Dados do Trabalho

Título

Pediatric Skull Base Tumors: literature review and analysis of 17 cases in a single center

Objetivo

The current study aims to review our personal experience and the literature about skull base surgery in children younger than 18 years-old, accessing epidemiology, surgery complications, functional outcomes and mortality.

Materiais e Métodos/Casuística

The paper describes our experience with 17 children operated for skull base tumors describing age, imagining, tumor location, surgery performed, histopathological diagnosis, degree of resection, surgical complications, adjuvant therapies and recurrence/progression. Additionally, a literature review was carried out describing a total of 267 children operated for skull base tumors.

Resultados

Of a total of 17 children most were male (11/64,7%). Mean age was 10.5 years-old. The neoplasms histology includes 3 schwannomas (17.6%) and 2 meningiomas (11.7%), and others individual occurrences. The main tumor localization was cerebellopontine angle (4/23.5%), sphenoid bone (4/23.5%), petrous temporal bone (3/17.6%). Surgical approach was retrosigmoid in 5 patients (29.4%), subtemporal in another 5 (29.4%), subfrontal in 2 (11.7%), pterional in 2 (11.7%). Total resection was achieved in 6/35.3% and subtotal in 11/64.7% patients. Radiotherapy was performed in one child (5.9%) and chemotherapy in 3 (17.6%). Regarding the long-term surgical complications 13/76,4% evolved with long term neurological impairment. Five (29.4%) patients evolved with facial paresis, 5 (29.4%) with hypoacusia, 4 (23.5%) with ocular motricity deficit, 2 with hydrocephalus (11.7%), dysphonia was present in 2 (11.7%), visual impairment in 2 (11.7%), epilepsy in one (5.9%) and dysphagia in one (5.9%). In four children (23.5%) there was no long-term impairment. Residual lesion was stable in 11/64.7% of children, progressing in 3/17.6%. One patient tumor recidivated after total tumor resection. No lesion was found after surgery in 3/17.6%.
The literature review included 267 patients. Mean age was 10.9 years (R:6.8-13.9) with 58.1% males and 41.9% females. Regarding to histopathology, the review found 10.1% JNA, 7.1% CP Craniopharyngioma (CP), 8.6% Schwannoma, 8.2% Sarcoma, 4.1% rhabdomyosarcoma (RMS), 4.9 % Meningioma (MGM), 3.8% Chordoma, other tumors representing 46.8%.

Discussão e Conclusões

Skull base tumors are relatively rare in pediatric age, predominating in boys, with very heterogeneous histopathology and lead to considerable morbidly and mortality in pediatric age.

Referências bibliográficas

Brockmeyer D, Gruber DP, Haller J, Shelton C, Walker ML (2003) Pediatric Skull Base Surgery. Pediatric Neurosurgery 38:9-15. doi:10.1159/000067563
Donald PJ (1998) Surgery of the skull base. Lippincott-Raven Philadelphia,
Gil, Z. , Constantini, S. , Spektor, S. , Abergel, A. , Khafif, A. , Beni‐Adani, L. , Leonor, T. , DeRowe, A. and Fliss, D. M. (2005), Skull base approaches in the pediatric population. Head Neck, 27: 682-689. doi:10.1002/hed.20226
Hanbali F, Tabrizi P, Lang FF, DeMonte F (2004) Tumors of the skull base in children and adolescents. Journal of neurosurgery 100:169-178. doi:10.3171/ped.2004.100.2.0169
Hayhurst C, Williams D, Yousaf J, Richardson D, Pizer B, Mallucci C (2013) Skull base surgery for tumors in children: long-term clinical and functional outcome. Journal of Neurosurgery: Pediatrics 11:496-503
Lang, D., Neil-Dwyer, G., Evans, B. et al. (1998) Craniofacial Access in Children. Acta Neurochir (Wien) 140: 33. https://doi.org/10.1007/s007010050054
Mandonnet E, Kolb F, Tran Ba Huy P, George B (2008) Spectrum of skull base tumors in children and adolescents: a series of 42 patients and review of the literature. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 24:699-706. doi:10.1007/s00381-008-0580-1
Rosemberg S, Fujiwara D (2005) Epidemiology of pediatric tumors of the nervous system according to the WHO 2000 classification: a report of 1,195 cases from a single institution. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery 21:940-944. doi:10.1007/s00381-005-1181-x
Teo C, Dornhoffer J, Hanna E, Bower C (1999) Application of skull base techniques to pediatric neurosurgery. Child's Nervous System 15:103-109. doi:10.1007/s003810050343

Palavras Chaves

Skull Base, Tumor, Surgery, Children

Área

Neurocirurgia Pediátrica