Cancer Medicine - 6th Ed. Kufe, DW et al ors. BC Decker Inc, Hamilton, Ontario, 2003. v d e Pathology: Tumor, Neoplasm, Cancer, and Oncology C00-D48, 140-239 Benign tumors Hyperplasia - Cyst - Pseudocyst - Hamartoma - Benign tumor Malignant progression Dysplasia - Carcinoma in situ - Cancer - Metastasis Topography Oral - Head/Neck - Nasopharyngeal - digestive system - Respiratory system - Bone - Skin - Blood - Urogenital - Nervous system - Endocrine system Histology Carcinoma - Sarcoma - Papilloma - Adenoma Misc. Tumor suppressor genes/oncogenes - Staging/grading - Carcinogenesis - Carcinogen - Research - Paraneoplastic syndrome - List of oncology-related terms v d e Connective/soft tissue tumors and sarcomas ICD-O 8800-9179 C45-C49/D17-D21, 171/214-215 Not otherwise specified 8800-8809 Soft tissue sarcoma - Desmoplastic small round cell tumor Fibromatous 8810-8839 Fibroma/fibrosarcoma - Malignant fibrous histiocytoma - Dermatofibroma/dermatofibrosarcoma - Dermatofibrosarcoma protuberans Myxomatous 8840-8849 Myxoma/myxosarcoma - Angiomyxoma - Ossifying fibromyxoid tumour Lipomatous 8850-8889 Lipoma/liposarcoma Angiomyolipoma Myomatous 8890-8929 Myoma/myosarcoma Leiomyoma/leiomyosarcoma, Rhabdomyoma/rhabdomyosarcoma/Sarcoma botryoides Complex Mixed And Stromal 8930-8999 Adenomyoma - Pleomorphic adenoma - Mixed Müllerian tumor - Mesoblastic nephroma - Wilms' tumor - Rhabdoid tumour - Clear cell sarcoma of the kidney - Hepatoblastoma - Pancreatoblastoma - Carcinosarcoma Fibroepithelial 9000-9039 Brenner tumour - Fibroadenoma - Phyllodes tumor Synovial-like 9040-9049 Synovial sarcoma - Clear cell sarcoma, NOS Mesothelial 9050-9059 Mesothelioma - Adenomatoid tumor Germ cell tumors 9060-9119 Germinomatous Germinoma / Seminoma / Dysgerminoma Nongerminomatous Embryonal carcinoma - Endodermal sinus tumor / Yolk sac tumor - Teratoma / Fetus in fetu / Dermoid cyst / Struma ovarii - Gestational trophoblastic disease Hydatidiform mole - Choriocarcinoma - Polyembryoma - Gonadoblastoma see also disorders Retrieved from http://en..org/wiki/Botryoid_rhabdomyosarcoma Categories: Oncology | Gynecology | Pediatrics | Rare cancers Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages Deutsch This page was last modified on 31 July 2008, at 13:40
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