Interdisziplinäre Behandlung von Raumforderungen in Assoziation zu peripheren Nerven: Tumore und tumorähnliche Läsionen

Zusammenfassung

Tumuröse Läsionen oder tumorähnliche Läsionen peripherer Nerven sind insgesamt selten, heterogen und herausfordernd in Diagnose und Therapie. Sie können durch tastbare Schwellungen in Nähe zu Nerven, sensible und/oder motorische Defizite, Berührungsschmerz oder neuropathischen Schmerz auffällig werden. In 91% der Fälle sind Tumore gutartig. Vor diesem Hintergrund sind eine Differenzierung der Entitäten, ihrer Charakteristika und eine funktionserhaltene Resektionsstrategie hochgradig relevant. Fehldiagnosen können gravierende Effekte für die Patienten mit schweren neurologischen Schäden und Schmerzsyndromen haben. Gutartige Tumore sind Schwannome und Neurofibrome, die sporadisch auftreten können aber auch bei Häufung mit neurogenetischen Tumordispositionssyndromen vergesellschaftet sein können. Seltenere gutartige Nerventumore sind Perineuriome, Lipome, die aggressive Fibrose (Desmoidtumor), Paragangliome und Hämangiome. Als tumorähnliche Läsionen werden die Ganglienzyste beschrieben. Die Assoziation von Nerventumoren zu neurogenetischen Syndromen und eine richtige Zuordnung von möglicherweise malignen Läsionen, wie dem MPNST (Maligne Periphere Nervenscheidentumor) oder den Zwischenstufen wie z. B. den ANNUBPs (atypische neurofibromatöse Neoplasien mit unbekanntem biologischem Potential) stellen besondere Herausforderungen dar. Die Interdisziplinarität hat eine hohe Relevanz für die klinische Behandlung und korrekte Diagnosestellung. Das Ziel unserer Arbeit ist die Übersicht über die relevanten Entitäten, die Diagnostik und zeitgemäße Therapiestrategien anhand der aktuellen Datenlage und unter Berücksichtigung der kürzlich erschienen interdisziplinären AWMF S2K-Leitlinie „Diagnostik und Therapie peripherer Nerventumore“.

Abstract

Tumorous or tumour-like lesions of peripheral nerves are generally rare, heterogeneous and challenging to diagnose and treat. They may become apparent by a palpable swelling (lump) near nerves, sensory and/or motor deficits, pain to touch or neuropathic pain. In 91% of cases, tumours are benign. The differentiation of entities and their characteristics as well as a function-preserving resection strategy are highly relevant. Misdiagnosis and inadequate treatment can lead to severe deficits and pain syndromes. Benign tumours include schwannomas and neurofibromas, which can occur sporadically but can also be associated with neurogenetic tumour disposition syndromes if they occur more frequently. Rarer benign nerve tumours include perineuriomas, lipomas, aggressive fibrosis (desmoid tumours), paragangliomas and haemangiomas. Ganglion cysts are described as tumour-like lesions. The association of nerve tumours with neurogenetic syndromes and the correct classification of potentially malignant lesions such as MPNST (malignant peripheral nerve sheath tumour) or intermediate stages such as ANNUBPs (atypical neurofibromatous neoplasms with unknown biological potential) pose particular challenges. Interdisciplinarity is highly relevant for clinical treatment and a correct diagnosis. The aim of our work is to provide an overview of the relevant entities, diagnostic evaluation and contemporary treatment strategies based on the current data situation and taking into account the recently published interdisciplinary AWMF S2k guideline “Diagnosis and Treatment of Peripheral Nerve Tumours”.

Schlüsselwörter

Tumor, Pathologie - Schwannom - Neurofibrom - Perineuriom - Ganglionzyste - Maligner Peripherer Nervenscheidentumor - Neurofibromatose - Paragangliom - Aggressive Fibromatose

Key words

tumour - schwannoma - neurofibroma - perineurioma - ganglion cyst - malignant peripheral nerve sheath tumour - neurofibromatosis - paraganglioma - aggressive fibromatosis

Publication History

Received: 27 November 2023

Accepted: 21 December 2023

Article published online:
20 March 2024

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