Resolving mystery behind autonomous retrogression of low-grade gliomas; a systematic review

2020 ◽  
pp. 1-28
Author(s):  
Syed Ijlal Ahmed ◽  
Syeda Beenish Bareeqa ◽  
Syeda Sana Samar ◽  
Syed Daniyal Ahmed Jilanee
Keyword(s):  
Systematic Review ◽  
Therapeutic Intervention ◽  
Spontaneous Regression ◽  
Experimental Studies ◽  
Optic Pathway Glioma ◽  
Low Grade ◽  
Evidence Based ◽  
Optic Pathway ◽  
Diffuse Astrocytoma ◽  
Low Grade Gliomas

Abstract Objective: To review evidence-based data on spontaneous retrogression of low-grade gliomas with respect to interval till regression, type of glioma and patient outcome. Method: The systematic review comprised medical literature in English language published from January 1997 to January 2017 on Scopus, PubMed and Google Scholar databases to establish consensus about the possible mechanism of spontaneous regression, the role of therapeutic intervention and failure of management strategies in low-grade gliomas. Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines were followed during the review. Results: Of the 176 articles identified, 73(41.5%) were shortlisted for detailed assessment. Of them, 10(13.7%) were included; 5(50%) case reports and 5(50%) case series. There were 23 cases of spontaneous regression; 15(65.2%) males and 8(34.7%) females. The interval of regression varied from 3 months to 15.5 years, and the most commonly presenting low-grade glioma type was optic pathway glioma 11(47.4%). Conclusion: The phenomenon of regression was most evident in optic pathway glioma. Literature suggested that low-grade gliomas should undergo serial imaging before implying any therapeutic intervention. However, the evidence-based proof, large-scale experimental studies and ethical considerations are still required to standardise this strategy. Key Words: Pilocytic astrocytomas, Desmoplastic infantile ganglioglioma, DIG, Desmoplastic infantile astrocytomas, DIA, Diffuse astrocytoma, Spontaneous regression. Continuous...

scholarly journals Management of low-grade glioma: a systematic review and meta-analysis

2018 ◽  
Vol 6 (4) ◽  
pp. 249-258 ◽  
Author(s):  
Timothy J Brown ◽  
Daniela A Bota ◽  
Martin J van Den Bent ◽  
Paul D Brown ◽  
Elizabeth Maher ◽  
...  
Keyword(s):  
Systematic Review ◽  
Meta Analysis ◽  
Progression Free Survival ◽  
Low Grade Glioma ◽  
World Health ◽  
Subtotal Resection ◽  
Low Grade ◽  
Evidence Based ◽  
Free Survival ◽  
Low Grade Gliomas

Abstract Background Optimum management of low-grade gliomas remains controversial, and widespread practice variation exists. This evidence-based meta-analysis evaluates the association of extent of resection, radiation, and chemotherapy with mortality and progression-free survival at 2, 5, and 10 years in patients with low-grade glioma. Methods A quantitative systematic review was performed. Inclusion criteria included controlled trials of newly diagnosed low-grade (World Health Organization Grades I and II) gliomas in adults. Eligible studies were identified, assigned a level of evidence for every endpoint considered, and analyzed according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. The relative risk of mortality and of progression at 2, 5, and 10 years was calculated for patients undergoing resection (gross total, subtotal, or biopsy), radiation, or chemotherapy. Results Gross total resection was significantly associated with decreased mortality and likelihood of progression at all time points compared to subtotal resection. Early radiation was not associated with decreased mortality; however, progression-free survival was better at 5 years compared to patients receiving delayed or no radiation. Chemotherapy was associated with decreased mortality at 5 and 10 years in the high-quality literature. Progression-free survival was better at 5 and 10 years compared to patients who did not receive chemotherapy. In patients with isocitrate dehydrogenase 1 gene (IDH1) R132H mutations receiving chemotherapy, progression-free survival was better at 2 and 5 years than in patients with IDH1 wild-type gliomas. Conclusions Results from this review, the first to quantify differences in outcome associated with surgery, radiation, and chemotherapy in patients with low-grade gliomas, can be used to inform evidence-based management and future clinical trials.

