Prognostic signs in the surgical management of plexiform neurofibroma: The Children’s Hospital of Philadelphia experience, 1974-1994

Objectives: To estimate the rate of progression of plexiform neurofibroma after surgery and to identify prognostic factors that predict progression. Study design: A retrospective review of the inpatient and outpatient records of 121 patients, who had 302 procedures on 168 tumors over a 20-year perio...

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Published in:	The Journal of pediatrics Vol. 131; no. 5; pp. 678 - 682
Main Authors:	Needle, Michael N., Cnaan, Avital, Dattilo, James, Chatten, Jane, Phillips, Peter C., Shochat, Stephen, Sutton, Leslie N., Vaughan, Sheila N., Zackai, Elaine H., Zhao, Huaqing, Molloy, Patricia T.
Format:	Journal Article
Language:	English
Published:	New York, NY Elsevier Inc 01-11-1997 Elsevier
Subjects:	Adolescent Adult Biological and medical sciences Child Child, Preschool Female Head and Neck Neoplasms - epidemiology Head and Neck Neoplasms - surgery Hospitals, Pediatric Humans Infant Infant, Newborn Longitudinal Studies Male Medical sciences Neoplasms - epidemiology Neoplasms - surgery Neurofibroma, Plexiform - epidemiology Neurofibroma, Plexiform - surgery Neurofibromatosis 1 - epidemiology Neurofibromatosis 1 - surgery Neurology Pennsylvania Peripheral Nervous System Neoplasms - epidemiology Peripheral Nervous System Neoplasms - surgery Postoperative Complications - epidemiology Prognosis Retrospective Studies Spinal Neoplasms - epidemiology Spinal Neoplasms - surgery Time Factors Tumors of the nervous system. Phacomatoses Pennsylvania Human Nervous system diseases Prognosis Phacomatosis Surgery Cohort study Neurofibromatosis Central nervous system disease Benign neoplasm Child Neurofibroma Result
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Summary:	Objectives: To estimate the rate of progression of plexiform neurofibroma after surgery and to identify prognostic factors that predict progression. Study design: A retrospective review of the inpatient and outpatient records of 121 patients, who had 302 procedures on 168 tumors over a 20-year period at a single large pediatric referral center. Data on age, location, indication for surgery, and extent of resection was analyzed for prognostic significance. Results: The overall freedom from progression was 54%. Children < 10 years old had a shorter interval of tumor control than older children ( p = 0.0004). Tumors of the head/neck/face fared worse than tumors of the extremities ( p = 0.0003). Less 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 serious problem for children with plexiform neurofibroma. Younger children, 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 are most likely to benefit from surgical intervention. (J Pediatr 1997;131:678-82)
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0022-3476 1097-6833
DOI:	10.1016/S0022-3476(97)70092-1