Pediatric rhabdomyosarcoma

Bowman F, Champigneulle J, Schmitt C, et al. Clear cell rhabdomyosarcoma. Pediatr Pathol Lab Med. 1996 16 951-959. 

Vincristine has been effectively combined with prednisone for remission induction in acute lymphoblastic leukemia in children. It is also active in various hematologicmalignancies such as Hodgkin s and non-Hodgkin s lymphomas, and multiple myeloma, and in several pediatric tumors including rhabdomyosarcoma, neuroblastoma, Ewing s sarcoma, and Wilms tumor. 

The antibiotics that bind to DNA are nonspecific to the cell-cycle phase. Dactinomycin (actinomycin D and Cosmegen) binds to double-stranded DNA and prevents RNA synthesis by inhibiting DNA-dependent RNA polymerase. It is administered intravenously in the treatment of pediatric solid tumors such as Wilms tumor and rhabdomyosarcoma and for gestational choriocarcinoma. Dactinomycin causes skin reactions, gastrointestinal injury, and delayed bone marrow depression. 

Dactinomycin is mainly used to treat pediatric tumors such as Wilms tumor, rhabdomyosarcoma, and Ewing s sarcoma, but it has activity also against germ cell tumors and gestational trophoblastic disease. Dactinomycin can also induce a "radiation recall reaction." See Table 55 1 for other toxi cities. 

Kobayshi M, Sjoberg G, Soderhall S, et al. Pediatric rhabdomyosarcomas express the intermediate filament nestin. Pediatr Res. 1998 43 86-92. 

Carpentieri DF, Nichols K, Chou PM, et al. The expression of WT-1 in the differentiation of rhabdomyosarcoma from other pediatric small rotmd blue cell trrmors. Mod Pathol. 2002 15 1080-1086. 

Raney RB, Anderson JR, Barr FG, et al. Rhabdomyosarcoma and undifferentiated sarcoma in the first two decades of life a selective review of intergroup rhabdomyosarcoma study group experience and rationale for Intergroup Rhabdomyosarcoma Study V. J Pediatr Hematol Oncol. 2001 23 215. 

Qualman SJ, Coffin CM, Newton WA, et al. Intergroup Rhabdomyosarcoma Study update for pathologists. Pediatr Dev Pathol. 1998 1 550-561. 

Michelagnoli MP, Burchill SA, Gullinane G, et al. Myogenin— a more specific target for RT-PGR detection of rhabdomyosarcoma than MyoDl. Med Pediatr Oncol. 2003 40 1-8. 

Tokuc G, Dogan O, Ayan I, et al. Prognostic value of prolifet-ating cell nuclear antigen immunostaining in pediatric rhabdomyosarcomas. Acta Paediatr]pn. 1998 40 573-579. 

Ayan I, Dogan O, Kebudi R, et al. Immunohistochemical detection of p53 protein in rhabdomyosarcoma association with clinicopathological features and outcome. J Pediatr Hema-tol Oncol. 1997 19 48-53. 

Roebuck DJ, Yang WT, Lam WWM et al (1998) Hepatobiliary rhabdomyosarcoma in children diagnostic radiology. Pediatr Radiol 22 101-108... 

Magnusson S, Gisselsson D, Wiebe T, Kristofferson U, Borg A, Olsson H. Prevalence of germline TP53 mutations and history of Li-Fraumeni syndrome in families with childhood adrenocortical tumors, choroid plexus tumors, and rhabdomyosarcoma a population-based survey. Pediatr Blood Cancer. 2012 50 846-53. 

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