title: Meningiomas With Rhabdoid Features Lacking Other Histologic Features of Malignancy: A Study of 44 Cases and Review of the Literature.
abstract: The behavior of rhabdoid meningiomas otherwise lacking malignant features remains unknown as most of the originally reported aggressive cases showed anaplastic histologic features independently of rhabdoid phenotype. We studied 44 patients with rhabdoid meningiomas lacking anaplastic features. Median age at diagnosis was 48.6 years (range 10-79). Location was supratentorial in 28 (63.6%), skull base in 15 (34.1%), and spinal in 1 (2.3%). Tumor grade was otherwise World Health Organization grade I (n = 22, 50%) or II (n = 22, 50%). Rhabdoid cells represented <20% of the tumor in 12 cases (27.3%), 20% to 50% in 18 (40.9%), and >50% in 14 (31.8%). Median clinical follow-up, available for 38 patients, was 5.0 years (range 0.17-14.2). Recurrence occurred in 9 patients (5-year recurrence-free survival, 73.7%) with a significantly higher risk in subtotally resected tumors (p = 0.043). Rhabdoid cell percentage was not associated with recurrence. Six patients died (4 of disease, 2 of unclear causes); 5-year overall survival was 86.7%, a mortality in excess of that expected in grade I-II meningiomas but much lower than originally reported. Review of 50 similar previously reported cases confirmed our findings. We suggest that rhabdoid meningiomas be graded analogously to nonrhabdoid tumors, with caution that some may still behave aggressively and close follow-up is recommended.
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Rachael A
authors.initials: RA
authors.lastname: Vaubel
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Selby G
authors.initials: SG
authors.lastname: Chen
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: David R
authors.initials: DR
authors.lastname: Raleigh
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Link
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Chicoine
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Igor
authors.initials: I
authors.lastname: Barani
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Sarah M
authors.initials: SM
authors.lastname: Jenkins
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Patrice Abell
authors.initials: PA
authors.lastname: Aleff
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Fausto J
authors.initials: FJ
authors.lastname: Rodriguez
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Peter C
authors.initials: PC
authors.lastname: Burger
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Sonika
authors.initials: S
authors.lastname: Dahiya
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California.
authors.forename: Arie
authors.initials: A
authors.lastname: Perry
authors.affiliation: From the Department of Anatomic Pathology (RAV, CG), Mayo Clinic, Rochester Minnesota; Department of Neurosurgery (SGC), Mayo Clinic, Jacksonville, Florida; Department of Radiation Oncology (DRR, IB), University of California, San Francisco, California; Department of Neurosurgery (MJL), Mayo Clinic, Rochester Minnesota; Department of Neurologic Surgery (MRC), Washington University, St. Louis, Missouri; Departments of Biomedical Statistics and Informatics (SMJ), Mayo Clinic, Rochester Minnesota, Departments of Biochemistry and Molecular Biology (PAA), Mayo Clinic, Rochester Minnesota; Department of Pathology (FJR, PCB), Johns Hopkins University, Baltimore, Maryland; Departments of Pathology and Immunology/Anatomic and Molecular Pathology (SD), Washington University, St. Louis, Missouri; Department of Pathology (AP), University of California, San Francisco, California. giannini.caterina@mayo.edu.
authors.forename: Caterina
authors.initials: C
authors.lastname: Giannini
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001932:Brain Neoplasms / Q000175:diagnosis* / Q000401:mortality; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008577:Meningeal Neoplasms / Q000175:diagnosis* / Q000401:mortality; D008579:Meningioma / Q000175:diagnosis* / Q000401:mortality; D008875:Middle Aged; D018335:Rhabdoid Tumor / Q000175:diagnosis* / Q000401:mortality; D013125:Spinal Neoplasms / Q000175:diagnosis* / Q000401:mortality; D015996:Survival Rate / Q000639:trends; D055815:Young Adult
keywords: Anaplastic meningioma; Meningioma; Rhabdoid meningioma; WHO grade