Brachial plexus peripheral nerve sheath tumors (PNSTs): clinical and surgical management in the pediatric population.

Autor: Guedes F; Department of Surgery, School of Medicine, Division of Neurosurgery, Gaffrée and Guinle University Hospital, Federal University of Rio de Janeiro State (UNIRIO), 775 Mariz E Barros Street, Rio de Janeiro, RJ, Brazil. neuroguedes@yahoo.com.br., Llorian E; Department of Surgery, School of Medicine, Division of Neurosurgery, Gaffrée and Guinle University Hospital, Federal University of Rio de Janeiro State (UNIRIO), 775 Mariz E Barros Street, Rio de Janeiro, RJ, Brazil., Henriques VM; Department of Surgery, School of Medicine, Division of Neurosurgery, Gaffrée and Guinle University Hospital, Federal University of Rio de Janeiro State (UNIRIO), 775 Mariz E Barros Street, Rio de Janeiro, RJ, Brazil., Torrão-Junior FJL; Department of Surgery, School of Medicine, Division of Neurosurgery, Gaffrée and Guinle University Hospital, Federal University of Rio de Janeiro State (UNIRIO), 775 Mariz E Barros Street, Rio de Janeiro, RJ, Brazil.
Jazyk: angličtina
Zdroj: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery [Childs Nerv Syst] 2024 Nov; Vol. 40 (11), pp. 3789-3800. Date of Electronic Publication: 2024 Jun 28.
DOI: 10.1007/s00381-024-06509-2
Abstrakt: Purpose: Peripheral nerve sheath tumors (PNSTs) are rare in pediatric patients, especially in the brachial plexus. Research on PNSTs is lacking. This article presents a retrospective cohort study of pediatric patients diagnosed and treated with PNSTs, specifically brachial plexus tumors.
Methods: All pediatric patients intervened in a single center between 2007 and 2023 with brachial plexus tumors were systemically analyzed.
Results: Eleven pediatric patients with 14 brachial plexus PNSTs were studied. The gender distribution was 64% female and 36% male, with an average age of 10.7 years. Ninety-one percent had a previous NF-1 diagnosis. Right brachial plexus presented a higher prevalence (64%). Pain, Tinel's sign, and stiffness masses were common during diagnosis. Motor deficits were noted in 43% of the patients. Surgery was indicated for symptoms, particularly pain and rapid growth, increasing malignancy risk. Due to suspected malignancy, an en bloc resection with safety margins was performed. Among the patients, 57% received a histopathological diagnosis of MPNST (malignant peripheral nerve sheath tumor). Treatment included radiotherapy and chemotherapy. Clinical follow-up was conducted for all cases, involving clinical and oncological evaluations for all MPNSTs.
Conclusions: This article present a series of pediatric brachial plexus tumors, especially in NF-1, and emphasizes the importance of thorough evaluation for this group. Swift diagnosis is crucial in pediatrics, enabling successful surgery for small lesions with limited neurological symptoms, improving long-term outcomes. Prompt referral to specialized services is urged for suspected masses, irrespective of neurological symptoms. Benign tumor postsurgical progression shows better outcomes than MPNSTs, with complete resection as the primary goal. Needle-guided biopsy is not recommended.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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