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Neuro-oncology Apr 2017Leptomeningeal metastases (LM) currently lack standardization with respect to response assessment. A Response Assessment in Neuro-Oncology (RANO) working group with... (Review)
Review
Leptomeningeal metastases (LM) currently lack standardization with respect to response assessment. A Response Assessment in Neuro-Oncology (RANO) working group with expertise in LM developed a consensus proposal for evaluating patients treated for this disease. Three basic elements in assessing response in LM are proposed: a standardized neurological examination, cerebral spinal fluid (CSF) cytology or flow cytometry, and radiographic evaluation. The group recommends that all patients enrolling in clinical trials undergo CSF analysis (cytology in all cancers; flow cytometry in hematologic cancers), complete contrast-enhanced neuraxis MRI, and in instances of planned intra-CSF therapy, radioisotope CSF flow studies. In conjunction with the RANO Neurological Assessment working group, a standardized instrument was created for assessing the neurological exam in patients with LM. Considering that most lesions in LM are nonmeasurable and that assessment of neuroimaging in LM is subjective, neuroimaging is graded as stable, progressive, or improved using a novel radiological LM response scorecard. Radiographic disease progression in isolation (ie, negative CSF cytology/flow cytometry and stable neurological assessment) would be defined as LM disease progression. The RANO LM working group has proposed a method of response evaluation for patients with LM that will require further testing, validation, and likely refinement with use.
Topics: Clinical Trials as Topic; Disease Progression; Humans; Magnetic Resonance Imaging; Meningeal Neoplasms; Treatment Outcome
PubMed: 28039364
DOI: 10.1093/neuonc/now183 -
Chinese Clinical Oncology Jul 2017Maximal safe resection has long been the cornerstone of treatment for WHO grade I benign meningioma. However, as technology for both imaging and radiation delivery has... (Review)
Review
Maximal safe resection has long been the cornerstone of treatment for WHO grade I benign meningioma. However, as technology for both imaging and radiation delivery has advanced, radiation therapy has played an increasingly important role in the management of patients with WHO grade I meningioma. Radiation therapy, whether delivered as standard fractionated treatment over several weeks, stereotactic radiosurgery over 1 session, or multisession stereotactic radiation therapy, has been shown to provide excellent local control when used as an adjunct to surgery or as primary treatment. Here, we review the indications for radiation therapy for patients with WHO grade I meningioma, as well as the various techniques that have been developed. We also review the toxicities and late effects associated with treatment.
Topics: Adult; Brain Neoplasms; Dose Fractionation, Radiation; Humans; Meningeal Neoplasms; Meningioma; Neoplasm Grading; Radiosurgery
PubMed: 28758408
DOI: 10.21037/cco.2017.06.01 -
Neurologia Medico-chirurgica 2015Meningiomas are the most common intracranial primary neoplasm in adults. Although the spectrum of clinical and molecular genetic issues regarding meningiomas remains... (Review)
Review
Meningiomas are the most common intracranial primary neoplasm in adults. Although the spectrum of clinical and molecular genetic issues regarding meningiomas remains undefined, novel genetic alterations that are associated with tumor morphology, malignancy, or location have recently been discovered. This review focuses on recent advances in understanding of the heterogenous pathology of meningiomas, particularly on associations between the clinical, histological, etiological, epidemiological, and molecular genetical aspects of the neoplasm.
