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

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.