Artikel
Surgical management of peripheral nerve sheath tumours in all age-groups with neurofibromatosis
Chirurgisches Management von peripheren Nervenscheidentumoren in Patienten aller Altersgruppen mit Neurofibromatose
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Veröffentlicht: | 4. Juni 2021 |
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Objective: Peripheral nerve sheath tumours are a heterogenous group, consisting mostly of benign tumours as well as malignant neoplasms. Especially in the setting of neurofibromatosis, diagnostics and indication for therapy pose relevant challenges for neurosurgeons.
Methods: We retrospectively reviewed all cases treated at our department between 2006 and 2017 for peripheral nerve sheath tumours. We analysed clinical signs, symptoms, histology, and sensory / motor function in the cohort with phacomatosis.
Results: Of 414 identified patients, the majority had sporadic tumours (n=169, 40.8%). 151 patients (36.5%) had NF1, 54 NF2 (13.0%), and 33 schwannomatosis (8.0%). 7 patients had other syndromes (1.7%) which? A total of 772 surgical interventions were performed and 1132 tumours removed. In 238 patients with phacomatosis, 584 surgeries were performed for.
Indication for surgery was in most instances pain (50.9%), followed by significant tumour growth (22.8%). New deficits led to surgery in 21.9% of interventions, malignancy was suspected in 4.5%. Histopathology revealed mostly schwannomas (48.4%), followed by neurofibromas (46.9%). This group could be divided into cutaneous neurofibromas (6.5%), infiltrating plexiform neurofibromas (8.5%) and peripheral nerve born neurofibromas (31.9%). 2.5% of tumors were MPNST, we found 3 ganglioneuromas, 8 hybrid-neurofibromas and 1 perineurinoma. Leading symptoms, such as pain, motor and sensory deficits improved after 641/772 interventions (83.0%), remained unchanged following 96 interventions (12.4%) and worsened in 35 occasions (4.5%).
Conclusion: Surgical outcome is in general favourable. Surgery is safe and effective for (phacomatosis associated) peripheral nerve sheath tumours, however management needs a multidisciplinary setting. We propose early surgical resection in patients with peripheral nerve sheath tumours with significant growth, or pain, or motor deficit, or suspected malignancy.