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Gliosarcoma

Gliosarcoma
Other namesSarcomatous glioblastoma [1]
Micrograph showing a gliosarcoma. Elastic van Gieson's stain.
SpecialtyNeuro-oncology
Usual onsetBetween 40 and 60 years old[2]
PrognosisFive-year survival rate: 5.6%[2]
Frequency~215 new diagnoses per year (United States)[2]

Gliosarcoma is a rare type of glioma, 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.[3] 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).[4] Because of a lack of specific and clear diagnostic criteria, the word "gliosarcoma" was frequently used to refer to glial tumours with mesenchymal properties,[5] such as the ability to make collagen and reticulin.[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]

They most commonly present in the temporal lobe[8][9] and frontal lobe.[10]

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.[6] Feigin's early reports components of perivascular sarcomatous and hyperplastic arteries in gliosarcoma offered evidence for the "collision tumor" hypothesis.[11] Also, Studies demonstrating the sarcomatous component's histological sensitivity to markers of vascular endothelium such factor CD34, von Willebrand factor, and VIII supported this theory.[12][13][14] An alternative view that has recently gained support suggests that both gliosarcoma components have a monoclonal origin, with the sarcomatous component deriving from abnormal differentiation of malignant gliomal mesenchyma. First, gliomatous and sarcomatous components were shown to have similar p53 alterations by Biernat and colleagues.[15] In both tumor regions, Reis and colleagues found similar nuclear accumulation of p53, deletion of p16, mutations of PTEN,and amplifications of CDK4.[16] Other scientists then noted that both gliosarcoma components had similar genetic changes and chromosomal abnormalities of the kind often seen in GBM.[17][18]

Clinical characteristics

Gliosarcoma is rare; incidence ranges from 1.8 to 2.8 percent lower than that of GBMs.[19] PGS affects persons in their 6th to 7th years of life, and it is much more frequent in males than in females (with 1.4-1.8:1 ratio).[19] 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 similarities.[20]

Imaging

On CT imaging, the lesions might show as Well-defined high-density lesion edges and homogeneous enhancement, replicating the meningioma appearance, or as lesions with large necrotic regions and GBM-like heterogeneous contrast enhancement.[21][22] Marked peritumoral edema is a characteristic and frequent hallmark of gliosarcomas observed on MRI.[19]

Metastasis

GBM and other cerebral gliomas rarely metastasize outside the brain. Numerous authors described incidences of metastatic foci that mixed gliomatous and sarcomatous components,[23][24] while others reported metastatic foci that were entirely composed of the sarcomatous component.[25][26][27] Most gliosarcoma extracranial metastases are found in the lung and liver, but there have been reports of metastases elsewhere as well,[28][29][30][31][32] including evidence of intramedullary metastases to the cervical spine.[33]

Treatment

Tumor removal, postoperative radiation treatment, and chemotherapy with nitrosureas, misonidazole, dacarbazine, temozolomide, doxorubicin , vincristine, cisplatin, mithramycin, ametophterin, thalidomide, or irinotecan have all been recorded as treatment options for gliosarcoma[34] and radiotherapy with temozolomide.[35]

Prognosis

PGS has a poor prognosis,[36] a prognosis of median survival of 4 months in untreated individuals.[37] The National Cancer Institute states that the relative five-year survival rate of gliosarcoma is only 5.6%.[2]

References

  1. ^ "Gliosarcoma: Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Archived from the original on 13 July 2019. Retrieved 13 July 2019.
  2. ^ a b c d "Gliosarcoma Diagnosis and Treatment". National Cancer Institute. 17 September 2018. Archived from the original on October 11, 2021. Retrieved March 8, 2023.
  3. ^ 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.
  4. ^ 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
  5. ^ Stroebe H (1895) Ueber Entstehung und Bau der Gehirnglioma. Beitr Pathol Anat Allg Pathol 19:405–486
  6. ^ a b Feigin I, Gross SW (1954) Sarcoma arising in glioblastoma of the brain. Am J Pathol 31:633–653
  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.
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  16. ^ Reis R, Konu-Lebleblicioglu D, Lopes JM, Kleihues P, Ohgaki H (2000) Genetic profile of gliosarcomas. Am J Pathol 156:425–432
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  34. ^ Rodriguez F, Scheithauer BW, Jenkins R, Burger PC, Rudzinskiy P, Vlodavsky E, Schooley A, Landolfi J (2007) Gliosarcoma arising in oligodendroglial tumors (‘‘oligosarcoma’’): a clinicopathologic study. Am J Surg Pathol 31:351–362
  35. ^ Stupp R, Mason WP, Van den Bent MJ, Weller M, Fisher B, Taphoorn MJ et al (2005) Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma. N Engl J Med 352:987–996
  36. ^ 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.
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Public Domain This article incorporates public domain material from Dictionary of Cancer Terms. U.S. National Cancer Institute.

Quelle: https://en.wikipedia.org/wiki/Gliosarcoma