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A brain tumor occurs when abnormal cells form within the brain. There are two main types of tumors: malignant (cancerous) tumors and benign (non-cancerous) tumors. These can be further classified as primary tumors, which start within the brain, and secondary tumors, which most commonly have spread from tumors located outside the brain, known as brain metastasis tumors. All types of brain tumors may produce symptoms that vary depending on the size of the tumor and the part of the brain that is involved. Where symptoms exist, they may include headaches, seizures, problems with vision, vomiting and mental changes. Other symptoms may include difficulty walking, speaking, with sensations, or unconsciousness.
Rhabdomyosarcoma (RMS) is a highly aggressive form of cancer that develops from mesenchymal cells that have failed to fully differentiate into myocytes of skeletal muscle. Cells of the tumor are identified as rhabdomyoblasts.
A glioma is a type of tumor that starts in the glial cells of the brain or the spine. Gliomas comprise about 30 percent of all brain tumors and central nervous system tumours, and 80 percent of all malignant brain tumours.
Oligodendrogliomas are a type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell. They occur primarily in adults but are also found in children.
Glioblastoma, previously known as glioblastoma multiforme (GBM), is the most aggressive and most common type of cancer that originates in the brain, and has a very poor prognosis for survival. Initial signs and symptoms of glioblastoma are nonspecific. They may include headaches, personality changes, nausea, and symptoms similar to those of a stroke. Symptoms often worsen rapidly and may progress to unconsciousness.
Astrocytoma is a type of brain tumor. Astrocytomas originate from a specific kind of star-shaped glial cell in the cerebrum called an astrocyte. This type of tumor does not usually spread outside the brain and spinal cord and it does not usually affect other organs. After glioblastomas, astrocytomas are the second most common glioma and can occur in most parts of the brain and occasionally in the spinal cord.
Pilocytic astrocytoma is a brain tumor that occurs most commonly in children and young adults. They usually arise in the cerebellum, near the brainstem, in the hypothalamic region, or the optic chiasm, but they may occur in any area where astrocytes are present, including the cerebral hemispheres and the spinal cord. These tumors are usually slow growing and benign, corresponding to WHO malignancy grade 1.
A blastoma is a type of cancer, more common in children, that is caused by malignancies in precursor cells, often called blasts. Examples are nephroblastoma, medulloblastoma, and retinoblastoma. The suffix -blastoma is used to imply a tumor of primitive, incompletely differentiated cells, e.g., chondroblastoma is composed of cells resembling the precursor of chondrocytes.
Gliomatosis cerebri is a rare growth pattern of some brain tumors, impacting at least three cerebral lobes, mostly with bilateral involvement of the cerebral hemispheres. It can be seen in some types of diffuse glioma, most notably glioblastoma. It consists of infiltrative threads that spread deeply into the brain, making them very difficult to remove with surgery or treat with radiation and is associated with poor prognosis.
The WHOclassification of tumours of the central nervous system is a World Health Organization Blue Book that defines, describes and classifies tumours of the central nervous system (CNS).
Neuro-oncology is the study of brain and spinal cord neoplasms, many of which are very dangerous and life-threatening. Among the malignant brain cancers, gliomas of the brainstem and pons, glioblastoma multiforme, and high-grade astrocytoma/oligodendroglioma are among the worst. In these cases, untreated survival usually amounts to only a few months, and survival with current radiation and chemotherapy treatments may extend that time from around a year to a year and a half, possibly two or more, depending on the patient's condition, immune function, treatments used, and the specific type of malignant brain neoplasm. Surgery may in some cases be curative, but, as a general rule, malignant brain cancers tend to regenerate and emerge from remission easily, especially highly malignant cases. In such cases, the goal is to excise as much of the mass and as much of the tumor margin as possible without endangering vital functions or other important cognitive abilities. The Journal of Neuro-Oncology is the longest continuously published journal in the field and serves as a leading reference to those practicing in the area of neuro-oncology.
