scholarly journals Low-Grade Gliomas in Patients with Noonan Syndrome: Case-Based Review of the Literature

Diagnostics ◽  
2020 ◽  
Vol 10 (8) ◽  
pp. 582
Author(s):  
Mariachiara Lodi ◽  
Luigi Boccuto ◽  
Andrea Carai ◽  
Antonella Cacchione ◽  
Evelina Miele ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Noonan Syndrome ◽  
Positive Outcome ◽  
Point Of View ◽  
Optic Pathway Glioma ◽  
Glioneuronal Tumor ◽  
Low Grade ◽  
Low Grade Gliomas ◽  
Solid Malignancies

Noonan syndrome (NS) is a congenital autosomic dominant condition characterized by a variable spectrum from a clinical and genetical point of view. Germline mutations in more than ten genes involved in RAS–MAPK signal pathway have been demonstrated to cause the disease. An higher risk for leukemia and solid malignancies, including brain tumors, is related to NS. A review of the published literature concerning low grade gliomas (LGGs) in NS is presented. We described also a 13-year-old girl with NS associated with a recurrent mutation in PTPN11, who developed three different types of brain tumors, i.e., an optic pathway glioma, a glioneuronal neoplasm of the left temporal lobe and a cerebellar pilocytic astrocytoma. Molecular characterization of the glioneuronal tumor allowed to detect high levels of phosphorylated MTOR (pMTOR); therefore, a therapeutic approach based on an mTOR inhibitor (everolimus) was elected. The treatment was well tolerated and proved to be effective, leading to a stabilization of the tumor, which was surgical removed. The positive outcome of the present case suggests considering this approach for patients with RASopathies and brain tumors with hyperactivated MTOR signaling.

Cellular and molecular characterization of IDH1-mutated diffuse low grade gliomas reveals tumor heterogeneity and absence of EGFR/PDGFRα activation

Glia ◽  
2017 ◽  
Vol 66 (2) ◽  
pp. 239-255 ◽  
Author(s):  
S. Azar ◽  
N. Leventoux ◽  
C. Ripoll ◽  
V. Rigau ◽  
C. Gozé ◽  
...  
Keyword(s):  
Molecular Characterization ◽  
Tumor Heterogeneity ◽  
Low Grade ◽  
Low Grade Gliomas

Biological significance of tissue plasminogen activator content in brain tumors

1991 ◽  
Vol 74 (3) ◽  
pp. 480-486 ◽  
Author(s):  
Raymond Sawaya ◽  
O. Juhani Rämö ◽  
Mei Lin Shi ◽  
George Mandybur
Keyword(s):  
Brain Tumors ◽  
Plasminogen Activator ◽  
Tumor Necrosis ◽  
Tumor Tissue ◽  
Biological Significance ◽  
Low Grade ◽  
Normal Brain ◽  
Content Type ◽  
Low Grade Gliomas ◽  
Fine Print

✓ Fresh brain-tumor samples were obtained at operation and analyzed for their content of tissue type plasminogen activator (tPA) using an activity assay (gel chromatography zymogram) and an enzyme-linked immunospecific assay. The specimens were taken from 23 glioblastomas, 35 metastatic tumors, 42 meningiomas, 16 low-grade gliomas, and seven acoustic neurinomas; seven specimens were from normal brain. A strong correlation was found between the results of the two assays (r = 0.77, p < 0.0001). There was a threefold difference in the tPA content of the benign tumors as compared to malignant tumors (p = 0.0006), the latter having less tPA. Histologically benign meningiomas contained higher tPA than malignant meningiomas (p = 0.01); however, the difference between low-grade gliomas and high-grade gliomas was less evident. Overall regression analysis data have shown an inverse relationship between the tissue content in tPA and the presence and degree of tumor necrosis and peritumoral brain edema (p = 0.004 and p = 0.0004, respectively). This finding was most consistent in the glioblastoma group where the correlation coefficient values were r = 0.53 and r = −0.55, respectively. There was no significant correlation between the tissue tPA content and the age and sex, steroid use, or plasma tPA of the patients or the duration of symptoms. In summary, this is the first demonstration of tPA in a large series of human brain tumors and in normal brain. The differences observed have clear biological significance and, although the source of tPA in tumor tissue is still unknown, a relative reduction in tPA in tumor tissue may play an integral role in the development of tissue necrosis and tissue edema. The lack of tPA in tumor necrosis was not due to tissue destruction and cell death since urokinase was readily detectable in that tissue.

