Medicine:Gliosarcoma

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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.[1] 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).[2] Because of a lack of specific and clear diagnostic criteria, the word "gliosarcoma" was frequently used to refer to glial tumours with mesenchymal properties,[3] such as the ability to make collagen and reticulin.[4]

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.[5]

They most commonly present in the temporal lobe[6][7] and frontal lobe.[8]

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] Feigin's early reports components of perivascular sarcomatous and hyperplastic arteries in gliosarcoma offered evidence for the "collision tumor" hypothesis.[9] 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.[10][11][12] 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.[13] In both tumor regions, Reis and colleagues found similar nuclear accumulation of p53, deletion of p16, mutations of PTEN, and amplifications of CDK4.[14] Other scientists then noted that both gliosarcoma components had similar genetic changes and chromosomal abnormalities of the kind often seen in GBM.[15][16]

Clinical characteristics

Gliosarcoma is rare; incidence ranges from 1.8 to 2.8 percent lower than that of GBMs.[17] 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).[17] 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.[18]

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.[19][20] Marked peritumoral edema is a characteristic and frequent hallmark of gliosarcomas observed on MRI.[17]

Metastasis

GBM and other cerebral gliomas rarely metastasize outside the brain. Numerous authors described incidences of metastatic foci that mixed gliomatous and sarcomatous components,[21][22] while others reported metastatic foci that were entirely composed of the sarcomatous component.[23][24][25] Most gliosarcoma extracranial metastases are found in the lung and liver, but there have been reports of metastases elsewhere as well,[26][27][28][29][30] including evidence of intramedullary metastases to the cervical spine.[31]

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[32] and radiotherapy with temozolomide.[33]

Prognosis

PGS has a poor prognosis,[34] a prognosis of median survival of 4 months in untreated individuals.[35] The National Cancer Institute states that the relative five-year survival rate of gliosarcoma is only 5.6%.[36]

