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Is "watchful waiting" superior to surgery in children with neurofibromatosis type 1 presenting with extracranial and extramedullary tumor mass at diagnosis? | LitMetric

Is "watchful waiting" superior to surgery in children with neurofibromatosis type 1 presenting with extracranial and extramedullary tumor mass at diagnosis?

Childs Nerv Syst

NF Outpatient Clinic, Clinical Department of Oncological Surgery for Children and Youth, National Research Institute of Mother and Child, Kasprzaka 17a, 01-211, Warsaw, Poland.

Published: December 2008

Aim: The aim of this study was to compare aggressive surgery to "watchful waiting" in neurofibromatosis type 1 (NF-1) patients with benign peripheral nerve sheath tumors exceeding 150 cm(3).

Materials And Methods: Observational study based on imaging studies and results of surgery was utilized.

Results: Twenty-seven out of 114 NF-1 patients (mean age of 12.8 years) registered throughout the last 10 years were studied. The mean tumor volume was 237 cm(3). Among them, 18 (66%) underwent surgery. In 15 (84%), regrowth of a tumor was observed. Reoperation was necessary in five (27%). Two out of these five children (2/18, 11%) required a third operation. Stable process has been shown in the remaining nine children (34%) since diagnosis and in most operated children (10/18; 56%), besides the regrowth of the tumor.

Conclusions: "Watchful waiting" could be superior to aggressive surgery in selected patients without "oncological awareness" confirmed by imaging techniques (mostly magnetic resonance imaging). NF-1 children with gross tumor mass require lifetime imaging monitoring and complex medical evaluation.

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Source
http://dx.doi.org/10.1007/s00381-008-0668-7DOI Listing

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