PROGNOSTIC SIGNS IN THE SURGICAL-MANAGEMENT OF PLEXIFORM NEUROFIBROMA- THE CHILDRENS-HOSPITAL-OF-PHILADELPHIA EXPERIENCE, 1974-1994

Objectives: To estimate the rate of progression of plexiform neurofibr oma after surgery and to identify prognostic factors that predict prog ression. Study design: A retrospective review of the inpatient and out patient records of 121 patients, who had 302 procedures on 168 tumors over a 20-year period at a single large pediatric referral center. Dat a on age, location, indication for surgery, and extent of resection wa s analyzed for prognostic significance. Results: The overall freedom f rom progression was 54%. Children < 10 years old had a shorter interva l of tumor control than older children (p = 0.0004). Tumors of the hea d/neck/face fared worse than tumors of the extremities (p = 0.0003). L ess extensive resection predicted shorter interval to progression (p < 0.0001). Indication for surgery was not of prognostic importance. In multivariable analysis older age and location in the extremities were predictors of a better outcome. Conclusions: Tumor progression is a se rious problem for children and plexiform neurofibroma. Younger childre n, children with tumors of the head/neck/face, and tumors that cannot be nearly completely removed are at particular risk. These data may be useful in helping clinicians decide which patients and which tumors a re most likely to benefit from surgical intervention.