Gliosarcoma

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.[2] 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).[3] Because of a lack of specific and clear diagnostic criteria, the word "gliosarcoma" was frequently used to refer to glial tumours with mesenchymal properties,[4] such as the ability to make collagen and reticulin.[5]

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

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.[6]

They are most commonly present in the temporal lobe[7][8] and frontal lobe.[9]

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.[5] Feigin's early reports components of perivascular sarcomatous and hyperplastic arteries in gliosarcoma offered evidence for the "collision tumor" hypothesis.[10] 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.[11][12][13] 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.[14] In both tumor regions, Reis and colleagues found similar nuclear accumulation of p53, deletion of p16, mutations of PTEN,and amplifications of CDK4.[15] Other scientists then noted that both gliosarcoma components had similar genetic changes and chromosomal abnormalities of the kind often seen in GBM.[16][17]

Clinical characteristics

Gliosarcoma is rare; incidence ranges from 1.8 to 2.8 percent lower than that of GBMs.[18] 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).[18] 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.[19]

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.[20][21] Marked peritumoral edema is a characteristic and frequent hallmark of gliosarcomas observed on MRI.[18]

Metastasis

GBM and other cerebral gliomas rarely metastasize outside the brain. Numerous authors described incidences of metastatic foci that mixed gliomatous and sarcomatous components,[22][23] while others reported metastatic foci that were entirely composed of the sarcomatous component.[24][25][26] Most gliosarcoma extracranial metastases are found in the lung and liver, but there have been reports of metastases elsewhere as well,[27][28][29][30][31] including evidence of intramedullary metastases to the cervical spine.[32]

Treatment

Tumor removal, postoperative radiation treatment, and chemo with nitrosureas, misonidazole, dacarbazine, temozolomide, doxorubicin , vincristine, cisplatin, mithramycin, ametophterin, thalidomide, or irinotecan have all been recorded as treatment options for gliosarcoma[33] and radiotherapy with temozolomide.[34]

Prognosis

PGS has a poor prognosis,[35] a prognosis of median survival of 4 months in untreated individuals.[36]

References
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  2. 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.
  3. 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
  4. Stroebe H (1895) Ueber Entstehung und Bau der Gehirnglioma. Beitr Pathol Anat Allg Pathol 19:405–486
  5. Feigin I, Gross SW (1954) Sarcoma arising in glioblastoma of the brain. Am J Pathol 31:633–653
  6. 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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 This article incorporates public domain material from Dictionary of Cancer Terms. U.S. National Cancer Institute.

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