scholarly journals Awake Craniotomy for Left Insular Low-Grade Glioma Removal on a Patient with Learning Disabilities

2017 ◽  
Vol 06 (01) ◽  
pp. 041-043 ◽  
Author(s):  
Andrej Vranic ◽  
Blaz Koritnik ◽  
Jasmina Markovic-Bozic
Keyword(s):  
Learning Disabilities ◽  
Case Report ◽  
Brain Tumors ◽  
Awake Craniotomy ◽  
Awake Surgery ◽  
Cortical Mapping ◽  
Low Grade ◽  
Low Grade Gliomas ◽  
Eloquent Areas ◽  
Slow Growing

Introduction Low-grade gliomas (LGG) are slow-growing primary brain tumors in adults, with high tropism for eloquent areas. Standard approach in treatment of LGG is awake craniotomy with intraoperative cortical mapping — a method which is usually used on adult and fully cooperative patients. Case Report We present the case of a patient with learning disabilities (PLD) who was operated for left insular LGG awake craniotomy, and intraoperative cortical mapping were performed and the tumor was gross totally removed. Conclusion Awake surgery for left insular LGG removal is challenging; however, it can be performed safely and successfully on PLD.

scholarly journals Patient-specific characterization of the invasiveness and proliferation of low-grade gliomas using serial MR imaging and a mathematical model of tumor growth

2015 ◽  
Vol 33 (6) ◽  
pp. 2883-2888 ◽  
Author(s):  
LEITH HATHOUT ◽  
BENJAMIN M. ELLINGSON ◽  
TIMOTHY F. CLOUGHESY ◽  
WHITNEY B. POPE
Keyword(s):  
Mathematical Model ◽  
Mr Imaging ◽  
Tumor Growth ◽  
Patient Specific ◽  
Low Grade ◽  
Low Grade Gliomas

Treatment of children with progressive or recurrent brain tumors with carboplatin or iproplatin: a Pediatric Oncology Group randomized phase II study.

1992 ◽  
Vol 10 (2) ◽  
pp. 249-256 ◽  
Author(s):  
H S Friedman ◽  
J P Krischer ◽  
P Burger ◽  
W J Oakes ◽  
B Hockenberger ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Phase Ii ◽  
Pediatric Oncology ◽  
Stable Disease ◽  
Phase Ii Study ◽  
Low Grade ◽  
Oncology Group ◽  
Low Grade Gliomas ◽  
Randomized Phase Ii ◽  
Pediatric Oncology Group

PURPOSE The Pediatric Oncology Group (POG) conducted a randomized phase II study to evaluate the activity of carboplatin and iproplatin in children with progressive or recurrent brain tumors. PATIENTS AND METHODS The study was designed to evaluate the activity of these agents and to compare the toxicities associated with their use. Treatment consisted of carboplatin 560 mg/m2 at 4-week intervals or iproplatin 270 mg/m2 at 3-week intervals. RESULTS The major toxicity observed was myelosuppression, particularly thrombocytopenia, for both agents. Ototoxicity (grade 1 or 2) was seen in 2.5% of patients treated with carboplatin and 1.3% of patients treated with iproplatin. The majority of patients with low-grade astrocytic neoplasms treated with carboplatin (nine of 12 patients) or iproplatin (eight of 12 patients) demonstrated tumor response or prolonged stable disease that persisted off-therapy. The duration of stable disease produced by carboplatin was particularly striking, ranging from 2 months to 68 + months (median, 40 + months). Neither drug demonstrated appreciable activity in the treatment of medulloblastoma (two of 26 responses to carboplatin, one of 14 responses to iproplatin), ependymoma (two of 17 responses to carboplatin, none of seven responses to iproplatin), high-grade glioma (two of 19 responses to carboplatin, one of 14 responses to iproplatin), or brain-stem tumors (one of 23 responses to carboplatin, none of 14 responses to iproplatin). CONCLUSION Carboplatin is active against low-grade gliomas. Further evaluation of the role of carboplatin in the preirradiation treatment of children with low-grade gliomas of the optic pathway is currently underway in a clinical trial.

scholarly journals Detection of human herpesvirus-6 variants in pediatric brain tumors: Association of viral antigen in low grade gliomas

