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An unusual finding of an anaplastic meningioma in NF2-related schwannomatosis.
- Source :
-
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. - Publication Year :
- 2024
-
Abstract
- 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.<br /> (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)
- Subjects :
- Humans
Male
Child
Neurofibromatosis 2 complications
Neurofibromatosis 2 surgery
Neurofibromatosis 2 diagnostic imaging
Neurilemmoma surgery
Neurilemmoma complications
Neurilemmoma diagnostic imaging
Neurilemmoma pathology
Skin Neoplasms surgery
Skin Neoplasms pathology
Skin Neoplasms complications
Magnetic Resonance Imaging
Meningioma surgery
Meningioma complications
Meningioma diagnostic imaging
Meningioma pathology
Meningeal Neoplasms surgery
Meningeal Neoplasms complications
Meningeal Neoplasms diagnostic imaging
Meningeal Neoplasms pathology
Neurofibromatoses complications
Neurofibromatoses surgery
Neurofibromatoses diagnostic imaging
Subjects
Details
- Language :
- English
- ISSN :
- 1433-0350
- Volume :
- 40
- Issue :
- 7
- Database :
- MEDLINE
- Journal :
- Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery
- Publication Type :
- Academic Journal
- Accession number :
- 38451298
- Full Text :
- https://doi.org/10.1007/s00381-024-06350-7