References

  1. ↑ "[Cerebral gliosarcoma: clinico-pathologic study of 8 cases]" (in fr). La Tunisie Médicale 88 (3): 142–6. March 2010. PMID 20415184. 
  2. ↑ "The 2007 WHO classification of tumours of the central nervous system". Acta Neuropathol 114 (2): 97–109. August 2007. doi:10.1007/s00401-007-0243-4. PMID 17618441. 
  3. ↑ "Ueber Entstehung und Bau der Gehirnglioma". Beitr Pathol Anat Allg Pathol 19: 405–486. 1895. https://books.google.com/books?id=OB4_AQAAMAAJ&pg=PA405. 
  4. ↑ 4.0 4.1 "Sarcoma arising in glioblastoma of the brain". Am J Pathol 31 (4): 633–53. 1955. PMID 14388124. 
  5. ↑ "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. 
  6. ↑ "Clinical outcome of gliosarcoma compared with glioblastoma multiforme: North Central Cancer Treatment Group results". J Neurosurg 89 (3): 425–30. September 1998. doi:10.3171/jns.1998.89.3.0425. PMID 9724117. 
  7. ↑ "Primary cerebral gliosarcoma: report of 17 cases". Br J Neurosurg 9 (2): 171–8. April 1995. doi:10.1080/02688699550041511. PMID 7632363. 
  8. ↑ "Mixed glioblastoma multiforme and sarcoma. A clinicopathologic study of 26 radiation therapy oncology group cases". Cancer 67 (9): 2342–9. May 1991. doi:10.1002/1097-0142(19910501)67:9<2342::aid-cncr2820670922>3.0.co;2-b. PMID 1849447. 
  9. ↑ "The endothelial hyperplasia of the cerebral blood vessels with brain tumors, and its sarcomatous transformation". Cancer 11 (2): 264–77. 1958. doi:10.1002/1097-0142(195803/04)11:2<264::aid-cncr2820110207>3.0.co;2-d. PMID 13511345. 
  10. ↑ "Immunohistochemical detection of factor VIII/von Willebrand factor in hyperplastic endothelial cells in glioblastoma multiforme and mixed glioma-sarcoma". J Neuropathol Exp Neurol 41 (5): 479–89. September 1982. doi:10.1097/00005072-198209000-00001. PMID 6286891. 
  11. ↑ "Gliosarcomas: histological, immunohistochemical, ultrastructural, and tissue culture studies". Acta Neuropathol 67 (3–4): 201–10. 1985. doi:10.1007/BF00687802. PMID 4050334. 
  12. ↑ "Gliosarcoma with areas of primitive neuroepithelial differentiation and extracranial metastasis". Clin Neuropathol 20 (5): 212–8. 2001. PMID 11594506. 
  13. ↑ "Identical mutations of the p53 tumor suppressor gene in the gliomatous and the sarcomatous components of gliosarcomas suggest a common origin from glial cells". J Neuropathol Exp Neurol 54 (5): 651–6. September 1995. doi:10.1097/00005072-199509000-00006. PMID 7666053. 
  14. ↑ "Genetic profile of gliosarcomas". Am J Pathol 156 (2): 425–32. February 2000. doi:10.1016/S0002-9440(10)64746-3. PMID 10666371. 
  15. ↑ "Comprehensive analysis of genomic alterations in gliosarcoma and its two tissue components". Genes Chromosomes Cancer 34 (4): 416–27. August 2002. doi:10.1002/gcc.10087. PMID 12112531. 
  16. ↑ "The glial and mesenchymal elements of gliosarcomas share similar genetic alterations". J Neuropathol Exp Neurol 55 (9): 973–81. September 1996. doi:10.1097/00005072-199609000-00004. PMID 8800093. 
  17. ↑ 17.0 17.1 17.2 "Gliosarcoma: a clinical study". Radiother Oncol 61 (1): 57–64. October 2001. doi:10.1016/s0167-8140(01)00415-7. PMID 11578729. 
  18. ↑ "Gliosarcoma: report of four cases with immunohistochemical findings". Arq Neuropsiquiatr 62 (3A): 608–12. September 2004. doi:10.1590/s0004-282x2004000400008. PMID 15334217. 
  19. ↑ "Computed tomography of gliosarcoma". AJNR Am J Neuroradiol 6 (4): 527–31. 1985. PMID 3927668. 
  20. ↑ "Cerebral gliosarcomas: correlation of computed tomographic findings, surgical aspect, pathological features, and prognosis". Neurosurgery 26 (2): 261–7. February 1990. doi:10.1227/00006123-199002000-00013. PMID 2308674. 
  21. ↑ "A complex of glioblastoma and spindle-cell sarcoma with pulmonary metastasis". AMA Arch Pathol 66 (4): 536–49. October 1958. PMID 13582394. 
  22. ↑ "Glioblastoma and fibrosarcoma of the brain with extracranial metastases". Cancer 11 (5): 888–94. 1958. doi:10.1002/1097-0142(195809/10)11:5<888::aid-cncr2820110504>3.0.co;2-t. PMID 13585341. 
  23. ↑ "Contiguous glioblastoma multiforme and fibrosarcoma with extracranial metastasis". Cancer 24 (2): 270–6. August 1969. doi:10.1002/1097-0142(196908)24:2<270::aid-cncr2820240210>3.0.co;2-5. PMID 4307749. 
  24. ↑ "October 1998--61 year old male with brain tumor and oral, lung, and palpebral masses". Brain Pathol 9 (2): 421–2. April 1999. PMID 10219754. 
  25. ↑ "Cerebral gliosarcoma, pulmonary adenoid-cystic carcinoma, and pulmonary metastatic gliosarcoma: report of an untreated case". Pathology 16 (2): 217–21. April 1984. doi:10.3109/00313028409059108. PMID 6087260. 
  26. ↑ "Selective peripancreatic sarcoma metastases from primary gliosarcoma. Case report". J Neurosurg 61 (3): 599–601. September 1984. doi:10.3171/jns.1984.61.3.0599. PMID 6747700. 
  27. ↑ "Extraneural metastases in gliosarcoma: a case report and review of the literature". Neurosurgery 17 (3): 413–8. September 1985. doi:10.1227/00006123-198509000-00003. PMID 4047352. 
  28. ↑ "Extracranial spreading of glioblastoma multiforme". Zentralbl Neurochir 41 (1): 57–68. 1980. PMID 6258355. 
  29. ↑ "[Gliosarcoma with multiple extracranial metastases. Case report]" (in Japanese). Neurol Med Chir (Tokyo) 29 (10): 938–43. October 1989. doi:10.2176/nmc.29.938. PMID 2482946. 
  30. ↑ "Extracranial metastasis of glioblastoma with sarcomatous component". Surg Neurol 24 (6): 641–5. December 1985. doi:10.1016/0090-3019(85)90122-3. PMID 2997942. 
  31. ↑ "Gliosarcoma metastatic to the cervical spinal cord: case report and review of the literature". Surg Neurol 54 (5): 373–8; discusiion 378–9. November 2000. doi:10.1016/s0090-3019(00)00315-3. PMID 11165614. 
  32. ↑ "Gliosarcoma arising in oligodendroglial tumors ("oligosarcoma"): a clinicopathologic study". Am J Surg Pathol 31 (3): 351–62. March 2007. doi:10.1097/01.pas.0000213378.94547.ae. PMID 17325476. 
  33. ↑ "Radiotherapy plus concomitant and adjuvant temozolomide for glioblastoma". N Engl J Med 352 (10): 987–96. March 2005. doi:10.1056/NEJMoa043330. PMID 15758009. 
  34. ↑ "Adult gliosarcoma: epidemiology, natural history, and factors associated with outcome". Neuro Oncol 11 (2): 183–91. April 2009. doi:10.1215/15228517-2008-076. PMID 18780813. 
  35. ↑ "Clinical and pathological study of 24 cases of gliosarcoma". J Neurosurg 45 (4): 398–408. October 1976. doi:10.3171/jns.1976.45.4.0398. PMID 956876. 
  36. ↑ Cite error: Invalid <ref> tag; no text was provided for refs named NCI
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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