2009 ◽  
Vol 46 (1) ◽  
pp. 37-42 ◽  
Author(s):  
John R. Crawford ◽  
Maria R. Santi ◽  
Halldora K. Thorarinsdottir ◽  
Robert Cornelison ◽  
Elisabeth J. Rushing ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Viral Antigen ◽  
Human Herpesvirus ◽  
Pediatric Brain Tumors ◽  
Low Grade ◽  
Human Herpesvirus 6 ◽  
Low Grade Gliomas ◽  
Herpesvirus 6 ◽  
Pediatric Brain

scholarly journals HGG-06. REMARKABLE RESPONSE TO BRAF INHIBITOR IN AN INFANT WITH DISSEMINATED DIFFUSE LEPTOMENINGEAL GLIONEURONAL TUMOR (DLGNT)

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii345-iii345
Author(s):  
Le Le Aung
Keyword(s):  
Braf Inhibitor ◽  
Braf V600e ◽  
Proliferation Index ◽  
Glioneuronal Tumor ◽  
Low Grade ◽  
Braf Inhibitors ◽  
Visual Contact ◽  
Ki 67 ◽  
Low Grade Gliomas ◽  
Diffuse Leptomeningeal Glioneuronal Tumor

Abstract INTRODUCTION Diffuse Leptomeningeal Glioneuronal Tumor (DLGNT) are rare CNS tumors and in infants, they can be lethal. There are several anecdotal reports in infants with low grade gliomas (LGG) with treated with BRAF inhibitors. METHODS A six-month old baby girl presented with a 2-week history of absent visual contact and vomiting. Imaging revealed a large 4.7 X 4.2 X 2.8 cm suprasellar charismatic region mass and multiple small extra-axial plaques in spinal canal. The child developed significant ascites post VP shunt requiring shunt externalization, extensive protein infusion support and hospitalization for six weeks. Immunohisto-chemical staining revealed Olig-2 and S-100, GFAP and synaptophysin positive. EMA showed patchy cytroplasmic reactivity in stromal cells and CD99 showed diffuse reactivity in stromal and lesional cells. INI-1, IDH-1, and CD117 were negative. Ki-67 proliferation index was 8–10%. PCR for BRAF V600E/E2/D was detected and KIAA1549-BRAF fusion as negative. This was confirmed by Genome Wide Next Generation Sequencing. While waiting for GNS testing results, the baby received one dose of Vinblastine. However, within seven days of initiating Debrafenib, significant clinical and radiological responses were observed. CONCLUSION The baby continues safely on Debrafenib with continued dramatic radiological response. This suggest that there may be a role in early initiation of targeted therapy such as BRAF inhibitors rather than giving standard chemotherapy such as Vinblastine or Carboplatin-Vincristine in extremely ill infants with low grade gliomas.

scholarly journals Prevalence of asymptomatic glioma and implications for survival

2020 ◽  
Author(s):  
Paula Province Warren ◽  
Mina Lobbous ◽  
Noah C. Peeri ◽  
Zachary J. Thompson ◽  
Reid C. Thompson ◽  
...  
Keyword(s):  
Brain Tumors ◽  
Brain Imaging ◽  
Medical Condition ◽  
Epidemiologic Study ◽  
Tumor Location ◽  
Low Grade ◽  
Who Grade ◽  
Low Grade Gliomas ◽  
Improve Patient Survival ◽  
Asymptomatic Patients

AbstractBackgroundBrain tumors can present as focal neurologic deficits (reflecting the tumor location) or generalized symptoms due to increased intracranial pressure. Occasionally, brain tumors can be found incidentally in asymptomatic patients or in patients with unrelated symptoms who undergo brain imaging. The term incidentaloma is used to refer to these imaging abnormalities.ObjectiveThe object of this study was to examine the prevalence and correlates of asymptomatic glioma in a large epidemiological study of brain tumors.MethodsThe analysis was based on a large series of patients with glioma (N = 1989) enrolled in a multicenter clinic-based epidemiologic study between 2005 and 2017. Patients were considered asymptomatic from the tumor, and thus as having an incidentally detected glioma (IDG), if the tumor was diagnosed during workup of injury or unrelated medical condition.ResultsA total of 32 of 1989 (1.6%) patients were asymptomatic at diagnosis. The leading indication for brain imaging in IDG was non-workplace injuries followed by medical workup for unrelated conditions. IDG was more prevalent in patients younger than 50 years of age (2.6% vs 1.0%). IDG was also more common in patients with low grade gliomas (4.7% for WHO grade II and 1.5% for WHO grade III) vs glioblastomas (0.6% in WHO grade IV).ConclusionThe present data suggest that gliomas may be found incidentally, especially among low grade gliomas. Studies of IDG may be useful as a proxy for early detection of tumor as a means to improve patient survival.

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