Astrocytoma natural history: Difference between revisions

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{{CMG}}; {{AE}} {{Fs}}, {{Ammu}}
{{CMG}}; {{AE}} {{Fs}}, {{Ammu}}
==Overview==
==Overview==
If left untreated, eventually 100% of patients with [[Low grade astrocytoma|low grade astrocytomas]] will growth rapidly similar to [[high grade astrocytoma]] tumors and 100% of patients with [[high grade astrocytoma]] will become [[symptomatic]] and deteriorate. Astrocytoma being a space occupying [[lesion]] can have following complications depending on the location of the [[tumor]]: [[Increased intracranial pressure]], [[cognitive]] dysfunction, [[Emotional disorders|emotional disturbances]], behavioral complications, [[Visual disturbance|visual defects]] and [[Muscle weakness]]. [[Low grade astrocytoma|Low-grade astrocytomas]] (grade I [<nowiki/>[[Pilocytic astrocytoma|pilocytic]]] and grade II) have a relatively favorable [[prognosis]], particularly for circumscribed, grade I lesions where complete excision may be possible. [[High grade astrocytoma|High-grade astrocytomas]] generally carry a poor [[prognosis]] in younger patients.
If left untreated, all of patients with [[Low grade astrocytoma|low grade astrocytomas]] will have a rapid growth of the tumor similar to [[high grade astrocytoma]] and all of the patients with [[high grade astrocytoma]] will become [[symptomatic]] and deteriorate. Astrocytoma being a space occupying [[lesion]] may have following complications depending on the location of the [[tumor]] including [[increased intracranial pressure]], [[cognitive]] dysfunction, [[Emotional disorders|emotional disturbances]], behavioral complications, [[Visual disturbance|visual defects]] and [[muscle weakness]]. [[Low grade astrocytoma|Low-grade astrocytomas]] (grade I [<nowiki/>[[Pilocytic astrocytoma|pilocytic]]] and grade II) have a relatively favorable [[prognosis]], particularly for circumscribed, grade I lesions where complete excision may be possible. [[High grade astrocytoma|High-grade astrocytomas]] generally carry a poor [[prognosis]] in younger patients.
 




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===Natural History===
===Natural History===
* [[Low grade astrocytoma]]:<ref name="pmid1586143">{{cite journal |vauthors=Recht LD, Lew R, Smith TW |title=Suspected low-grade glioma: is deferring treatment safe? |journal=Ann. Neurol. |volume=31 |issue=4 |pages=431–6 |date=April 1992 |pmid=1586143 |doi=10.1002/ana.410310413 |url=}}</ref><ref name="pmid10751254">{{cite journal |vauthors=Olson JD, Riedel E, DeAngelis LM |title=Long-term outcome of low-grade oligodendroglioma and mixed glioma |journal=Neurology |volume=54 |issue=7 |pages=1442–8 |date=April 2000 |pmid=10751254 |doi= |url=}}</ref><ref name="pmid19395201">{{cite journal |vauthors=Bauman G, Fisher B, Watling C, Cairncross JG, Macdonald D |title=Adult supratentorial low-grade glioma: long-term experience at a single institution |journal=Int. J. Radiat. Oncol. Biol. Phys. |volume=75 |issue=5 |pages=1401–7 |date=December 2009 |pmid=19395201 |doi=10.1016/j.ijrobp.2009.01.010 |url=}}</ref><ref name="pmid16470608">{{cite journal |vauthors=Claus EB, Black PM |title=Survival rates and patterns of care for patients diagnosed with supratentorial low-grade gliomas: data from the SEER program, 1973-2001 |journal=Cancer |volume=106 |issue=6 |pages=1358–63 |date=March 2006 |pmid=16470608 |doi=10.1002/cncr.21733 |url=}}</ref>
* [[Low grade astrocytoma]]:<ref name="pmid1586143">{{cite journal |vauthors=Recht LD, Lew R, Smith TW |title=Suspected low-grade glioma: is deferring treatment safe? |journal=Ann. Neurol. |volume=31 |issue=4 |pages=431–6 |date=April 1992 |pmid=1586143 |doi=10.1002/ana.410310413 |url=}}</ref><ref name="pmid10751254">{{cite journal |vauthors=Olson JD, Riedel E, DeAngelis LM |title=Long-term outcome of low-grade oligodendroglioma and mixed glioma |journal=Neurology |volume=54 |issue=7 |pages=1442–8 |date=April 2000 |pmid=10751254 |doi= |url=}}</ref><ref name="pmid19395201">{{cite journal |vauthors=Bauman G, Fisher B, Watling C, Cairncross JG, Macdonald D |title=Adult supratentorial low-grade glioma: long-term experience at a single institution |journal=Int. J. Radiat. Oncol. Biol. Phys. |volume=75 |issue=5 |pages=1401–7 |date=December 2009 |pmid=19395201 |doi=10.1016/j.ijrobp.2009.01.010 |url=}}</ref><ref name="pmid16470608">{{cite journal |vauthors=Claus EB, Black PM |title=Survival rates and patterns of care for patients diagnosed with supratentorial low-grade gliomas: data from the SEER program, 1973-2001 |journal=Cancer |volume=106 |issue=6 |pages=1358–63 |date=March 2006 |pmid=16470608 |doi=10.1002/cncr.21733 |url=}}</ref>
** The natural history of all [[Low grade astrocytoma|low grade astrocytomas]] are not the same.
** The natural history of all [[Low grade astrocytoma|low grade astrocytomas]] is not the same.
** Most of the patients experience 5 to 7 years of symptom stability.
** Most of the patients experience almost no progression of symptoms for initial 5 to 7 years.
** If left untreated, eventually 100% of patients with [[Low grade astrocytoma|low grade astrocytomas]] will growth rapidly similar to [[high grade astrocytoma]] tumors.
** If left untreated, all the patients with [[Low grade astrocytoma|low grade astrocytomas]] will have rapid growth of tumor similar to [[high grade astrocytoma]].
* [[High grade astrocytoma]]:
* [[High grade astrocytoma]]:
** If left untreated, 100% of patients will become symptomatic and deteriorate.  
** If left untreated, all of the patients become symptomatic and deteriorate.  
** Recurrence is more in [[high grade astrocytoma]] compared to [[low grade astrocytoma]].<ref name="pmid8727811">{{cite journal| author=Piepmeier J, Christopher S, Spencer D, Byrne T, Kim J, Knisel JP et al.| title=Variations in the natural history and survival of patients with supratentorial low-grade astrocytomas. | journal=Neurosurgery | year= 1996 | volume= 38 | issue= 5 | pages= 872-8; discussion 878-9 | pmid=8727811 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8727811  }} </ref>
** Recurrence is more common in [[high grade astrocytoma]] compared to [[low grade astrocytoma]].<ref name="pmid8727811">{{cite journal| author=Piepmeier J, Christopher S, Spencer D, Byrne T, Kim J, Knisel JP et al.| title=Variations in the natural history and survival of patients with supratentorial low-grade astrocytomas. | journal=Neurosurgery | year= 1996 | volume= 38 | issue= 5 | pages= 872-8; discussion 878-9 | pmid=8727811 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8727811  }} </ref>
===Complications===
===Complications===
* Astrocytoma being a space occupying [[lesion]] can have following complications depending on the location of the [[tumor]]:<ref name="pmid20026498">{{cite journal |vauthors=Ansell P, Johnston T, Simpson J, Crouch S, Roman E, Picton S |title=Brain tumor signs and symptoms: analysis of primary health care records from the UKCCS |journal=Pediatrics |volume=125 |issue=1 |pages=112–9 |date=January 2010 |pmid=20026498 |doi=10.1542/peds.2009-0254 |url=}}</ref><ref name="pmid16547083">{{cite journal |vauthors=Wilne SH, Ferris RC, Nathwani A, Kennedy CR |title=The presenting features of brain tumours: a review of 200 cases |journal=Arch. Dis. Child. |volume=91 |issue=6 |pages=502–6 |date=June 2006 |pmid=16547083 |pmc=2082784 |doi=10.1136/adc.2005.090266 |url=}}</ref><ref name="pmid17644483">{{cite journal |vauthors=Wilne S, Collier J, Kennedy C, Koller K, Grundy R, Walker D |title=Presentation of childhood CNS tumours: a systematic review and meta-analysis |journal=Lancet Oncol. |volume=8 |issue=8 |pages=685–95 |date=August 2007 |pmid=17644483 |doi=10.1016/S1470-2045(07)70207-3 |url=}}</ref>
* Astrocytoma being a space occupying [[lesion]] may have following complications depending on the location of the [[tumor]]:<ref name="pmid20026498">{{cite journal |vauthors=Ansell P, Johnston T, Simpson J, Crouch S, Roman E, Picton S |title=Brain tumor signs and symptoms: analysis of primary health care records from the UKCCS |journal=Pediatrics |volume=125 |issue=1 |pages=112–9 |date=January 2010 |pmid=20026498 |doi=10.1542/peds.2009-0254 |url=}}</ref><ref name="pmid16547083">{{cite journal |vauthors=Wilne SH, Ferris RC, Nathwani A, Kennedy CR |title=The presenting features of brain tumours: a review of 200 cases |journal=Arch. Dis. Child. |volume=91 |issue=6 |pages=502–6 |date=June 2006 |pmid=16547083 |pmc=2082784 |doi=10.1136/adc.2005.090266 |url=}}</ref><ref name="pmid17644483">{{cite journal |vauthors=Wilne S, Collier J, Kennedy C, Koller K, Grundy R, Walker D |title=Presentation of childhood CNS tumours: a systematic review and meta-analysis |journal=Lancet Oncol. |volume=8 |issue=8 |pages=685–95 |date=August 2007 |pmid=17644483 |doi=10.1016/S1470-2045(07)70207-3 |url=}}</ref>
:* [[Increased intracranial pressure]]
:* [[Increased intracranial pressure]]
:* Cognitive dysfunction
:* Cognitive dysfunction
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:* Local neurological deficit
:* Local neurological deficit
===Prognosis===
===Prognosis===
[[Low grade astrocytoma|Low-grade astrocytomas]]:
 
* [[Low grade astrocytoma|Low-grade astrocytomas]] (grade I [<nowiki/>[[Pilocytic astrocytoma|pilocytic]]] and grade II) have a relatively favorable [[prognosis]], particularly for circumscribed, grade I lesions where complete excision may be possible.<ref name="pmid7969301">{{cite journal| author=Pollack IF| title=Brain tumors in children. | journal=N Engl J Med | year= 1994 | volume= 331 | issue= 22 | pages= 1500-7 | pmid=7969301 | doi=10.1056/NEJM199412013312207 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7969301  }} </ref><ref name="pmid19322538">{{cite journal| author=Pfister S, Witt O| title=Pediatric gliomas. | journal=Recent Results Cancer Res | year= 2009 | volume= 171 | issue=  | pages= 67-81 | pmid=19322538 | doi=10.1007/978-3-540-31206-2_4 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19322538  }} </ref><ref name="pmid18386785">{{cite journal| author=Fisher PG, Tihan T, Goldthwaite PT, Wharam MD, Carson BS, Weingart JD et al.| title=Outcome analysis of childhood low-grade astrocytomas. | journal=Pediatr Blood Cancer | year= 2008 | volume= 51 | issue= 2 | pages= 245-50 | pmid=18386785 | doi=10.1002/pbc.21563 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18386785  }} </ref><ref name="pmid24482038">{{cite journal| author=Bandopadhayay P, Bergthold G, London WB, Goumnerova LC, Morales La Madrid A, Marcus KJ et al.| title=Long-term outcome of 4,040 children diagnosed with pediatric low-grade gliomas: an analysis of the Surveillance Epidemiology and End Results (SEER) database. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 7 | pages= 1173-9 | pmid=24482038 | doi=10.1002/pbc.24958 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24482038  }} </ref>  
==== Low-grade astrocytomas ====
* [[Tumor]] spread, when it occurs, is usually by contiguous extension; dissemination to other [[CNS]] sites is uncommon, but does occur.<ref name="pmid21319282">{{cite journal| author=von Hornstein S, Kortmann RD, Pietsch T, Emser A, Warmuth-Metz M, Soerensen N et al.| title=Impact of chemotherapy on disseminated low-grade glioma in children and adolescents: report from the HIT-LGG 1996 trial. | journal=Pediatr Blood Cancer | year= 2011 | volume= 56 | issue= 7 | pages= 1046-54 | pmid=21319282 | doi=10.1002/pbc.23006 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21319282  }} </ref><ref name="pmid22401918">{{cite journal| author=Mazloom A, Hodges JC, Teh BS, Chintagumpala M, Paulino AC| title=Outcome of patients with pilocytic astrocytoma and leptomeningeal dissemination. | journal=Int J Radiat Oncol Biol Phys | year= 2012 | volume= 84 | issue= 2 | pages= 350-4 | pmid=22401918 | doi=10.1016/j.ijrobp.2011.12.044 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22401918  }} </ref>  
* [[Low grade astrocytoma|Low-grade astrocytomas]] (grade I [<nowiki/>[[Pilocytic astrocytoma|pilocytic]]] and grade II) have a relatively favorable [[prognosis]], particularly for circumscribed, grade I lesions where complete excision is possible.<ref name="pmid7969301">{{cite journal| author=Pollack IF| title=Brain tumors in children. | journal=N Engl J Med | year= 1994 | volume= 331 | issue= 22 | pages= 1500-7 | pmid=7969301 | doi=10.1056/NEJM199412013312207 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=7969301  }} </ref><ref name="pmid19322538">{{cite journal| author=Pfister S, Witt O| title=Pediatric gliomas. | journal=Recent Results Cancer Res | year= 2009 | volume= 171 | issue=  | pages= 67-81 | pmid=19322538 | doi=10.1007/978-3-540-31206-2_4 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=19322538  }} </ref><ref name="pmid18386785">{{cite journal| author=Fisher PG, Tihan T, Goldthwaite PT, Wharam MD, Carson BS, Weingart JD et al.