An unusual finding of an anaplastic meningioma in NF2-related schwannomatosis.

Autor: Adelhoefer SJ; Division of Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA. sadelho1@jh.edu.; Faculty of Medicine, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany. sadelho1@jh.edu., Feghali J; Division of Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA., Rajan S; Division of Neuropathology, Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA., Eberhart CG; Division of Neuropathology, Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA., Staedtke V; Comprehensive Neurofibromatosis Center, Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA., Cohen AR; Division of Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, MD, 21205, USA.
Jazyk: angličtina
Zdroj: Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery [Childs Nerv Syst] 2024 Jul; Vol. 40 (7), pp. 2209-2214. Date of Electronic Publication: 2024 Mar 07.
DOI: 10.1007/s00381-024-06350-7
Abstrakt: NF2-related schwannomatosis (NF2) is a rare autosomal-dominant genetic disorder characterized by bilateral vestibular schwannomas and multiple meningiomas. This case report presents the extremely rare occurrence of an anaplastic meningioma in a 12-year-old male with previously undiagnosed NF2. The patient presented with a history of abdominal pain and episodic emesis, gait unsteadiness, right upper and lower extremity weakness, and facial weakness. He had sensorineural hearing loss and wore bilateral hearing aids. MR imaging revealed a sizable left frontoparietal, dural-based meningioma with heterogeneous enhancement with mass effect on the brain and midline shift. Multiple additional CNS lesions were noted including a homogenous lesion at the level of T5 indicative of compression of the spinal cord. The patient underwent a frontotemporoparietal craniotomy for the removal of his large dural-based meningioma, utilizing neuronavigation and transdural ultrasonography for precise en bloc resection of the mass. Histopathology revealed an anaplastic meningioma, WHO grade 3, characterized by brisk mitotic activity, small-cell changes, high Ki-67 proliferation rate, and significant loss of P16. We report an anaplastic meningioma associated with an underlying diagnosis of NF2 for which we describe clinical and histopathological features.
(© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
Databáze: MEDLINE
Externí odkaz: