oligodendroglioma pathology outlines

Oligodendrogliomas may be diagnosed at any age but occur most commonly in young and middle-aged adults between 25 and 45 years old. Evidence from in vivo models have demonstrated reduced levels of STAT1 and CXCL10 in IDH-mutant gliomas. Nature 483, 474478. IDH mutations have also been implicated in the regulation of the recruitment of inflammatory cells to tumor sites, specifically through D-2HG. 6(4):371-4. have a. Bello MJ, Vaquero J, de Campos JM, et al. Cairncross, G., Berkey, B., Shaw, E., Jenkins, R., Scheithauer, B., Brachman, D., et al. [QxMD MEDLINE Link]. [QxMD MEDLINE Link]. doi: 10.1200/JCO.2001.19.9.2449, Cloughesy, T. F., Filka, E., Kuhn, J., Nelson, G., Kabbinavar, F., Friedman, H., et al. Other agents have also been investigated for recurring disease including paclitaxel, irinotecan, carboplatin, etoposide, and cisplatin (Poisson et al., 1991; Yung et al., 1991; Warnick et al., 1994; Chamberlain and Kormanik, 1995, 1999; Fulton et al., 1996; Macdonald et al., 1996; Friedman et al., 1999; Chang et al., 2001; Cloughesy et al., 2003; Batchelor et al., 2004; Ascierto et al., 2016). J. Adv Anat Pathol. (WC/jensflorian), Demonstration of IDH1 R132H mutation in oligodendroglioma. The estimated completion date is August 2022. Clinical presentation of pediatric oligodendrogliomas - ScienceDirect 0000016053 00000 n (Aug 2014). 0000225852 00000 n 2017 Nov 6. ABM Salah Uddin, MD Private Practice, Norwood Neurology; Consulting Staff, Department of Neurology, St Vincent's Hospital 1995. Widespread dissemination in a gliomatosis cerebri fashion is very rare (DDx: Diffuse leptomeningeal glioneuronal tumour). Randomized Trial of Radiation Therapy Plus Procarbazine, Lomustine, and Vincristine Chemotherapy for Supratentorial Adult Low-Grade Glioma: Initial Results of RTOG 9802. NCT04541082 is another ongoing phase 1 study aiming to determine the maximum tolerated dose of the oral drug ONC206, a member of the imipridone class of anti-cancer small molecules which target G protein-coupled receptors. Adjuvant procarbazine, lomustine, and vincristine improves progression-free survival but not overall survival in newly diagnosed anaplastic oligodendrogliomas and oligoastrocytomas: a randomized European Organisation for Research and Treatment of Cancer phase III trial. Pekmezci, M., Rice, T., Molinaro, A. M., Walsh, K. M., Decker, P. A., Hansen, H., et al. Neuro Oncol. J. Clin. Stephen A Berman, MD, PhD, MBA Professor of Neurology, University of Central Florida College of Medicine Detection of p 16, RB, CDK4, and p53 gene deletion and amplification by fluorescence in situ hybridization in 96 gliomas. J. Neurooncol. Recently, immunotherapy has been explored as a potential treatment modality. When possible, the sample is removed during surgery to remove the tumor. Eur. Can J Neurol Sci. HA conceived the idea for the manuscript. U. S. A. 79, 153157. 10(13):4303-6. Clin. Their good prognosis relative to other parenchymal tumors probably stems from inherently less aggressive biological behavior and a favorable response to radiation and chemotherapy. However, upon multivariate analysis there was no significant difference in overall survival (OS). During radiation therapy, you lie on a table while a machine moves around you. Celli P, Nofrone I, Palma L, et al. Neurol. Pharm. Ajuria, L., Nieva, C., Winkler, C., Kuo, D., Samper, N., Andreu, M. J., et al. Lack of p16INK4 or retinoblastoma protein (pRb), or amplification-associated overexpression of cdk4 is observed in distinct subsets of malignant glial tumors and cell lines. Temozolomide treatment for newly diagnosed anaplastic oligodendrogliomas: a clinical efficacy trial. The clear cytoplasm around the nucleus is a fixation artefact and not seen in smear, frozen sections or rapid fixation. Shin, D.