Abstract
BackgroundPlexiform neurofibromas (PNF) are benign tumors of the peripheral nerve which mostly develop in patients with neurofibromatosis type 1 (NF1). Surgical interventions are usually not applied to children with small tumors. These are rather restricted to debulking of larger tumors in adults that cause clinical complications or aesthetic disfigurement. In most cases, a total resection of PNF is not possible due to the network-like growth of the tumors.Patients and methodsEarly surgical intervention was carried out for 9 small PNFs in 7 NF1 children. Tumor resection was performed following the graphical delineation of the affected skin and according the MRI findings.ResultsTotal resection was achieved for all 9 PNF without causing any neurological or organic deficit. Annual magnetic resonance tomography over a period of four years did not reveal any relapse of the tumors.ConclusionsEarly surgical intervention for small superficial PNFs in NF1 children have various advantages and may especially be considered a strategy to prevent progression.
Highlights
Plexiform neurofibromas (PNF) are benign tumors of the peripheral nerve which mostly develop in patients with neurofibromatosis type 1 (NF1)
Total resection was achieved for all 9 PNF without causing any neurological or organic deficit
Plexiform neurofibromas (PNF) are benign tumors originating from nerve sheath cells, subcutaneous, or visceral peripheral nerves and can involve multiple fascicles [1]
Summary
Plexiform neurofibromas (PNF) are benign tumors of the peripheral nerve which mostly develop in patients with neurofibromatosis type 1 (NF1). Surgical interventions are usually not applied to children with small tumors. These are rather restricted to debulking of larger tumors in adults that cause clinical complications or aesthetic disfigurement. Plexiform neurofibromas (PNF) are benign tumors originating from nerve sheath cells, subcutaneous, or visceral peripheral nerves and can involve multiple fascicles [1]. PNF can arise in various parts of the body, for example as anterior mediastinal masses, sciatic nerve lesions with pelvic extension, or perirectal plexiform and uterine tumors, often leading to severe clinical complications [7]. Due to large size of the tumors and involvement of multiple fascicles of nerves and tissues, the risk of neurological and functional destruction upon tumor resection is high
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