Medicine:Gliosarcoma

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Gliosarcoma
Other namesSarcomatous glioblastoma [1]
Gliosarcoma Histopathology 200x EVG.jpg
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". https://rarediseases.info.nih.gov/diseases/5653/index. 
  2. ↑ 2.0 2.1 2.2 2.3 "Gliosarcoma Diagnosis and Treatment". https://www.cancer.gov/rare-brain-spine-tumor/tumors/gliosarcoma. 
  3. ↑ "[Cerebral gliosarcoma: clinico-pathologic study of 8 cases]" (in fr). La Tunisie Médicale 88 (3): 142–146. March 2010. 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. ↑ 6.0 6.1 Feigin I, Gross SW (1954) Sarcoma arising in glioblastoma of the brain. Am J Pathol 31:633–653
  7. ↑ "Gliosarcoma with multiple extracranial metastases: case report and review of the literature". Journal of Neuro-Oncology 83 (1): 39–46. May 2007. doi:10.1007/s11060-006-9295-x. PMID 17171442. 
  8. ↑ Galanis E, Buckner JC, Dinapoli RP, Scheithauer BW, Jenkins RB, Wang CH, O’Fallon JR, Farr G Jr (1998) Clinical outcome of gliosarcoma compared with glioblastoma multiforme: north central cancer treatment group results. J Neurosurg 89:425–430
  9. ↑ Parekh H, O’Donovan DG, Sharma RR, Keogh AJ (1995) Primary cerebral gliosarcoma: report of 17 cases. Br J Neurosurg 9:171–178
  10. ↑ Meis J, Martz KL, Nelson JS (1990) Mixed glioblastoma multiforme and sarcoma: a clinicopathologic study of 26 radiation therapy oncology group cases. Cancer 67:2342–2349
  11. ↑ 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
  12. ↑ 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
  13. ↑ Slowik F, Jellinger K, Gaszo L, Fischer J (1985) Gliosarcomas: histological, immunohistochemical, ultrastructural, and tissue culture studies. Acta Neuropathol 67:201–210
  14. ↑ 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
  15. ↑ 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
  16. ↑ Reis R, Konu-Lebleblicioglu D, Lopes JM, Kleihues P, Ohgaki H (2000) Genetic profile of gliosarcomas. Am J Pathol 156:425–432
  17. ↑ 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
  18. ↑ 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
  19. ↑ 19.0 19.1 19.2 Lutterbach J, Guttenberger R, Pagenstecher A (2001) Gliosarcoma: a clinical study. Radiother Oncol 61:57–64
  20. ↑ 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
  21. ↑ Lee Y, Castillo M, Nauert C, Moser RP (1985) Computed tomography of gliosarcoma. Am J Neuroradiol 4:527–531
  22. ↑ Maiuri F, Stella L, Benvenuti D, Giamundo A, Pettinato G (1990) Cerebral gliosarcomas: correlation of computed tomographic findings, surgical aspect, pathological features, and prognosis. Neurosurgery 26:261–267
  23. ↑ Ehrenreich T, Devlin JF (1958) A complex of glioblastoma and spindle-cell sarcoma with pulmonary metastases. Arch Pathol 66:536–549
  24. ↑ Garret R (1958) Glioblastoma and fibrosarcoma of the brain with extracranial metastases. Cancer 11:888–894
  25. ↑ Smith D, Hardman JM, Earle KM (1969) Glioblastoma multiforme and fibrosarcoma with extracranial metastasis. Cancer 24:270–276
  26. ↑ Gjedrum L, Bojsen-Moller M (1999) 61-year old male with brain tumor and oral, lung, and palpebral masses. Brain Pathol 9:421–422
  27. ↑ Ojeda V, Sterrett GF (1984) Cerebral gliosarcoma, pulmonary adenoid-cystic carcinoma, and pulmonary metastatic gliosarcoma: report of an untreated case. Pathology 16:217–221
  28. ↑ Weaver D, Vandenberg S, Park TS, Jane JA (1984) Selective peripancreatic sarcoma metastases from primary gliosarcoma.J Neurosurg 61:599–601
  29. ↑ Cerame M, Guthikonda M, Kohli CM (1985) Extraneural metastases in gliosarcoma: a case report and review of literature. Neurosurgery 17:413–418
  30. ↑ Slowik F, Balogh I (1980) Extracranial spreading of glioblastoma multiforme. Zentralbl Neurochir 41:57–68
  31. ↑ Matsuyama J, Mori T, Hori S, Nakano T, Yamada A (1989) Gliosarcoma with multiple extracranial metastases. Case report. Neurol Med Chir (Tokyo) 29:938–943
  32. ↑ Yokoyama H, Ono H, Mori K, Kishikawa M, Kihara M (1985) Extracranial metastasis of glioblastoma with sarcomatous component. Surg Neurol 24:641–645
  33. ↑ Witwer B, Salamat MS, Resnick DK (2000) Gliosarcoma metastatic to the cervical spine cord: case report and review of the literature. Surg Neurol 54:373–379
  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.
  37. ↑ Morantz R, Feigin I, Ransohoff J (1976) Clinical and pathological study of 24 cases of gliosarcoma. J Neurosurg 45:398–408

External links

Classification
External resources

 This article incorporates public domain material from the U.S. National Cancer Institute document "Dictionary of Cancer Terms".



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