-W., Lee, S., Song, S. W., Cho, Y. H., Hong, S. H., Kim, J. H., et al. Appl. 17, 20172022. [QxMD MEDLINE Link]. The tumor may enhance with contrast and is most often seen in anaplastic oligodendrogliomas. Virtually all oligodendrogliomas also have a mutation in isocitrate dehydrogenase (IDH1 or IDH2). CBTRUS Statistical Report: Primary brain and other central nervous system tumors diagnosed in the United States in 2010-2014. Long-term efficacy of early versus delayed radiotherapy for low-grade astrocytoma and oligodendroglioma in adults: the EORTC 22845 randomised trial. Seizure characteristics and control following resection in 332 patients with low-grade gliomas. Safety and efficacy of temozolomide in patients with recurrent anaplastic oligodendrogliomas after standard radiotherapy and chemotherapy. (2016). (2013b). 123, 853860. Throughout the entire process, educate the patient and family through regular follow-up care and involvement of support groups to cope with physical, emotional, and spiritual stress. doi: 10.1038/nature08617, Uittenbogaard, M., and Chiaramello, A. PLoS One 8:e68782. Curr. 0000005169 00000 n Neurosurgery 43, 10661073. doi: 10.1215/s1152851703000218, Baumgarten, P., Harter, P., Tnjes, M., Capper, D., Blank, A. E., Sahm, F., et al. (2007). 20 (8):2076-84. Cleveland Clinic Lerner College of Medicine, School of Medicine, Case Western Reserve University, United States, Department of Neurosurgery, School of Medicine, Tohoku University, Japan. Surgery to remove the tumor. 2004 Dec. 17(6):675-80. A., Tosoni, A., Cavallo, G., Reni, M., Franceschi, E., Bonaldi, L., et al. Ahmad H, Martin D, Patel SH, Donahue J, Lopes B, Purow B, et al. Consider Glioblastoma with oligo features when tumor is IDH1/2 wildtype and has no LOH 1p/19q and no ATRX loss. 19 (suppl_5):v1-v88. Amplification of the platelet-derived growth factor receptor-A (PDGFRA) gene occurs in oligodendrogliomas with grade IV anaplastic features. Oligodendrogliomas are molecularly defined by the presence of complete deletion of the short arm of chromosome 1 (1p) and the long arm of chromosome 19 (19q) (1p/19q co-deletion). Subsequently, this codeletion appeared to not only carry diagnostic, but also prognostic and predictive information, the latter aspect only recently resolved after carefully constructed clinical trials with very long follow-up times. 57(2):172-5. The brain surgeon, who is also called a neurosurgeon, works to remove the tumor without harming healthy brain tissue. Phase III trial of chemoradiotherapy for anaplastic oligodendroglioma: long-term results of RTOG 9402. 56(1):195-9. This occurs via 2-HGs inhibition of KDM4A, an -KG dependent deoxygenase, and destabilization of DEPTOR, a negative regulator of mTORC1/2, resulting in mTOR pathway activation (Carbonneau et al., 2016). Acta Neuropathol. This previously diagnosed oligoastrocytoma,, For unequivocal assessment of 1p/19q codeletion in oligodendroglial tumors, detection of whole-arm losses, MeSH This image demonstrates no contrast enhancement. 100, 22352241. doi: 10.1200/jco.1991.9.5.860, Zaatreh, M. M., Firlik, K. S., Spencer, D. D., and Spencer, S. S. (2003). Brandes, A. (2001). Molecular classification of anaplastic oligodendroglioma using next-generation sequencing: a report of the prospective randomized EORTC Brain Tumor Group 26951 phase III trial. -. The end result is DNA hypermethylation and thus the blockage of cellular differentiation (Kaminska et al., 2019). ; Tihan, T.; Lin, D.; McDonald, W.; Nigro, J.; Feuerstein, B.; Jackson, S.; Cohen, K. et al. Pang Y, Zhou S, Zumbo P, Betel D, Cisse B. Isocitrate dehydrogenase mutations suppress STAT1 and CD8+ T cell accumulation in gliomas. Treat. TCF12 Deficiency Impairs the Proliferation of Glioblastoma Tumor Cells and Improves Survival. (1994) and showed that the median time to progression for patients was at least 25.2 months for complete responders, 14.2 months for partial responders and 6.8 months for stable patients. No use, distribution or reproduction is permitted which does not comply with these terms. J. Clin. 2016 Mar;18(3):388-400. doi: 10.1093/neuonc/nov182. Another factor that increases probability of survival in low-grade gliomas is a high performance status. "Significant" or "brisk" mitotic activity. Table 1. Mason WP. Ruas, M., and Peters, G. (1998). One analysis looking at the downstream effect of TCF12 alterations showed a downregulation of TCF21, EZH2, and BMI1 pathway and especially CDH1 (E-cadherin), which has been shown to be implicated in tumor characteristics and metastasis (Lee et al., 2012). In an ongoing phase 3 study (NCT00887146), patients with AO or low-grade gliomas were split into two arms. When the latter is overproduced in cancer cells, it inhibits histone and DNA methylases and interferes with cellular metabolism. Radiation therapy uses powerful energy beams to kill tumor cells. However, in those groups with IDH mutations, including AO, TERT promoter mutation status was not a statistically significant prognostic factor (Dahlin et al., 2016). Oncol. 115, 240244. doi: 10.1016/j.celrep.2015.11.029, Warnick, R. E., Prados, M. D., Mack, E. E., Chandler, K. L., Doz, F., Rabbitt, J. E., et al. doi: 10.1093/jnen/59.6.495, Smits, A., and Duffau, H. (2011). Treatment is with surgery, when possible. 60, 11811189. (2003). (2001) reported that 16.7% of patients experienced a complete response and 27.1% experienced a partial response when receiving temozolomide (TMZ) after previous PCV. Neurology 51, 11401145. IDH1 mutation is sufficient to establish the glioma hypermethylator phenotype. (2008). Dysembryoplastic neuroepithelial tumor: a surgically curable tumor of young patients with intractable partial seizures. J. Clin. A neuropathologist should then review the tumor tissue. 226, 716. All in all, patients with 1p19q co-deleted tumors should be treated with RT and adjuvant PCV while those lacking this co-deletion should receive adjuvant TMZ. This co-deletion, along with the IDH mutation, enables a tumor to be classified as an oligodendroglioma according to the WHO 2016 criteria (Louis et al., 2016). Symptomatic spinal cord metastasis from cerebral oligodendroglioma. PCV and TMZ are also used in cases of recurrence but result in lower response rates and disease-free survival. Int. van den Bent, M. J., Dubbink, H. J., Marie, Y., Brandes, A. (1995). Diffuse gliomas histopathologically form a spectrum, both with regard to cell type (astrocytic, oligodendroglial, mixed) and malignancy grade. It is believed that anaplastic oligodendroglioma (AO) can progress from a lower grade oligodendroglioma after the acquisition of specific genetic alterations (Youssef and Miller, 2020). Interestingly, an in vitro treatment of cells with D-2HG also induced a similar methylation pattern (Lu et al., 2012) which further supports the vital role of this metabolite in epigenetic alteration and tumor formation. Your IP address is listed in our blacklist and blocked from completing this request. This image reveals increased nuclear pleomorphism and vascular proliferation. Neurology 68, 18311836. K.-Y., Pang, J. C.-S., Chung, N. Y.-F., Li, K. K.-W., Poon, W. S., Chan, D. T.-M., et al. Jorge C Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, New York Academy of SciencesDisclosure: Nothing to disclose. Better prognosis compared to astrocytic tumors. Int. Oligodendrogliomas are usually tumors of middle-aged adults, occurring most commonly in the 4 th and 5 th decades of life, somewhat older for grade 3 tumors 10,11. Targeting the RTK-PI3K-mTOR axis in malignant glioma: overcoming resistance. 