Crenolanib besylate is an investigational inhibitor being developed by AROG Pharmaceuticals, LLC. The compound is currently being evaluated for safety and efficacy in clinical trials for various types of cancer, including acute myeloid leukemia (AML), gastrointestinal stromal tumor (GIST), and glioma. Crenolanib is an orally bioavailable benzimidazole that selectively and potently inhibits signaling of wild-type and mutant isoforms of class III receptor tyrosine kinases (RTK) FLT3, PDGFR α, and PDGFR β. Unlike most RTK inhibitors, crenolanib is a type I mutant-specific inhibitor that preferentially binds to phosphorylated active kinases with the ‘DFG in’ conformation motif.
Temozolomide, sold under the brand name Temodar among others, is an anticancer medication used to treat brain tumors such as glioblastoma and anaplastic astrocytoma. It is taken by mouth or via intravenous infusion.
O6-Benzylguanine (O6-BG) is a synthetic derivative of guanine. It is an antineoplastic agent. It exerts its effect by acting as a suicide inhibitor of the enzyme O6-alkylguanine-DNA alkyltransferase which leads to interruption of DNA repair. O6-BG was used clinically in combination with the alkylating agent temozolomide for glioblastoma, however the combination was found to be overly toxic without adding significant benefit.
Diffuse midline glioma, H3 K27-altered (DMG) is a fatal tumour that arises in midline structures of the brain, most commonly the brainstem, thalamus and spinal cord. When located in the pons it is also known as diffuse intrinsic pontine glioma (DIPG).
Zotiraciclib (TG02) is a potent oral spectrum selective kinase inhibitor for the treatment of cancer. It was discovered in Singapore by S*BIO Pte Ltd and falls under the category of small molecule macrocycles. It crosses the blood brain barrier and acts by depleting Myc through the inhibition of cyclin-dependent kinase 9 (CDK9). It is one of a number of CDK inhibitors under investigation; others targeting CDK9 for the treatment of acute myeloid leukemia include alvocidib and atuveciclib. Myc overexpression is a known factor in many cancers, with 80 percent of glioblastomas characterized by this property. Zotiraciclib has been granted orphan drug designation by the US Food and Drug Administration (FDA) and the European Medicines Agency (EMA) for the treatment of gliomas.
Anaplastic oligodendroglioma is a neuroepithelial tumor which is believed to originate from oligodendrocytes, a cell type of the glia. In the World Health Organization (WHO) classification of brain tumors, anaplastic oligodendrogliomas are classified as grade III. In the course of the disease, it can degenerate into highly malignant oligodendroglioma, grade IV. The vast majority of oligodendrogliomas occur sporadically, without a confirmed cause and without inheritance within a family.
Epitopoietic Research Corporation (ERC) is a Belgian Pharmaceutical company that is specialized in the development of ERC1671, a treatment for Glioblastoma multiforme, which is the most aggressive form of brain cancer. In 2019 ERC provided treatment under the US Federal Right-to-try law.
Angiocentric glioma (AG) refers to a rare neuroepithelial tumor when the superficial brain malignant cells enclose the brain vessels, commonly found in children and young adults. Initially identified in 2005 by Wang and his team from the University of Texas, AG was classified as Grade I by 2007 WHO Classification of Tumors of the Central Nervous System due to its benign clinical behavior, low proliferation index, and curative properties. AG primarily affects children and young adults at an average initial diagnosis age of 16 years old. Over 85% AG patients experience intractable seizures since childhood, especially partial epilepsy.
Thalamic gliomas are very rare, deep-seated, generally high-grade glial neoplasms that form in the thalamus, representing 1–5% of all pediatric brain tumors. Because of their difficult to reach position, they are a unique and difficult challenge for neuro-oncologists and neurosurgeons.
