Sarcoma botryoides
Sarcoma botryoides
Classification & external resources
| ICD-O: |
8910/3 |
| DiseasesDB |
1557 |
| MeSH |
D018233 |
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).
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
- ^ 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.
- ^ 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. 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 editors. BC Decker Inc, Hamilton, Ontario, 2003.
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Pathology: Cancer, Tumors, neoplasia, and oncology (C00-D48, 140-239) |
| Benign tumors |
Hyperplasia - Cyst - Pseudocyst - Hamartoma - Benign tumor |
| Malignant progression |
Dysplasia - Carcinoma in situ - Cancer - Metastasis |
| Topography |
lip, oral cavity and pharynx: Oral - Head/Neck - Nasopharyngeal
digestive system: tract (Esophagus, Stomach, Small intestine, Colon/rectum, Appendix, Anus) - glands (Liver, Bile duct, Gallbladder, Pancreas)
respiratory system: Larynx - Lung
bone, articular cartilage, skin, and connective tissue: Bone - Skin - Blood
urogenital: breast and female genital organs (Breast, Vagina, Cervix, Uterus, Endometrium, Ovaries) - male genital organs (Penis, Prostate, Testicles) - urinary organs (Kidney, Bladder)
nervous system: Eye - Brain
endocrine system: Thyroid (Papillary, Follicular, Medullary, Anaplastic) - Adrenal tumor (Adrenocortical carcinoma, Pheochromocytoma) - Pituitary |
| Misc. |
Tumor suppressor genes/oncogenes - Staging/grading - Carcinogenesis - Carcinogen - Research - Paraneoplastic syndrome - List of oncology-related terms |
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