title: Prognostic factors and secondary malignancies in childhood medulloblastoma.
abstract: OBJECTIVE
  Little is known of the outcome of long-term survivors of childhood medulloblastoma, one of the most common pediatric malignancies. To determine the potential for secondary malignancies, a retrospective outcome evaluation in 88 consecutive cases of childhood medulloblastoma was performed.
  
  
  METHODS
  The records of all patients with childhood medulloblastoma diagnosed at Children's National Medical Center in Washington, DC from 1969 through 1997 were reviewed.
  
  
  RESULTS
  The median follow-up time was 92 months (range 6-257 months). Overall survival was 59% at 5 years and 52% at 10 years. Univariate analysis showed that age at diagnosis, extent of surgical resection, presence of metastatic disease (M stage), ventriculoperitoneal shunt placement within 30 days from diagnosis, posterior fossa radiation therapy dose, and adjuvant chemotherapy significantly affected survival. Although based on small numbers, the risk of second neoplasms was significantly increased in this cohort. Multiple basal cell carcinomas developed in the areas of radiation therapy in two patients; these patients also had nevoid basal cell carcinoma syndrome (NBCCS) diagnosed. One other patient died of glioblastoma multiforme 8 years after treatment of medulloblastoma. A meningioma developed in another patient 10 years after radiation therapy.
  
  
  CONCLUSIONS
  As survival of medulloblastoma patients improves, increased surveillance regarding secondary malignancies is required, especially because radiation-induced tumors may occur many years after treatment. These two cases of NBCCS also illustrate the importance of considering the concomitant diagnosis of NBCCS in young patients with medulloblastoma. In those patients, alternative therapy should be considered to minimize radiation therapy-related sequelae.
authors.affiliation: Department of Hematology-Oncology, Children's National Medical Center, Washington, DC, USA.
authors.forename: T
authors.initials: T
authors.lastname: Stavrou
authors.forename: C M
authors.initials: CM
authors.lastname: Bromley
authors.forename: H S
authors.initials: HS
authors.lastname: Nicholson
authors.forename: J
authors.initials: J
authors.lastname: Byrne
authors.forename: R J
authors.initials: RJ
authors.lastname: Packer
authors.forename: A M
authors.initials: AM
authors.lastname: Goldstein
authors.forename: G H
authors.initials: GH
authors.lastname: Reaman
mesh_terms: D000293:Adolescent; D000328:Adult; D002280:Basal Cell Carcinoma / Q000209:etiology*; D002528:Cerebellar Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D017024:Chemotherapy, Adjuvant; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D008527:Medulloblastoma / Q000175:diagnosis* / Q000401:mortality / Q000628:therapy; D009364:Neoplasm Recurrence, Local; D009381:Neoplasms, Radiation-Induced / Q000209:etiology*; D011379:Prognosis; D011878:Radiotherapy; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000209:etiology*; D015996:Survival Rate