Topics: Humans; Immunohistochemistry; Meningeal Neoplasms; Meningioma; Molecular Biology
PubMed: 25744347
DOI: 10.2176/nmc.ra.2014-0233 -
Neuro-oncology Oct 2017We have recently shown that the breast cancer (BRCA)1-associated protein-1 tumor suppressor gene (BAP1) is inactivated in a subset of clinically aggressive meningiomas... (Review)
Review
We have recently shown that the breast cancer (BRCA)1-associated protein-1 tumor suppressor gene (BAP1) is inactivated in a subset of clinically aggressive meningiomas that display rhabdoid histomorphology. Immunohistochemistry for BAP1 protein provides a rapid and inexpensive method for screening suspected cases. Notably, some patients with BAP1-mutant meningiomas have germline BAP1 mutations and BAP1 tumor predisposition syndrome (TPDS). It appears that nearly all patients with germline BAP1 mutations develop malignancies by age 55, most frequently uveal melanoma, cutaneous melanoma, pleural or peritoneal malignant mesothelioma, or renal cell carcinoma, although other cancers have also been associated with BAP1 TPDS. Therefore, when confronted with a patient with a potentially high-grade rhabdoid meningioma, it is important that neuropathologists assess the BAP1 status of the tumor and that the patient's family history of cancer is carefully ascertained. In the appropriate clinical setting, genetic counseling and germline BAP1 DNA sequencing should be performed. A cancer surveillance program for individuals who carry germline BAP1 mutations may help identify tumors such as uveal melanoma, cutaneous melanoma, and renal cell carcinoma at early and treatable stages. Because BAP1-mutant meningiomas are rare tumors, multi-institutional efforts will be needed to evaluate therapeutic strategies and to further define the clinicopathologic features of these tumors.
Topics: Humans; Meningeal Neoplasms; Meningioma; Mutation; Neoplasm Grading; Patient Care; Tumor Suppressor Proteins; Ubiquitin Thiolesterase
PubMed: 28482042
DOI: 10.1093/neuonc/nox094 -
Journal of Neuro-oncology Jan 2022Meningiomas are generally considered "benign," however, these tumors can demonstrate variability in behavior and a surprising aggressiveness with elevated rates of... (Review)
Review
INTRODUCTION
Meningiomas are generally considered "benign," however, these tumors can demonstrate variability in behavior and a surprising aggressiveness with elevated rates of recurrence. The advancement of next-generation molecular technologies have led to the understanding of the genomic and epigenomic landscape of meningiomas and more recent correlations with clinical characteristics and behavior.
METHODS
Based on a thorough review of recent peer-reviewed publications (PubMed) and edited texts, we provide a molecular overview of meningiomas with a focus on relevant clinical implications.
RESULTS
The identification of specific somatic driver mutations has led to the classification of several major genomic subgroups, which account for more than 80% of sporadic meningiomas, and can be distinguished using noninvasive clinical variables to help guide management decisions. Other somatic genomic modifications, including non-coding alterations and copy number variations, have also been correlated with tumor characteristics. Furthermore, epigenomic modifications in meningiomas have recently been described, with DNA methylation being the most widely studied and potentially most clinically relevant. Based on these molecular insights, several clinical trials are currently underway in an effort to establish effective medical therapeutic options for meningioma.
CONCLUSION
As we enhance our multiomic understanding of meningiomas, our ability to care for patients with these tumors will continue to improve. Further biological insights will lead to additional progress in precision medicine for meningiomas.
Topics: DNA Copy Number Variations; Genomics; Humans; Meningeal Neoplasms; Meningioma
PubMed: 34846640
DOI: 10.1007/s11060-021-03874-9 -
Acta Neuropathologica Communications Apr 2019Meningiomas are the most common primary brain tumor of adults. The majority are benign (WHO grade I), with a mostly indolent course; 20% of them (WHO grade II and III)...
Meningiomas are the most common primary brain tumor of adults. The majority are benign (WHO grade I), with a mostly indolent course; 20% of them (WHO grade II and III) are, however, considered aggressive and require a more complex management. WHO grade II and III tumors are heterogeneous and, in some cases, can develop from a prior lower grade meningioma, although most arise de novo. Mechanisms leading to progression or implicated in de novo grade II and III tumorigenesis are poorly understood. RNA-seq was used to profile the transcriptome of grade I, II, and III meningiomas and to identify genes that may be involved in progression. Bioinformatic analyses showed that grade I meningiomas that progress to a higher grade are molecularly different from those that do not. As such, we identify GREM2, a regulator of the BMP pathway, and the snoRNAs SNORA46 and SNORA48, as being significantly reduced in meningioma progression. Additionally, our study has identified several novel fusion transcripts that are differentially present in meningiomas, with grade I tumors that did not progress presenting more fusion transcripts than all other tumors. Interestingly, our study also points to a difference in the tumor immune microenvironment that correlates with histopathological grade.
