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Gliosarcoma

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Gliosarcoma
Other namesSarcomatous glioblastoma [1]
Micrograph showing a gliosarcoma. Elastic van Gieson's stain.
SpecialtyNeuro-oncology

Gliosarcoma is a rare clinicopathologic subtype of glioblastoma, has a poor prognosis[2]. Stroebe first identified gliosarcoma as a brain tumor with both glial and mesenchymal components in 1895[3]. However, due to a lack of precise and consistent diagnostic criteria, the term "gliosarcoma" was often used to refer to tumors of glial origin that had mesenchymal characteristics, such as the ability to produce reticulin and collagen[4]. A cancer of the brain that comes from glial, or supportive, brain cells, as opposed to the neural brain cells. Gliosarcoma is a malignant cancer, and is defined as a glioblastoma consisting of gliomatous and sarcomatous components.[5]Primary gliosarcoma (PGS) is classified as a grade IV tumor and a subtype of glioblastoma multiforme in the 2007 World Health Organization classification system (GBM)[6]

It is estimated that approximately 2.1% of all glioblastomas are gliosarcomas. Although most gliomas rarely show metastases outside the cerebrum, gliosarcomas have a propensity to do so, most commonly spreading through the blood to the lungs, and also liver and lymph nodes.[7]

Gliosarcomas have an epidemiology similar to that of glioblastomas, with the average age of onset being 54 years, and males being affected twice as often as females. They are most commonly present in the temporal lobe.[citation needed]

Pathogenesis

Early reports claimed that the hyperplastic blood vessels that are frequently present in high grade gliomas underwent neoplastic change to become the sarcomatous components[4]. Early descriptions by Feigin of hyperplastic arteries and perivascular arrangement of sarcomatous components in gliosarcoma provided support for this "collision tumor" theory[8]. Studies demonstrating the sarcomatous component's histological sensitivity to vascular endothelial markers such factor VIII, von Willebrand factor, and CD34 also supported this theory[9][10][11]. A alternative view that has recently gained support suggests that both gliosarcoma components have a monoclonal origin, with the sarcomatous component deriving from abnormal mesenchymal differentiation of the malignant glioma. First, gliomatous and sarcomatous components were shown to have similar p53 alterations by Biernat and colleagues[12]. In both tumor regions, Reis and colleagues found similar PTEN mutations, p53 nuclear accumulation, p16 deletion, and CDK4 amplifications[13]. Other scientists then noted that both gliosarcoma components had similar genetic changes and chromosomal abnormalities of the kind often seen in GBM[14][15].

Clinical characteristics

Gliosarcoma is rare incidence ranges from 1.8 to 2.8 percent lower than that of GBMs[16]. PGS affects people in their sixth to seventh decade of life, and, like other glial-based cancers, it is much more common in men than in women (M:F ratio, 1.4–1.8:1)[16]. Depending on where the tumor is located, the reported presenting signs and symptoms, such as aphasia, headaches, hemiparesis, seizures, and cognitive loss, are similar with those of a fast developing intracranial tumor. Many researchers have come to the conclusion that these tumors are clinically identical to GBM due to their clinical resemblances[17].

References

  1. ^ "Gliosarcoma: Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 13 July 2019.
  2. ^ Kozak KR, Mahadevan A, Moody JS. Adult gliosarcoma: epidemiology, natural history and factors associated with outcome. April. 2009;Cancer:183–191. doi:10.1215/15228517.
  3. ^ Stroebe H (1895) Ueber Entstehung und Bau der Gehirnglioma. Beitr Pathol Anat Allg Pathol 19:405–486
  4. ^ a b Feigin I, Gross SW (1954) Sarcoma arising in glioblastoma of the brain. Am J Pathol 31:633–653
  5. ^ Ayadi L, Charfi S, Khabir A, Kalle R, Sellami A, Makni S, et al. (March 2010). "[Cerebral gliosarcoma: clinico-pathologic study of 8 cases]". La Tunisie Médicale (in French). 88 (3): 142–146. PMID 20415184.
  6. ^ Louis D, Ohgaki H, Wiestler OD, Cavenee WK, Burger PC, Jouvet A, Scheithauer BW, Kleihues P (2007) The 2007 WHO classification of tumours of the central nervous system. Acta Neuropathol 114:97–109
  7. ^ Beaumont TL, Kupsky WJ, Barger GR, Sloan AE (May 2007). "Gliosarcoma with multiple extracranial metastases: case report and review of the literature". Journal of Neuro-Oncology. 83 (1): 39–46. doi:10.1007/s11060-006-9295-x. PMID 17171442. S2CID 13171064.
  8. ^ Feigin I, Allen LB, Lipkin L, Gross SW (1958) The endothelial hyperplasia of cerebral blood vessels with brain tumors, and its sarcomatous transformation. Cancer 2:264–277
  9. ^ McComb R, Jones TR, Pizzo SV, Bigner DD (1982) Immunohistochemical detection of factor VIII/von Willebrand factor in hyperplastic endothelial cells in glioblastoma multiforme and mixed glioma-sarcoma. J Neuropathol Exp Neurol 41:479–489
  10. ^ Slowik F, Jellinger K, Gaszo L, Fischer J (1985) Gliosarcomas: histological, immunohistochemical, ultrastructural, and tissue culture studies. Acta Neuropathol 67:201–210
  11. ^ Wharton S, Whittle IR, Collie DA, Bell HS, Ironside JW (2001) Gliosarcoma with areas of primitive neuroepithelial differentiation and extracranial metastasis. Clin Neuropathol 20:212–218
  12. ^ Biernat W, Aguzzi A, Sure U, Grant JW, Kleihues P, Hegi ME (1995) Identical mutations of the p53 tumor suppressor gene in the gliomatous and the sarcomatous components of gliosarcomas suggest a common or
  13. ^ Reis R, Konu-Lebleblicioglu D, Lopes JM, Kleihues P, Ohgaki H (2000) Genetic profile of gliosarcomas. Am J Pathol 156:425–432
  14. ^ Actor B, Cobbers JM, Buschges R, Wolter M, Knobbe CB, Lichter P, Reifenberger G, Weber RG (2002) Comprehensive analysis of genomic alterations in gliosarcoma and its two tissue components. Genes Chromosomes Cancer 34:416–427
  15. ^ Boerman R, Anderl K, Herath J, Borell T, Johnson N, SchaefferKlein J, Kirchof A, Raap AK, Scheithauer BW, Jenkins RB (1996) The glilal and mesenchymal elements of gliosarcomas share similar genetic alterations. J Neuropathol Exp Neurol 55:973–981
  16. ^ a b Lutterbach J, Guttenberger R, Pagenstecher A (2001) Gliosarcoma: a clinical study. Radiother Oncol 61:57–64
  17. ^ Machuca T, Prevedello DM, Pope LZ, Haratz SS, Araujo JC, Torres LF (2004) Gliosarcoma: report of four cases with immunohistochemical findings. Arq Neuropsiquiatr 62:608–612

Public Domain This article incorporates public domain material from Dictionary of Cancer Terms. U.S. National Cancer Institute.

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