CC BY-NC-ND 4.0 · Arquivos Brasileiros de Neurocirurgia: Brazilian Neurosurgery 2018; 37(S 01): S1-S332
DOI: 10.1055/s-0038-1672722
E-Poster – Peripheral Nerve
Thieme Revinter Publicações Ltda Rio de Janeiro, Brazil

A Surgical Series of Pediatrics Peripheral Nerve Tumors: Clinical Results and Lessons Learned

Rosana Siqueira Brown
1   Unirio
,
Stephanie Oliveira Fernandes de Bulhões
1   Unirio
,
Rogério Martin Pires de Amorim
1   Unirio
,
José Fernando Guedes-Corrêa
1   Unirio
› Author Affiliations
Further Information

Publication History

Publication Date:
06 September 2018 (online)

 

Introduction: Peripheral nerve sheath tumors (PNST) are relatively rare pathologies, especially at young age. PNST in children are frequently related to Neurofibromatosis type 1 (NF1). The odds of malignant transformation and metastasis also increases with NF1, demanding sometimes immediate treatment. Previous studies suggest that palpable/visible mass, palsy nerve and pain are more common in malignant lesions than benign.

Objectives: The aim of this study is to is to present a clinical series of eight patients, with and without NF1, with rare tumors affecting peripheral nerves.

Methodology: This is a retrospective study of eight children, with clinical data retrieved from medical records of our division in the period of 2007–2017. Surgical management for benign PNSTs was performed with total lesion resection in most of the cases, while the surgical approach for malignant PNSTs was attempted to achieve gross total or full resection.

Results: All patients presented intense pain and a palpable mass in the tumor area and two of them demonstrated a neurological deficit, that was progressively worsened. The excision was total in four patients (50%) of the patients and gross total in four patients (50%). In four patients (50%), it was found a benign PNST through histopathologic diagnosis (three neurofibroma and one desmoid tumor) after surgical procedure, while three patients (37.5%) had malignant PNST and one (12,5%) had Ewing sarcoma (table 1). In the patients diagnosed with NF1, three (50%) of them had neurofibroma, two (25%) had malignant PNST and one (12.5%) Ewing Sarcoma.

Conclusions: We conclude that progressively growth of a mass in the area of peripheral nerve in children, with pain that increases steadily, at rest or at night, with or without NF1, need to be targeted to histological diagnosis and surgical resection. Mainly patients with NF1, since its percentages of malignization are higher and the risks of metastasis also increases.