Topics: Computational Biology; Disease Progression; Female; Humans; Male; Meningeal Neoplasms; Meningioma; Neoplasm Grading; Neurofibromin 2; RNA-Seq; Transcriptome; Tumor Microenvironment
PubMed: 31039818
DOI: 10.1186/s40478-019-0690-x -
Medicine Mar 2023Meningiomas are mostly benign brain tumors with minimal malignant cases. Anaplastic meningioma has malignant morphological characteristics and a World Health...
RATIONALE
Meningiomas are mostly benign brain tumors with minimal malignant cases. Anaplastic meningioma has malignant morphological characteristics and a World Health Organization grade of III.
PATIENT CONCERNS
The present study reports a case of an occipital meningioma in a patient who initially chose observation and follow-up after diagnosis. The patient ultimately underwent surgery due to the enlargement of the tumor and the development of visual field defects after a decade of imaging follow-up. The postoperative pathology slides indicated the presence of an anaplastic meningioma (World Health Organization-grade III).
DIAGNOSES
The patient's diagnosis was established through cranial magnetic resonance imaging, which revealed an irregular mixed mass in the right occipital region with isointense T1 and hypointense T2 signal, irregular lobulation, and a maximum diameter of approximately 5.4 cm. Heterogenous enhancement was observed in the contrast-enhanced scan.
INTERVENTIONS
The patient opted for surgical intervention to remove the tumor, and the pathology slides of the tumor sample confirmed the diagnosis of anaplastic meningioma. The patient also received radiotherapy (40Gy/15fr).
OUTCOMES
No recurrence was observed during the 9-month follow-up.
LESSONS
This case highlights the potential for low-grade meningiomas to undergo malignant transformation, particularly in the presence of irregular lobulation, peritumoral brain edema, and heterogeneous enhancement on contrast-enhanced scans. Total excision (Simpson grade I) is the preferred treatment option, and long-term imaging follow-up is recommended.
Topics: Humans; Meningioma; Meningeal Neoplasms; Brain Neoplasms; Brain Edema; Magnetic Resonance Imaging; Neoplasm Recurrence, Local; Retrospective Studies
PubMed: 37000075
DOI: 10.1097/MD.0000000000033409 -
Journal of Medicine and Life 2021Meningiomas are common primary tumors of the central nervous system. The incidence at the age of fertility is low, although there are some hormonal mechanisms involved.... (Review)
Review
Meningiomas are common primary tumors of the central nervous system. The incidence at the age of fertility is low, although there are some hormonal mechanisms involved. Growth in size was observed during the luteal phase of the menstrual cycle, which could lead to developing new symptoms during pregnancy or worsening of the already existing ones. Visual impairment is the chief complaint, followed by headache, nausea, vomiting, and seizures. Diagnosis is based on neurological examination, ophthalmoscopy, imaging techniques like gadolinium-enhanced magnetic resonance imaging (MRI), and contrast-enhanced computed tomography (CT) scans, bearing in mind the patient's irradiation and prejudice on the fetus together with the histopathological examination. The objective of the review is to determine the influence of meningioma on pregnancy and vice-versa and provide a strategy of follow-up for maternal-fetal specialists and not only. We performed a systematic review by searching relevant information in PubMed and Wiley databases using keywords as meningioma, pregnancy, progesterone receptors. The results showed that besides a similar incidence of meningioma in pregnant and non-pregnant women, symptoms might flare during pregnancy due to water retention, engorgement of vessels, and the presence of sex hormone receptors on tumor cells. Meningioma may impact the route of pregnancy with adverse effects on the fetus. Thus, fetal monitoring by biophysical profile and cardiotocography (CTG) is needed. The preferred treatment option is surgery, but gestational age and the woman's status must be taken into consideration.
