Intradurale, extramedulläre Tumore: Epidemiologie, Diagnostik und Therapie

Zusammenfassung

Intradurale, extramedulläre (IDEM) Tumore treten mit einer Inzidenz von 0,97/100 000 Personen pro Jahr auf. Die Kontrastmittel-gestützte Magnetresonanztomografie stellt den Goldstandard in der präoperativen Diagnostik und Nachsorge dar. Das klinische Beschwerdebild der Patienten richtet sich nach der Lokalisation des Befundes. Einschränkungen der Sensorik, Motorik, Propriozeption und des Vegetativum sind häufige Symptome ebenso wie Schmerzen. Schwannome, Meningeome und myxopapilläre Ependymome gehören zu den häufigsten, benignen IDEM-Tumorentitäten. Maligne IDEM-Tumore sind weitaus seltener. Differenzialdiagnostisch kann es sich hierbei um Abtropfmetastasen eines Hirntumors, eine Meningeosis carcinomatosa oder um einen malignen peripheren Nervenscheidentumor handeln. Die mikrochirurgische Resektion stellt die Therapie der Wahl dar bei der Behandlung benigner Läsionen. Mit einer vollständigen Resektion des Tumors kann eine sehr gute lokale Kontrolle erzielt werden. Im Fall von Resttumorgewebe oder einem Rezidiv kann eine stereotaktische Bestrahlung notwendig bzw. sinnvoll sein. Eine Systemtherapie spielt bei der Behandlung von IDEM-Tumoren eine nachrangige Rolle. Bei malignen Prozessen richtet sich das therapeutische Vorgehen nach der zugrundeliegenden Erkrankung. Anforderungen an die genannten Therapieverfahren sind es zum Funktionserhalt beizutragen, Symptome zu lindern und eine größtmögliche Tumorkontrolle zu erzielen.

Abstract

Intradural extramedullary (IDEM) tumours occur with an incidence of 0.97/100,000 individuals per year. Contrast-enhanced magnetic resonance imaging is the gold standard in preoperative diagnostics and post-operative follow up. Clinical symptoms of the patients depend on the localization of the tumour. Restrictions of sensory function, motor function, proprioception and the vegetative function are common symptoms as well as pain. Schwannomas, meningiomas and myxopapillary ependymomas are the most common benign IDEM tumour entities. Malignant IDEM tumours are rare (i. e. drip metastases of a brain tumour, meningeosis neoplastica or malignant peripheral nerve sheath tumour). Microsurgical resection is the therapy of choice for the treatment of benign lesions. The best local control can be achieved with a complete resection of the tumour. In the case of residual tumour tissue or a relapse, stereotactic radiation can be indicated. Systemic therapies play a subordinate role in the treatment of IDEM tumours. In malignant processes, therapeutic options depend on the underlying disease. The goals of the above-mentioned therapeutic strategies are to contribute to maintaining function, alleviating symptoms and achieving the greatest possible tumour control.

Schlüsselwörter

spinale Meningeome - myxopapilläre Epenymome - intradurale Neurinome - Mikrochirurgie - Radiochirurgie - intradurale, extramedulläre Tumore - Meningeom - Schwannom - Ependymom

Keywords

spinale meningeoma - myxopapillary ependymoma - intradural neurinoma - microsurgery - radiosurgery

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