Journal Article

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

Torrão-Junior, Francisco José Lourenço, Llorian, Evelina, Guedes, Fernando, Henriques, Vinícius M.

2024

Overview

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.

• Publisher: Springer Berlin Heidelberg,Springer Nature B.V
• Subject: Adolescent / Brachial Plexus - pathology / Brachial Plexus - surgery / Child / Child, Preschool / Cohort Studies / Female / Humans / Male / Medicine / Medicine & Public Health / Nerve Sheath Neoplasms - surgery / Neurosciences / Neurosurgery / Neurosurgical Procedures - methods / Peripheral Nervous System Neoplasms - pathology / Peripheral Nervous System Neoplasms - surgery / Retrospective Studies