Diagnostic and Therapeutic Strategy in Anaplastic (Malignant) Meningioma, CNS WHO Grade 3

Background: Meningiomas are the most common primary central nervous system malignancies accounting for 36% of all intracranial tumors. However, only 1% of meningioma is classified as malignant (anaplastic) meningioma. Due to their rarity, clinical management of these tumors presents several gaps. Me...

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Published inCancers Vol. 14; no. 19; p. 4689
Main Authors Di Nunno, Vincenzo, Giannini, Caterina, Asioli, Sofia, Conti, Alfredo, Furtner, Julia, Balestrini, Damiano, Tosoni, Alicia
Format Journal Article
LanguageEnglish
Published Basel MDPI AG 26.09.2022
MDPI
Subjects
anaplastic meningioma
Brain cancer
Care and treatment
Central nervous system
Clinical trials
Copy number
Cyclin-dependent kinases
Diagnosis
Kinases
Management
Medical prognosis
Meningioma
Metastasis
Mutation
Neurofibromatosis
Neurofibromatosis 2
Neurofibromin 2
NF2
Review
Stains & staining
Surgery
Tumors
Italy
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Summary:Background: Meningiomas are the most common primary central nervous system malignancies accounting for 36% of all intracranial tumors. However, only 1% of meningioma is classified as malignant (anaplastic) meningioma. Due to their rarity, clinical management of these tumors presents several gaps. Methods: We carried out a narrative review aimed to investigate current knowledge of anaplastic meningioma focusing on their pathological and radiological diagnosis, molecular assessment, and loco-regional and systemic management. Results: The most frequent genetic alteration occurring in meningioma is the inactivation in the neurofibromatosis 2 genes (merlin). The accumulation of copy number losses, including 1p, 6p/q, 10q, 14q, and 18p/q, and less frequently 2p/q, 3p, 4p/q, 7p, 8p/q, and 9p, compatible with instability, is restricted to NF2 mutated meningioma. Surgery and different RT approaches represent the milestone of grade 3 meningioma management, while there is a marginal role of systemic therapy. Conclusions: Anaplastic meningiomas are rare tumors, and diagnosis should be suspected and confirmed by trained radiologists and pathologists. Despite the current marginal role of systemic therapy, it is possible that the increasing knowledge of molecular altered pathways of the disease will lead to the development of novel effective systemic treatments.
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ISSN:2072-6694
2072-6694
DOI:10.3390/cancers14194689