References
- ↑ Picart, Thiébaud; Barritault, Marc; Poncet, Delphine; Berner, Lise-Prune; Izquierdo, Cristina; Tabouret, Emeline; Figarella-Branger, Dominique; Idbaïh, Ahmed; Bielle, Franck; Bourg, Véronique; Vandenbos, Fanny Burel; Moyal, Elizabeth Cohen-Jonathan; Uro-Coste, Emmanelle; Guyotat, Jacques; Honnorat, Jérôme (2021). "Characteristics of diffuse hemispheric gliomas, H3 G34-mutant in adults". Neuro-Oncology Advances. 3 (1): vdab061. doi:10.1093/noajnl/vdab061. ISSN 2632-2498. PMC 8156974 . PMID 34056608.
- 1 2 Weller, Michael; van den Bent, Martin; Preusser, Matthias; Le Rhun, Emilie; Tonn, Jörg C.; Minniti, Giuseppe; Bendszus, Martin; Balana, Carmen; Chinot, Olivier; Dirven, Linda; French, Pim; Hegi, Monika E.; Jakola, Asgeir S.; Platten, Michael; Roth, Patrick (March 2021). "EANO guidelines on the diagnosis and treatment of diffuse gliomas of adulthood". Nature Reviews Clinical Oncology. 18 (3): 170–186. doi:10.1038/s41571-020-00447-z. ISSN 1759-4782. PMC 7904519 . PMID 33293629.
- 1 2 Shao, Hanbing; Gong, Jing; Su, Xiaorui; Chen, Ni; Li, Shuang; Yang, Xibiao; Zhang, Simin; Huang, Zhangfeng; Hu, Wei; Gong, Qiyong; Liu, Yaou; Yue, Qiang (2024-03-01). "MRI characteristics of H3 G34-mutant diffuse hemispheric gliomas and possible differentiation from IDH-wild-type glioblastomas in adolescents and young adults". Journal of Neurosurgery. Pediatrics. 33 (3): 236–244. doi:10.3171/2023.10.PEDS23235. ISSN 1933-0715. PMID 38157540.
- ↑ d’Amati, Antonio; Bargiacchi, Lavinia; Rossi, Sabrina; Carai, Andrea; Bertero, Luca; Barresi, Valeria; Errico, Maria Elena; Buccoliero, Anna Maria; Asioli, Sofia; Marucci, Gianluca; Del Baldo, Giada; Mastronuzzi, Angela; Miele, Evelina; D’Antonio, Federica; Schiavello, Elisabetta (2024-03-13). "Pediatric CNS tumors and 2021 WHO classification: what do oncologists need from pathologists?". Frontiers in Molecular Neuroscience. 17. doi: 10.3389/fnmol.2024.1268038 . ISSN 1662-5099. PMC 10966132 . PMID 38544524.
- ↑ Osborn, A. G.; Louis, D. N.; Poussaint, T. Y.; Linscott, L. L.; Salzman, K. L. (2022-07-01). "The 2021 World Health Organization Classification of Tumors of the Central Nervous System: What Neuroradiologists Need to Know". American Journal of Neuroradiology. 43 (7): 928–937. doi:10.3174/ajnr.A7462. ISSN 0195-6108. PMC 9262075 . PMID 35710121.
- ↑ Antonelli, Manila; Poliani, Pietro Luigi (December 2022). "Adult type diffuse gliomas in the new 2021 WHO Classification". Pathologica. 114 (6): 397–409. doi:10.32074/1591-951X-823. ISSN 1591-951X. PMC 9763975 . PMID 36534419.
- ↑ "" Systematic review of diffuse hemispheric glioma, H3 G34-mutant: Outcomes and associated clinical factors "". academic.oup.com. Retrieved 2024-06-09.
- 1 2 Kurokawa, Ryo; Baba, Akira; Kurokawa, Mariko; Pinarbasi, Emile S.; Makise, Naohiro; Ota, Yoshiaki; Kim, John; Srinivasan, Ashok; Moritani, Toshio (January 2022). "Neuroimaging features of diffuse hemispheric glioma, H3 G34-mutant: A case series and systematic review". Journal of Neuroimaging. 32 (1): 17–27. doi:10.1111/jon.12939. hdl: 2027.42/171545 . ISSN 1051-2284. PMID 34632671.