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72. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC)
Joint Meeting mit der Polnischen Gesellschaft für Neurochirurgie

Deutsche Gesellschaft für Neurochirurgie (DGNC) e. V.

06.06. - 09.06.2021

Surgical management of peripheral nerve sheath tumours in all age-groups with neurofibromatosis

Chirurgisches Management von peripheren Nervenscheidentumoren in Patienten aller Altersgruppen mit Neurofibromatose

Meeting Abstract

  • Julian Zipfel - Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Tübingen, Deutschland
  • presenting/speaker Meizer Al Hariri - Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Tübingen, Deutschland
  • Karin Haas-Lude - Universitätsklinikum Tübingen, Kinderklinik, Tübingen, Deutschland
  • Isabel Gugel - Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Tübingen, Deutschland
  • Martin Ulrich Schuhmann - Universitätsklinikum Tübingen, Klinik für Neurochirurgie, Tübingen, Deutschland

Deutsche Gesellschaft für Neurochirurgie. 72. Jahrestagung der Deutschen Gesellschaft für Neurochirurgie (DGNC), Joint Meeting mit der Polnischen Gesellschaft für Neurochirurgie. sine loco [digital], 06.-09.06.2021. Düsseldorf: German Medical Science GMS Publishing House; 2021. DocP027

doi: 10.3205/21dgnc315, urn:nbn:de:0183-21dgnc3151

Published: June 4, 2021

© 2021 Zipfel et al.
This is an Open Access article distributed under the terms of the Creative Commons Attribution 4.0 License. See license information at http://creativecommons.org/licenses/by/4.0/.


Outline

Text

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.

Figure 1 [Fig. 1], Figure 2 [Fig. 2]