title: Rhabdoid tumors in children: prognostic factors in 70 patients diagnosed in Germany.
abstract: We retrospectively analysed the data of 70 patients (41 boys and 29 girls) with Rhabdoid tumors (RT) regardless of localisation, recorded in the German Childhood Cancer Registry (GCCR) from 1984 to 1999. The primary tumor was located in the kidney in 32 cases, in the central nervous system (CNS) in 13 cases and in the soft tissue in 25 cases. Variables examined were tumor stage, sex, age at diagnosis, surgical radicality, radiotherapy and chemotherapeutic regimens. Metastatic disease at diagnosis was observed in 18 of the 70 individuals. Outcome of this group was very poor with a 5-year overall survival of 11%. There were no differences in survival between males and females, or younger and older children. Chemotherapeutic regimens were mainly given according to the primary site of the tumor. Radiotherapy was given in 28 of the 70 patients with a mean dose of 35 Gray, though this did not improve the outcome. Overall survival of the whole cohort was 27% at 5 years and there was no significant difference in prognosis regarding the different locations of the tumor (kidney 24%, soft tissue 30%, CNS 29%). In conclusion, RT in infants and children has a dismal prognosis, independent from localisation. The presence of metastasis at diagnosis seems to be the only prognostic factor of outcome.
authors.affiliation: Department of Pediatric Hematology and Oncology, University Hospital, D-66421 Homburg/Saar, Germany. harald.reinhard@uniklinikum-saarland.de
authors.forename: Harald
authors.initials: H
authors.lastname: Reinhard
authors.forename: Jennifer
authors.initials: J
authors.lastname: Reinert
authors.forename: Rita
authors.initials: R
authors.lastname: Beier
authors.forename: Rhoikos
authors.initials: R
authors.lastname: Furtwängler
authors.forename: Muhanat
authors.initials: M
authors.lastname: Alkasser
authors.forename: Stefan
authors.initials: S
authors.lastname: Rutkowski
authors.forename: Michael
authors.initials: M
authors.lastname: Frühwald
authors.forename: Ewa
authors.initials: E
authors.lastname: Koscielniak
authors.forename: Ivo
authors.initials: I
authors.lastname: Leuschner
authors.forename: Peter
authors.initials: P
authors.lastname: Kaatsch
authors.forename: Norbert
authors.initials: N
authors.lastname: Graf
mesh_terms: D017668:Age of Onset; D002675:Child, Preschool; D005260:Female; D005858:Germany; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D009367:Neoplasm Staging; D011379:Prognosis; D018335:Rhabdoid Tumor / Q000401:mortality* / Q000473:pathology* / Q000628:therapy; D015996:Survival Rate