Is “watchful waiting” superior to surgery in children with neurofibromatosis type 1 presenting with extracranial and extramedullary tumor mass at diagnosis?
Identifieur interne : 000335 ( Main/Exploration ); précédent : 000334; suivant : 000336Is “watchful waiting” superior to surgery in children with neurofibromatosis type 1 presenting with extracranial and extramedullary tumor mass at diagnosis?
Auteurs : Wojciech Wozniak [Pologne] ; Marek W. Karwacki [Pologne, Niger]Source :
- Child's Nervous System [ 0256-7040 ] ; 2008-12-01.
English descriptors
Abstract
Abstract: 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 cm3. 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 cm3. 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.
Url:
DOI: 10.1007/s00381-008-0668-7
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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 cm3. 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). 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