13:861-873. Hence, cellular proliferation and dysregulation of pro-apoptotic pathways ensues (Ruas and Peters, 1998). MRI of an oligodendroglioma in the brain. The chromatin appearance is finely threadlike to smudgy, often associated with pointlike basophilic chromocenters, rather than nucleoli. Am. However, a clear distinction of both grades is not always possible. Grade II and grade III tumors can be differentiated most of the times by the presence of anaplastic features. Their predictive value stems from their close relationship to human gliomas. These tumors are called oligodendrogliomas because the cells resemble oligodendrocytes, a type of brain cell that supports and insulates nerve fibers in the CNS. (2019) depicted how the mutant IDH1 (R132H) blocks cellular differentiation and contributes to antitumor immunity. [3] This risk classification may change in the future as our understanding of the contributions of genetic markers to survivability evolves. Recurrence following neurosurgeon-determined gross-total resection of adult supratentorial low-grade glioma: results of a prospective clinical trial. 19, (Suppl._5) v1v88. This reaction promotes the formation of NADPH, the reduced form of NADP+, which helps in protecting the cell from oxidative radicals that can damage DNA (Soffietti et al., 1998; van den Bent et al., 1998). Cairncross, G.; Wang, M.; Shaw, E.; Jenkins, R.; Brachman, D.; Buckner, J.; Fink, K.; Souhami, L. et al. The Spectrum of Long-term Epilepsyassociated Tumors: Long-term Seizure and Tumor Outcome and Neurosurgical Aspects. Pekmezci et al. Such tumors may be considered oligoastrocytomas. 112, 801809. Analysis from the initial study design. MRI: frontal lobe tumor with cystic change, Vote for your favorite image from the PathologyOutlines.com Directory. Federal government websites often end in .gov or .mil. Irinotecan therapy in adults with recurrent or progressive malignant glioma. Lancet. Gene Expression Patterns 1, 115121. Bethesda, MD 20894, Web Policies 0000225968 00000 n They are responsible for histone methylation on lysine residues. More recently described biomarkers, including the non-balanced translocation leading to 1p/19q codeletion, promoter hypermethylation of the MGMT gene, mutations of the IDH1 or IDH2 gene, and mutations of FUBP1 (on 1p) or CIC (on 19q), have greatly enhanced our understanding of oligodendroglioma biology, although their diagnostic, prognostic, and predictive roles are less clear. Intravenous carboplatin for recurrent malignant glioma: a phase II study. Numbers may be higher when stringent classifiation criteria are not applied. This includes the tumor grade and type, traits of the cancer, the persons age and health when diagnosed, and how they respond to treatment. In the case of permitted digital reproduction, please credit the National Cancer Institute as the source and link to the original NCI product using the original product's title; e.g., Oligodendroglioma Diagnosis and Treatment was originally published by the National Cancer Institute.. Concurrent CIC mutations, IDH mutations, and 1p/19q loss distinguish oligodendrogliomas from other cancers. Pediatric oligodendroglioma is a low-grade glioma that remains relatively rare when compared to adults. IDH1 mutations seem to be at the core of this tumorigenesis (Johnson et al., 2014). Proc. Acad. 1985 Jul 1. Cairncross, G., Macdonald, D., Ludwin, S., Lee, D., Cascino, T., Buckner, J., et al. 2020 May 15. "Lack of H3K27 trimethylation is associated with 1p/19q codeletion in diffuse gliomas.". Contrast-enhanced computed tomography scan in a 44-year-old man with a 3-year history of epileptic seizures. ; Ohgaki, H.; Wiestler, OD. Oligodendroglioma - Libre Pathology Oligodendroglioma Oligodendroglioma, IDH mutant and 1p/19q codeleted is CNS tumour that is typically in the cerebral hemispheres. The patient sustained a disease-free response to nivolumab at least 12 months after surgery. 