scholarly journals PATH-30. TARGETED THERAPIES FOR PEDIATRIC LOW-GRADE GLIOMAS: A CASE SERIES

2019 ◽  
Vol 21 (Supplement_6) ◽  
pp. vi149-vi149
Author(s):  
Nidhi Shah ◽  
Andrew Walter ◽  
Gurcharanjeet Kaur
Keyword(s):  
Targeted Therapies ◽  
Case Series ◽  
Braf V600e ◽  
Optic Pathway Glioma ◽  
Low Grade ◽  
Optic Pathway ◽  
Intravenous Chemotherapy ◽  
Low Grade Gliomas ◽  
Patient Reported ◽  
Cns Malignancies

Abstract Pediatric central nervous system (CNS) malignancies are the most common malignancies of childhood. The standard treatment plan for most CNS malignancies involves surgery, chemotherapy, radiation, and/or a combination of above therapies. Unresectable symptomatic low grade gliomas, such as pilocytic astrocytomas and gangliogliomas, are slow growing tumors that are typically responsive to a single course of intravenous chemotherapy, but in select patients these WHO grade I or II tumors can recur and be refractory to multiple courses. Molecular diagnostics can offer valuable insight into the tumor microenvironment, where targeted therapies can be offered for specific actionable mutations. Here we report a case series of 3 pediatric patients, with unique CNS malignancies, currently on targeted therapies for tumor-specific somatic mutations. Patient A is a 5 year old male with unresectable Neurofibromatosis-1 related plexiform neurofibroma of the nasopharyngeal space as well as optic pathway glioma, with a mutation of the MAPK/ERK pathway. Patient B is a 6 year old male with recurrent, refractory pilocytic astrocytoma of the optic pathway and hypothalamus, with progression through several courses of intravenous chemotherapy, noted to have somatic NACC2-NTRK mutation. Patient C is a 17 year old female with unresectable, pontomedullary ganglioglioma, noted to have BRAF-V600E mutation. Patient A is treated with Selumetinib, for about 6 months, with near resolution of nasopharyngeal mass. Patient B is treated with Larotrectinib, for about 3 months, with stability of clinical symptoms. Patient C is treated with Vemurafenib, for about 10 months, with stability of lesion size and patient-reported clinical improvement. No adverse events were noted for any of these patients and all medications were administered orally. Significant improvement in quality of life was reported, as they did not have central lines or bone marrow suppression. Targeted inhibitors provide a reasonable treatment option for relapsed, refractory CNS malignancies with actionable mutations.

Molecular Alterations in Pediatric Low-Grade Gliomas That Led to Death

2021 ◽  
Author(s):  
Jared T Ahrendsen ◽  
Claire Sinai ◽  
David M Meredith ◽  
Seth W Malinowski ◽  
Tabitha M Cooney ◽  
...  
Keyword(s):  
Braf V600e ◽  
Molecular Characteristics ◽  
Low Grade ◽  
Diffuse Astrocytoma ◽  
Term Survival ◽  
Pten Loss ◽  
Low Grade Gliomas ◽  
Molecular Alterations ◽  
Idh1 R132h ◽  
Poor Outcomes

