Multiple meningiomas: Epidemiology, management, and outcomes

Abstract

Meningiomas are the most common nonmalignant brain tumor in adults, with an increasing incidence of asymptomatic meningiomas diagnosed on more ubiquitous neuroimaging. A subset of meningioma patients bear 2 or more spatially separated synchronous or metachronous tumors termed “multiple meningiomas” (MM), reported to occur in only 1%–10% of patients, though recent data indicate higher incidence. MM constitute a distinct clinical entity, with unique etiologies including sporadic, familial and radiation-induced, and pose special management challenges. While the pathophysiology of MM is not established, theories include independent origin in disparate locations through unique genetic events, and the “monoclonal hypothesis” of a transformed neoplastic clone with subarachnoid seeding precipitating numerous distinct meningiomas. Patients with solitary meningiomas carry the risk of long-term neurological morbidity and mortality, as well as impaired health-related quality of life, despite being a generally benign and surgically curable tumor. For patients with MM, the situation is even less favorable. MM should be regarded as a chronic disease, and in many cases, the management goal is disease control, as cure is seldom possible. Multiple interventions and lifelong surveillance are sometimes necessary. We aim to review the MM literature and create a comprehensive overview, including an evidence-based management paradigm.

An Uncommon Coexistence of Dural and Intraventricular Meningiomas

“…Although the pathophysiology of multiple meningiomas is unknown, suggestions include independent development in different sites through distinct genetic events and the "monoclonal hypothesis," wherein many distinct meningiomas are precipitated by a modified neoplastic clone with subarachnoid seeding [5].…”

Advances in Mass Spectrometry of Gangliosides Expressed in Brain Cancers

“…The types of biomarkers analyzed were genetic, proteomic and glycomic. Meningiomas occur either spontaneously, in patients who suffer from neurofibromatosis type 2 (NF2), Li-Fraumeni (TP53/CHEK2), Turcot, Gardener, von Hippel-Lindau (VHL), Cowden (PTEN), Gorlin (PTCH1, SUFU), Werner (LMNA) or multiple endocrine neoplasia type I (MEN1) sindroms [75,79]. The most frequent gene mutation was found in NF2 located in chromosome 22q.…”

“…Moreover, a loss of heterozygosity (LOH) [77,78,[80][81][82] was found in this chromosome. Some genetic alterations discovered in higher grade meningioma are deletions on 1p, 6q, 10q, 14q, 9p (CDKNA, p14 ARF , CDKN2B) and 18q chromosomes and gains on 1q, 9q, 12q, 15q, 17q, and 20q [77][78][79][80]82]. Several genes associated with oncogenesis of meningioma are: TRAF7, ATK1, KLF4, SMO, PIK3CA, BAP1, POLR2A, SMARCB1, AKT1E17K, hTERT/telomerase, MADH2, MADH4, APM-1, DCC, CDKN2A, p14 ARF , CDKN2B, TP53, MEG3, ALPL, Notch, WNT, IGF, NDRG2, TERT, H3K27me3, Cx43, SMARCE1, AKP12, ARID4B, DNA methylation and loss of heterozygosity of DAL1 [76,77,[79][80][81][82][83][84][85][86][87].…”

Comprehensive genomic analysis of sporadic multiple meningiomas reveals clonal origin and histotype-specific evolution: a case report

“…In patients with multiple meningiomas, clinical decisions regarding the lesions that should be surgically resected and the order of their resection are often di cult. It is a chronic disease that requires repeated interventions and lifelong surveillance to achieve disease control [4]. In terms of prognosis, patients with multiple meningiomas exhibit shorter overall survival, progression-free survival, and time to second intervention than patients with a solitary meningioma [3,5].…”