27, 149155. Oligodendrogliomas. ; Mork, S.; Graeber, MB. . (2017). 21, 251255. (2012) reported the survival benefit of immunotherapy in patients with relapsed AO enrolled in the HGG-IMMUNO-2003 trial. Features of CNS WHO grade 3 oligodendroglioma: Strict mitotic figure cutoffs do not currently exist; some authors suggest 6 mitoses per 10 high power fields for WHO grade 3 designation in tumors without necrosis or vascular proliferation (, Positive in > 90% of oligodendrogliomas (, Negative staining is not incompatible with oligodendroglioma if 1p / 19q codeletion is present, Grade 2 tumors: usually < 5% of tumor nuclei, Grade 3 tumors: generally > 10% of tumor nuclei (, Not routinely used for diagnostic purposes. (2013b). 13, 15011512. 2013;126:267276. (2014). All claims expressed in this article are solely those of the authors and do not necessarily represent those of their affiliated organizations, or those of the publisher, the editors and the reviewers. This website also contains material copyrighted by 3rd parties. Neuro Oncol. Jaeckle KA, Ballman KV, van den Bent M, Giannini C, Galanis E, et al. et al. Loss of heterozygosity of microsatellite loci on chromosome 9p in astrocytic tumors and its prognostic implications. for: Medscape. Oligodendroglioma is a molecularly defined diagnosis requiring demonstration of both: Unbalanced translocation between chromosome 1 and 19, resulting in whole arm loss of 1p and 19q chromosomal material (1p / 19q codeletion), Incomplete or partial deletions are not compatible with oligodendroglioma diagnosis, Often absent in teenagers with oligodendroglioma (, Loss of H3K27 trimethylation by immunohistochemistry (. While often found upregulated in many tumors, FUBP1 acts as a tumor suppressor gene due to its inactivating mutations reported in around 15% of oligodendroglial tumors (Baumgarten et al., 2014). J Clin Neurosci. Exposure to radiation and certain gene changes that can be passed down through families have been linked to a higher chance of developing oligodendrogliomas. (Feb 2007). Li S, Yan C, Huang L, Qiu X, Wang Z, Jiang T. Molecular prognostic factors of anaplastic oligodendroglial tumors and its relationship: a single institutional review of 77 patients from China. Pathology and Genetics of Tumours of the Nervous System. doi: 10.1007/s00401-012-0993-5, Sasaki, H., Zlatescu, M. C., Betensky, R. A., Ino, Y., Cairncross, J. G., and Louis, D. N. (2001). Computed tomography scan of a low-grade oligodendroglioma. (2016). A., Brandel, M. G., Hirshman, B. R., Dong, X., Carroll, K. T., Ali, M. A., et al. Int. (2006). Amide Proton Transfer-Chemical Exchange Saturation Transfer Imaging of Intracranial Brain Tumors and Tumor-like Lesions: Our Experience and a Review. Patients with co-deleted tumors demonstrate favorable prognoses (Smith et al., 2000a; Ino et al., 2001; Cairncross et al., 2006; Kaloshi et al., 2007; Cairncross et al., 2013). The unbalanced translocation of the centromeric regions of chromosomes 1p and 19q attribute to the loss of the whole arm on both chromosomes. J. Clin. 0000003918 00000 n Most commonly occurring between 25 and 45 years of age, grade III oligodendrogliomas tend to present 10 years later than grade II tumors and can rarely develop in younger and older populations. 11, 167175. Salvage chemotherapy with paclitaxel for recurrent primary brain tumors. (WC/jensflorian), Combined losses of 1p and 19q both and presence of IDH1 mutation in codon 132 or IDH2 mutation in codon 172 is required for final diagnosis and is prognostic and therapeutic relevant:[15][16][17]. [QxMD MEDLINE Link]. (2008). Cell 81, 323330. (2011). doi: 10.1016/S0140-6736(17)31442-3, van den Bent, M. J., Brandes, A. [QxMD MEDLINE Link]. 