Brain Cancer
Peter G. Shields in Cancer Risk Assessment, 2005
The identification of the genetical alterations found in astrocytomas led to the recognition that the nonrandom series of genetical changes that take place reflects increase of malignancy and clinical grade (4). Several common chromosomal alterations are observed and lead to changes in the expression of several genes. For example, mutations in the p53 gene (located on chromosome 17p) have been reported in 40% of astrocytic tumors of all grades. These mutations are primarily found in gliomas in young adults and not in supratentorial astrocytic tumors in children (5). Another tumor suppressor gene frequently inactivated in astrocytic neoplasms is cyclin-depen-dent kinase N2 (CDKN2) or p16. The CDKN2 gene is located on chromosome 9p and is inactivated by deletion of both copies of the gene. Loss of CDKN2 occurs rarely in low-grade astrocytomas but frequently in high-grade astrocytomas (6). Deletions of chromosome 10 commonly occur in astrocytic tumors, and there is considerable evidence for the presence of several tumor suppressor genes on chromosome 10 (7). Loss of heterozygosity at 10q23 has been reported to occur in approximately 70% of glioblastomas. Mutated in multiple advanced cancers (MMAC1) or phosphatase tensin homolog, also know as PTEN, is a tumor suppressor gene located on 10q and mutated in 40% of glioblastomas. Because MMAC1 mutations are rarely found in low-grade gliomas, MMAC1 is assumed to play an important role in progression from low-grade to high-grade tumors (8).
Precision medicine for brain gliomas
Debmalya Barh in Precision Medicine in Cancers and Non-Communicable Diseases, 2018
Gliomas are the most common primary intracranial tumors, representing about 80% of malignant brain tumors (Zong et al., 2012; Rajesh et al., 2017). Three different types of gliomas have been described in the brain as astrocytoma, oligodendroglioma, and ependymoma (Izci, 2014). Astrocytoma is the most frequent histological type of glioma and arises from the astrocytes (Figures 4.1 and 4.2) (Zong et al., 2012). About 10% of the gliomas are oligodendrogliomas (Figure 4.3). Mixed gliomas, primarily oligoastrocytomas, account for about 5%–10% of all gliomas. Ependymomas arise from the ependymal cells, which lie in the ventricular system and the central canal of the spinal cord. It is relatively rare in adults, accounting for 2%–3% of primary brain tumors, but it is frequent in children. Today, biological markers help pathologists separate oligodendrogliomas from other types of gliomas. Glioma has a poor prognosis (Perry and Wesseling, 2016). Although relatively rare, it causes significant mortality and morbidity. Glioblastoma is the most common and malignant histological type of glioma (approximately 45% of all gliomas) (Akay et al., 2002; Zong et al., 2012). It may be solitary or multicentric in the brain (Izci et al., 2005). Glioblastoma may present with different clinical and radiological characteristics and has a 5-year relative survival of about 5% (Akay et al., 2002; Izci et al., 2005; Bondy et al., 2008; de Robles et al., 2015).
Pediatric Oncology
Pat Price, Karol Sikora in Treatment of Cancer, 2020
Complete surgical resection of a grade II astrocytoma has a good prognosis, and no further therapy is required. Patients with residual disease have a significant likelihood of recurrence, and may show grade progression; for this reason, a more aggressive approach is often taken, although a single strategy is not agreed. The authors’ own practice is to recommend focal radiotherapy (54 Gy) and adjuvant PCV chemotherapy as reported by Buckner et al.179
The Expressional Pattern of Invasion-Related Extracellular Matrix Molecules in CNS Tumors
Published in Cancer Investigation, 2018
József Virga, László Bognár, Tibor Hortobágyi, Éva Csősz, Gergő Kalló, Gábor Zahuczki, László Steiner, Gábor Hutóczki, Judit Reményi-Puskár, Almos Klekner
Astrocytomas are central nervous system neoplasms of high interest because of their relatively high frequency (63% of all intracranial malignancies is astrocytoma). Furthermore, peritumoral infiltration, a feature which basically prevents total surgical resection, is typical even in low-grade astrocytomas (1–5). WHO divides astrocytomas into four grades, based upon histopathological features, namely grade I pilocytic astrocytoma, grade II diffuse astrocytoma, grade III anaplastic astrocytoma, and grade IV glioblastoma (6). WHO grades are not only for classification but they carry prognostic and therapeutic relevancies as well. WHO grade II astrocytoma (low-grade astrocytoma) is a clinically semi-benign, slow-growing entity, median overall survival varies between 6–8 years (7–11). Complete surgical resection is essential for curing the patient but is hindered by the infiltrative growth of the tumor tissue (2). Previous research has proved that the extracellular matrix (ECM) in astrocytomas differs from those of non-tumor brain both in quantity and in quality (12–15). Various components of the ECM, such as cell-surface receptors and their ligands, ECM macromolecules, and enzymes have been shown to play a pivotal role in the invasion of cancer cells (12, 15–19). It is currently not entirely understood, though, how much a certain molecule affects the invasive character of low-grade astrocytomas.
Expression and clinicopathological significance of Nck1 in human astrocytoma progression
Published in International Journal of Neuroscience, 2019
Ravindra Pramod Deshpande, Manas Panigrahi, Chandra Sekhar Y.B.V.K, Phanithi Prakash Babu
Astrocytoma represents the most common among the central nervous system malignancies [1]. World Health Organization (WHO) has classified astrocytoma in four grades based on histopathological features as necrosis, endothelial proliferation [2, 3]; however, molecular profiling has contributed substantially for understanding of molecular biology of glial tumors [4–6]. Standard treatment consists of surgical resection, chemotherapy, usually with temozolomide and radiotherapy [7]. Despite of aggressive chemo and radiotherapy, only 33% of diagnosed patients are reported to survive beyond 5 years [8]. Median survival age of patients diagnosed with progressive astrocytoma, i.e. glioblastoma still remains 12–15 months [9]. We previously reported lower median age of patients’ suffering from glioblastoma with high incidence in middle age group in Indian population [10]. Present situation highlights the urgent need to understand astrocytoma biology for possible therapeutic interventions.
Fahr’s disease associated with anaplastic ependymoma: a case report and review of the literature
Published in British Journal of Neurosurgery, 2023
Abdussamet Batur, Ömer Faruk Topaloğlu
In the literature, the association of Fahr’s disease and brain tumor is not frequently defined. In a recent study by Lin et al. the fifth case of brain tumor associated with Fahr’s disease was presented and all were reported as low-grade glioma.3 Also, Jaworski et al. described coexistence of Fahr’s syndrome with low-grade brain tumors such as astrocytoma and pineal body gangliocytoma.19 Histopathology studies in patients with Fahr’s disease have noted extensive calcification accompanied by hypertrophy and hyperplasia of astrocytes. Furthermore, several genetic alterations such as platelet-derived growth factor β polypeptide gene, have also been demonstrated to be involved in the development of glioma.3 For all that, whether the occurrence of astrocytoma could be linked etiologically to long-standing astroglial proliferation remains speculative. According to our knowledge, the study showing the association of anaplastic ependymoma and Fahr’s disease in the literature has not been previously presented. However, we do not have sufficient data neither in the literature to prove that the cause of the tumor is Fahr’s disease. There are publications stating that the disease is associated with astrocyte proliferation, but we could not reach data to show its relationship with ependymia.