A chance to cut is a chance to cure: complete resection of an atypical neurofibroma prevents further progression to malignancy.

Autor: Vaassen P; Department of Pediatrics, Sana Kliniken, Duisburg, Germany. pia.vaassendr@sana.de., Feldkamp A; Department of Pediatrics, Sana Kliniken, Duisburg, Germany., Scholz M; Department of Neurosurgery, Sana Kliniken, Duisburg, Germany., Blau T; Department of Neuropathology, Universitätsklinikum, Essen, Germany., Dürr NR; Department of Radiology and Neuroradiology, Sana Kliniken, Duisburg, Germany., Rosenbaum T; Department of Pediatrics, Sana Kliniken, Duisburg, Germany.
Jazyk: angličtina
Zdroj: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery [Childs Nerv Syst] 2023 Nov; Vol. 39 (11), pp. 3301-3304. Date of Electronic Publication: 2023 Jun 21.
DOI: 10.1007/s00381-023-06029-5
Abstrakt: Plexiform neurofibromas are the hallmark of neurofibromatosis type 1 (NF1) and significantly contribute to the overall burden of disease. While surgical excision has long been the only available therapy, the MEK inhibitor (MEKi) selumetinib has been approved as a non-surgical treatment option for these tumors in 2020 (USA) and 2021 (Europe), respectively. However, selumetinib will result in tumor shrinkage only after several months of therapy and might not prevent malignant transformation of a plexiform neurofibroma that occurs with a frequency of 10-15%. Here, we demonstrate that surgical excision might be the therapy of choice in some plexiform neurofibromas despite the availability of MEKi therapy.
(© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
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