title: Quality of life in children with primitive neuroectodermal tumors (medulloblastoma) of the posterior fossa.
abstract: We reviewed our experience in 43 consecutive patients with primitive neuroectodermal tumors (medulloblastoma), PNET (MB), treated between 1975 and 1984, to characterize their quality of life and identify factors which impacted on long-term function. Twenty-four of forty-three (56%) of children are alive and free of disease, a median of 4.5 years after diagnosis. The quality of life was analyzed for the 24 long-term survivors. 79% (19 of 24) were functioning well in everyday activities. The median full-scale intelligence quotient (FSIQ), obtained a median of 3.5 years after diagnosis for those tested (n = 17) was 97, with all but 3 (12%) of the patients functioning in the normal range. Specific learning, memory and fine-motor disabilities were found in over one half of patients. Factors associated with poorer performance and lower FSIQ included preoperative obtundation, the need for a permanent shunt, younger age at diagnosis, and a complicated postoperative course. It is concluded that (1) the majority of long-term survivors have 'normal' intellectual function, but may have specific intellectual and academic disabilities, and (2) preoperative and postoperative factors strongly impact on the quality of life of survivors.
authors.affiliation: Division of Neurology, Children's Hospital of Philadelphia, Philadelphia.
authors.forename: R J
authors.initials: RJ
authors.lastname: Packer
authors.forename: R
authors.initials: R
authors.lastname: Sposto
authors.forename: T E
authors.initials: TE
authors.lastname: Atkins
authors.forename: L N
authors.initials: LN
authors.lastname: Sutton
authors.forename: D A
authors.initials: DA
authors.lastname: Bruce
authors.forename: K R
authors.initials: KR
authors.lastname: Siegel
authors.forename: L B
authors.initials: LB
authors.lastname: Rorke
authors.forename: P A
authors.initials: PA
authors.lastname: Littman
authors.forename: L
authors.initials: L
authors.lastname: Schut
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003388:Cranial Fossa, Posterior; D005260:Female; D006801:Humans; D007223:Infant; D007361:Intelligence Tests; D008297:Male; D008527:Medulloblastoma / Q000503:physiopathology / Q000523:psychology*; D011788:Quality of Life; D012888:Skull Neoplasms / Q000503:physiopathology / Q000523:psychology*