Abstract Pediatric low-grade gliomas (PLGGs) have excellent long-term survival, but death can occasionally occur. We reviewed all PLGG-related deaths between 1975 and 2019 at our institution: 48 patients were identified; clinical data and histology were reviewed; targeted exome sequencing was performed on available material. The median age at diagnosis was 5.2 years (0.4–23.4 years), at death was 13.0 years (1.9–43.2 years), and the overall survival was 7.2 years (0.0–33.3 years). Tumors were located throughout CNS, but predominantly in the diencephalon. Diagnoses included low-grade glioma, not otherwise specified (n = 25), pilocytic astrocytoma (n = 15), diffuse astrocytoma (n = 3), ganglioglioma (n = 3), and pilomyxoid astrocytoma (n = 2). Recurrence occurred in 42/48 cases, whereas progression occurred in 10. The cause of death was direct tumor involvement in 31/48 cases. Recurrent drivers included KIAA1549-BRAF (n = 13), BRAF(V600E) (n = 3), NF1 mutation (n = 3), EGFR mutation (n = 3), and FGFR1-TACC1 fusion (n = 2). Single cases were identified with IDH1(R132H), FGFR1(K656E), FGFR1 ITD, FGFR3 gain, PDGFRA amplification, and mismatch repair alteration. CDKN2A/B, CDKN2C, and PTEN loss was recurrent. Patients who received only chemotherapy had worse survival compared with patients who received radiation and chemotherapy. This study demonstrates that PLGG that led to death have diverse molecular characteristics. Location and co-occurring molecular alterations with malignant potential can predict poor outcomes.

The management of incidental low-grade gliomas using magnetic resonance imaging: systematic review and optimal treatment paradigm

2011 ◽  
Vol 31 (6) ◽  
pp. E12 ◽  
Author(s):  
Ashish H. Shah ◽  
Karthik Madhavan ◽  
Deborah Heros ◽  
Daniel M. S. Raper ◽  
J. Bryan Iorgulescu ◽  
...  
Keyword(s):  
Systematic Review ◽  
Mr Imaging ◽  
Active Surveillance ◽  
Case Series ◽  
Treatment Decisions ◽  
Low Grade ◽  
Management Options ◽  
Low Grade Gliomas ◽  
Presenting Symptoms ◽  
Radiological Evidence

Object The discovery of incidental low-grade gliomas (LGGs) on MR imaging is rare, and currently there is no existing protocol for management of these lesions. Various studies have approached the dilemma of managing patients with incidental LGGs. While some advocate surgery and radiotherapy, others reserve surgery until there is radiological evidence of growth. For neurosurgeons and radiologists, determining the course of action after routine brain imaging poses not only a medical but also an ethical dilemma. The authors conducted a systematic review of case reports and case series in hopes of enhancing the current understanding of the management options for these rare lesions. Methods A PubMed search was performed to include all relevant MR imaging studies in which management of suspected incidental LGG was reported. Comparisons were made between the surgical treatment arm and the active surveillance arm in terms of outcome, mode of discovery, reasons for treatment, and histology. Results Nine studies with 72 patients were included in this study (56 in the surgical arm and 16 in the active surveillance arm). Within the surgical arm, 49% remained deficit free after treatment, 25% showed evidence of tumor progression, 13% underwent a second treatment, and 7% died. The active surveillance group resulted in no unanticipated adverse events, with serial imaging revealing no tumor growth in all cases. Lesion regression was reported in 31% of this group. The surgical arm's mortality rate was 7% compared with 0% in the active surveillance arm. Conclusions Treatment decisions for incidental LGG should be individualized based on presenting symptoms and radiological evidence of growth. The asymptomatic patient may be monitored safely with serial MR imaging and occasionally PET scanning before treatment is initiated. In patients presenting with nonspecific symptoms or concurrent symptomatic lesions, treatment may be initiated earlier to reduce potential morbidity. All treatment decisions must be tempered by patient factors and expectations of anticipated benefit.

scholarly journals Single-Agent Oral Vinorelbine in the Treatment of Pediatric Progressive Optic Pathway Glioma: A Single Institutional Experience

2021 ◽  
pp. 1-6
Author(s):  
Natalia Dassi ◽  
Natalia Dassi ◽  
N.S. Silva ◽  
F.A. Silva ◽  
D.B. Almeida ◽  
...  
Keyword(s):  
Single Agent ◽  
Drug Acquisition ◽  
Gastrointestinal Toxicity ◽  
Optic Pathway Glioma ◽  
Oral Drug ◽  
Low Grade ◽  
Optic Pathway ◽  
Oral Vinorelbine ◽  
Minor Response ◽  
Response To Chemotherapy