Topics: Female; Fetal Monitoring; Follow-Up Studies; Humans; Meningeal Neoplasms; Meningioma; Pregnancy; Pregnant Women
PubMed: 33767778
DOI: 10.25122/jml-2021-0012 -
Journal of Cancer Research and Clinical... Jul 2023Despite best clinical management, meningioma patients experience tumor recurrence. Efforts have been made to improve the prognostic stratification of meningiomas....
BACKGROUND
Despite best clinical management, meningioma patients experience tumor recurrence. Efforts have been made to improve the prognostic stratification of meningiomas. Recently, a multi-faceted molecular classification suggested that the marker S100 is associated with a favorable outcome, making a further analysis in a larger cohort interesting.
MATERIALS AND METHODS
The immunohistochemical staining for S100 was analyzed in 1669 paraffin-embedded meningioma samples. The distribution and association with clinical data and progression-free survival via radiographic tumor recurrence were assessed.
RESULTS
Of 1669 cases, 218 tumors showed strong S100 expression (13.1%). A significantly higher frequency of S100 positive meningiomas was observed in meningiomas of female patients, tumors with spinal and convexity/falx location, primary tumor surgery, NF2, higher extent of resection, lower WHO CNS grade, adjuvant radiotherapy and recurrence-free tumors during follow-up. Univariate analysis revealed a favorable progression-free survival for meningiomas with S100 expression (p = 0.0059) but not in the multivariate analysis. Higher S100 frequency was independently associated with female gender (p = 0.0003), NF2 (p < 0.0001), tumor location (p < 0.0001) and lower WHO CNS grade (p = 0.0133).
CONCLUSIONS
The positive prognostic impact of S100 is mostly attributed to the confounding clinical factors gender, tumor location, NF2 status and WHO CNS grade.
Topics: Humans; Female; Meningioma; Prognosis; Meningeal Neoplasms; Neoplasm Recurrence, Local; Retrospective Studies
PubMed: 35838837
DOI: 10.1007/s00432-022-04186-9 -
Neurosurgical Review Aug 2022Treatment of meningiomas refractory to surgery and irradiation is challenging and effective chemotherapies are still lacking. Recently, in vitro analyses revealed...
Treatment of meningiomas refractory to surgery and irradiation is challenging and effective chemotherapies are still lacking. Recently, in vitro analyses revealed decitabine (DCT, 5-aza-2'-deoxycytidine) to be effective in high-grade meningiomas and, moreover, to induce hypomethylation of distinct oncogenes only sparsely described in meningiomas in vivo yet.Expression of the corresponding onco- and tumor suppressor genes TRIM58, FAM84B, ELOVL2, MAL2, LMO3, and DIO3 were analyzed and scored by immunohistochemical staining and RT-PCR in samples of 111 meningioma patients. Correlations with clinical and histological variables and prognosis were analyzed in uni- and multivariate analyses.All analyzed oncogenes were highly expressed in meningiomas. Expression scores of TRIM58 tended to be higher in benign than in high-grade tumors 20 vs 16 (p = .002) and all 9 samples lacking TRIM58 expression displayed WHO grade II/III histology. In contrast, median expression scores for both FAM84B (6 vs 4, p ≤ .001) and ELOVL2 (9 vs 6, p < .001) were increased in high-grade as compared to benign meningiomas. DIO3 expression was distinctly higher in all analyzed samples as compared to the reference decitabine-resistant Ben-Men 1 cell line. Increased ELOVL2 expression (score ≥ 8) correlated with tumor relapse in both uni- (HR: 2.42, 95%CI 1.18-4.94; p = .015) and multivariate (HR: 2.09, 95%CI 1.01-4.44; p = .046) analyses.All oncogenes involved in DCT efficacy in vitro are also widely expressed in vivo, and expression is partially associated with histology and prognosis. These results strongly encourage further analyses of DCT efficiency in meningiomas in vitro and in situ.
Topics: Decitabine; Humans; Meningeal Neoplasms; Meningioma; Neoplasm Recurrence, Local; Oncogenes; Prognosis
PubMed: 35445910
DOI: 10.1007/s10143-022-01789-1