Diagnostic and Therapeutic Strategy in Anaplastic (Malignant) Meningioma, CNS WHO Grade 3
<i>Background</i>: 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 presen...
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MDPI AG
2022-09-01
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| Series: | Cancers |
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| Online Access: | https://www.mdpi.com/2072-6694/14/19/4689 |
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| author | Vincenzo Di Nunno Caterina Giannini Sofia Asioli Alfredo Conti Julia Furtner Damiano Balestrini Alicia Tosoni |
| author_facet | Vincenzo Di Nunno Caterina Giannini Sofia Asioli Alfredo Conti Julia Furtner Damiano Balestrini Alicia Tosoni |
| author_sort | Vincenzo Di Nunno |
| collection | DOAJ |
| description | <i>Background</i>: 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. <i>Methods</i>: 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. <i>Results</i>: 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. <i>Conclusions</i>: 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. |
| first_indexed | 2024-03-09T21:58:04Z |
| format | Article |
| id | doaj.art-d00da6886da74758bc4b42bc545ef3a5 |
| institution | Directory Open Access Journal |
| issn | 2072-6694 |
| language | English |
| last_indexed | 2024-03-09T21:58:04Z |
| publishDate | 2022-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Cancers |
| spelling | doaj.art-d00da6886da74758bc4b42bc545ef3a52023-11-23T19:54:59ZengMDPI AGCancers2072-66942022-09-011419468910.3390/cancers14194689Diagnostic and Therapeutic Strategy in Anaplastic (Malignant) Meningioma, CNS WHO Grade 3Vincenzo Di Nunno0Caterina Giannini1Sofia Asioli2Alfredo Conti3Julia Furtner4Damiano Balestrini5Alicia Tosoni6Department of Oncology, AUSL Bologna, 40139 Bologna, ItalyDepartment of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN 59005, USAPituitary Unit, IRCCS Istituto delle Scienze Neurologiche di Bologna, 49139 Bologna, ItalyDipartimento di Scienze Biomediche e Neuromotorie (DIBINEM), IRCCS Istituto delle Scienze Neurologiche di Bologna, University of Bologna, 40139 Bologna, ItalyDepartment of Biomedical Imaging and Image-Guided Therapy, Medical University of Vienna, 1090 Vienna, AustriaRadiotherapy Department, AUSL-IRCCS Scienze Neurologiche, 40139 Bologna, ItalyUOC Oncologia Sistema Nervoso, IRCCS Istituto delle Scienze Neurologiche di Bologna, 40139 Bologna, Italy<i>Background</i>: 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. <i>Methods</i>: 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. <i>Results</i>: 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. <i>Conclusions</i>: 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.https://www.mdpi.com/2072-6694/14/19/4689meningiomaanaplastic meningiomaNF2 |
| spellingShingle | Vincenzo Di Nunno Caterina Giannini Sofia Asioli Alfredo Conti Julia Furtner Damiano Balestrini Alicia Tosoni Diagnostic and Therapeutic Strategy in Anaplastic (Malignant) Meningioma, CNS WHO Grade 3 Cancers meningioma anaplastic meningioma NF2 |
| title | Diagnostic and Therapeutic Strategy in Anaplastic (Malignant) Meningioma, CNS WHO Grade 3 |
| title_full | Diagnostic and Therapeutic Strategy in Anaplastic (Malignant) Meningioma, CNS WHO Grade 3 |
| title_fullStr | Diagnostic and Therapeutic Strategy in Anaplastic (Malignant) Meningioma, CNS WHO Grade 3 |
| title_full_unstemmed | Diagnostic and Therapeutic Strategy in Anaplastic (Malignant) Meningioma, CNS WHO Grade 3 |
| title_short | Diagnostic and Therapeutic Strategy in Anaplastic (Malignant) Meningioma, CNS WHO Grade 3 |
| title_sort | diagnostic and therapeutic strategy in anaplastic malignant meningioma cns who grade 3 |
| topic | meningioma anaplastic meningioma NF2 |
| url | https://www.mdpi.com/2072-6694/14/19/4689 |
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