Purpose: The vinca alkaloids’ activity against pediatric low-grade glioma (PLGG) is well established. The goal of the present study is to describe our experience with oral vinorelbine in patients with progressive optic pathway glioma (OPG), not only regarding the clinical response, but also the cost benefit using an oral medication. Methods: Patients under 21 years of age with unresectable and/or progressive OPG were eligible. Oral vinorelbine was administered at a dose of 90mg/m2 daily on days 0, 8 and 22, in a scheme of 4 weekly cycles for a total of 18 cycles (54 doses). Results: From 2013 to 2018, sixteen patients were enrolled onto the study, with a median age of 9,1 years (range 4,6-17,8y). The most common histology was pilocytic astrocytoma (88,8%). Best response to chemotherapy was reviewed with a response rate (complete, partial, or minor response) of 30% for the patients treated exclusively with the oral drug. Five-year event-free survival (EFS) rate was 43.4%. Six patients had to change to intravenous vinorelbine due to gastrointestinal toxicity, vomiting grade III. None of the patients showed neurotoxicity. The total cost including drug acquisition, administration and toxicity management was lower with the oral formulation comparing to IV one. Conclusion: Single-agent oral vinorelbine seems to have some clinical activity in the management of recurrent or refractory pediatric OPG, being an interesting and cost-effective option, minding that gastrointestinal toxicity may be limiting and a combination of antiemetics should be considered in this treatment regimen.

Clinicopathological Features and Management of Appendiceal Mucoceles: A Systematic Review

2018 ◽  
Vol 84 (2) ◽  
pp. 273-281 ◽  
Author(s):  
William F. Morano ◽  
Elizabeth M. Gleeson ◽  
Sean H. Sullivan ◽  
Vennila Padmanaban ◽  
Beth L. Mapow ◽  
...  
Keyword(s):  
Systematic Review ◽  
Odds Ratio ◽  
Right Hemicolectomy ◽  
Clinicopathological Features ◽  
Low Grade ◽  
Evidence Based ◽  
Retention Cyst ◽  
Medline Search ◽  
Pathologic Evaluation ◽  
Colonic Malignancy

Appendiceal mucoceles (AMs) are rare mucin-containing neoplasms with malignant potential. Lack of evidence-based data exists defining clinicopathological features for management. MEDLINE search between 1995 and 2015 was performed using search criteria “Appendix mucocele.” Systematic review of patient-, pathologic-, and treatment-related characteristics was performed and data analyzed. Among 276 cases of non-perforated AMs, 163 (59%) patients were female, with variable and nonspecific presentation. Patients were treated with appendectomy (52.1%), right hemicolectomy (17.6%), partial cecectomy (17.2%), and ileocecetomy (13.1%). Pathologic evaluation revealed the following: cystadenoma/low-grade appendiceal mucinous neoplasm (54%), unspecified/benign (25%), retention cyst (14.1%), cystadenocarcinoma (4.2%), and mucosal hyperplasia (2.9%). All 11 (4.2%) patients with cystadenocarcinoma were female ( P = 0.004), odds ratio for malignancy 1.07 times higher for women. Synchronous colonic malignancy was reported in three patients (27%) with cystadenocarcinoma ( P = 0.007), odds ratio of 12.1. AMs have low risk for malignancy. Treatment should begin with appendectomy-only and subsequently guided by pathologic diagnosis.

Prognostic factors for progression of childhood optic pathway glioma: A systematic review

2006 ◽  
Vol 42 (12) ◽  
pp. 1807-1816 ◽  
Author(s):  
Enrico Opocher ◽  
Leontien C.M. Kremer ◽  
Liviana Da Dalt ◽  
Marianne D. van de Wetering ◽  
Elisabetta Viscardi ◽  
...  
Keyword(s):  
Systematic Review ◽  
Prognostic Factors ◽  
Optic Pathway Glioma ◽  
Optic Pathway
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