126, 907915. 2010, 111112. (2012) showed that an IDH1 mutation can induce a methylation profile known as the G-CIMP signature, which is a glioma specific methylation pattern at CpG islands. D-2-hydroxyglutaric acid induces oxidative stress in cerebral cortex of young rats. 0000230693 00000 n :: BTRT :: Brain Tumor Research and Treatment Accounting to up to 5% of all neuroepithelial tumors (Ostrom et al., 2017), oligodendroglial tumors have an incidence rate of around 1,000 new cases per year in the United States. Afterward in 2001, Chinot et al. ROS and brain gliomas: an overview of potential and innovative therapeutic strategies. Glioblastoma with oligodendroglioma component (GBM-O): molecular genetic and clinical characteristics. 1994 Apr 15. The wildtype (WT) TERT group was associated with good prognosis only in IDH1/IDH2 WT (IDH-WT) grade II/III astrocytomas. 24(18):2715-22. In 8 recurring oligodendrogliomas, the number of GFAP positive tumour cells was the same in the primary tumour and in its recurrence. Phosphoinositide 3-Kinase Health Dis. Oligodendrogliomas usually appear as a single tumor with well-defined borders. CDK inhibitors: positive and negative regulators of G1-phase progression. Ueki, K., Ono, Y., Henson, J. W., Efird, J. T., von Deimling, A., and Louis, D. N. (1996). Neurobiol. Prospective studies are required to better . doi: 10.1038/nature16490, Friedman, H., Petros, W. P., Friedman, A. H., Schaaf, L. J., Kerby, T., Lawyer, J., et al. 2002 Apr 15. Clin Cancer Res. If you are the site owner (or you manage this site), please whitelist your IP or if you think this block is an error please open a support ticket and make sure to include the block details (displayed in the box below), so we can assist you in troubleshooting the issue. 2019 Sep 23. Zou et al. (2013). CODEL: phase III study of RT, RT+ TMZ, or TMZ for newly diagnosed 1p/19q codeleted oligodendroglioma. Oncol. at the National Cancer Institute, An official website of the United States government, Oligodendroglioma Diagnosis and Treatment, Finding Your Best Path and Fighting Brain Cancer, 5-year survival rate for oligodendroglioma is 74.1%, Outcomes and Risk Project for Patients with Rare CNS Cancers, Evaluation of the Natural History and Specimen Banking for Patients with CNS Cancers, Virtual Reality Study for Patients with Brain Cancer, Sleep Observation Study for Patients with Brain Cancer, CALM Therapy Intervention Study for Patients with Brain Cancer, Nivolumab for Patients with IDH-Mutant Gliomas, ONC206 for Patients with Rare CNS Neoplasms, A New Web-based Study of Low-Grade Gliomas, Two Sons with a Rare Brain Cancer? (2013). doi: 10.1007/s11060-012-0946-9, Carbonneau, M., Gagn, L. M., Lalonde, M.-E., Germain, M.-A., Motorina, A., Guiot, M.-C., et al. 589 0 obj <>stream 133, 10011016. 17:984. doi: 10.3390/ijms17060984. The PFS and OS were 3.4 and 18.8 months, respectively. A., Taphoorn, M. J., Wesseling, P., et al. Shaw EG, Wang M, Coons SW, Brachman DG, Buckner JC, Stelzer KJ, et al. Oligodendroglioma is genetically defined as a tumor confirmed to harbor either an IDH1 or IDH2 mutation along with co-deletion of chromosome arms 1p and 19q. Predictors of seizure freedom after resection of supratentorial low-grade gliomas: a review. 0000245854 00000 n Buckner JC, Shaw EG, Pugh SL, Chakravarti A, Gilbert MR, Barger GR, et al. Epilepsia 44, 822830. (2014). All of the cases have completed central pathological review and met the PLNTY criteria of the following: 1) infiltrative and nodular growth pattern; 2) invariable presence of oligodendroglioma-like cellular component with more or less polymorphic cellular elements including pleomorphic and spindle cells; 3) intense immunolabeling for cluster of
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