| title=Outcome analysis of childhood low-grade astrocytomas. | journal=Pediatr Blood Cancer | year= 2008 | volume= 51 | issue= 2 | pages= 245-50 | pmid=18386785 | doi=10.1002/pbc.21563 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=18386785  }} </ref><ref name="pmid24482038">{{cite journal| author=Bandopadhayay P, Bergthold G, London WB, Goumnerova LC, Morales La Madrid A, Marcus KJ et al.| title=Long-term outcome of 4,040 children diagnosed with pediatric low-grade gliomas: an analysis of the Surveillance Epidemiology and End Results (SEER) database. | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 7 | pages= 1173-9 | pmid=24482038 | doi=10.1002/pbc.24958 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24482038  }} </ref>  
* Although [[metastasis]] is uncommon, [[tumor]]s may be of multi-focal origin, especially when associated with [[NF1]].
* [[Tumor]] spread, when it occurs, is usually by contiguous extension; dissemination to other [[CNS]] sites may occur, although uncommon.<ref name="pmid21319282">{{cite journal| author=von Hornstein S, Kortmann RD, Pietsch T, Emser A, Warmuth-Metz M, Soerensen N et al.| title=Impact of chemotherapy on disseminated low-grade glioma in children and adolescents: report from the HIT-LGG 1996 trial. | journal=Pediatr Blood Cancer | year= 2011 | volume= 56 | issue= 7 | pages= 1046-54 | pmid=21319282 | doi=10.1002/pbc.23006 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21319282  }} </ref><ref name="pmid22401918">{{cite journal| author=Mazloom A, Hodges JC, Teh BS, Chintagumpala M, Paulino AC| title=Outcome of patients with pilocytic astrocytoma and leptomeningeal dissemination. | journal=Int J Radiat Oncol Biol Phys | year= 2012 | volume= 84 | issue= 2 | pages= 350-4 | pmid=22401918 | doi=10.1016/j.ijrobp.2011.12.044 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22401918  }} </ref>  
* Unfavorable prognostic features for childhood [[Low grade astrocytoma|low-grade astrocytomas]] include the following:<ref name="pmid20861086">{{cite journal| author=Stokland T, Liu JF, Ironside JW, Ellison DW, Taylor R, Robinson KJ et al.| title=A multivariate analysis of factors determining tumor progression in childhood low-grade glioma: a population-based cohort study (CCLG CNS9702). | journal=Neuro Oncol | year= 2010 | volume= 12 | issue= 12 | pages= 1257-68 | pmid=20861086 | doi=10.1093/neuonc/noq092 | pmc=PMC3018938 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20861086  }} </ref><ref name="pmid24039013">{{cite journal| author=Mirow C, Pietsch T, Berkefeld S, Kwiecien R, Warmuth-Metz M, Falkenstein F et al.| title=Children <1 year show an inferior outcome when treated according to the traditional LGG treatment strategy: a report from the German multicenter trial HIT-LGG 1996 for children with low grade glioma (LGG). | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 3 | pages= 457-63 | pmid=24039013 | doi=10.1002/pbc.24729 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24039013  }} </ref>
* Although, [[metastasis]] is uncommon, [[tumor]]s may be of multi-focal origin, especially when associated with [[neurofibromatosis 1]] (NF1).
* Unfavorable prognostic features for childhood [[Low grade astrocytoma|low-grade astrocytomas]] include:<ref name="pmid20861086">{{cite journal| author=Stokland T, Liu JF, Ironside JW, Ellison DW, Taylor R, Robinson KJ et al.| title=A multivariate analysis of factors determining tumor progression in childhood low-grade glioma: a population-based cohort study (CCLG CNS9702). | journal=Neuro Oncol | year= 2010 | volume= 12 | issue= 12 | pages= 1257-68 | pmid=20861086 | doi=10.1093/neuonc/noq092 | pmc=PMC3018938 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=20861086  }} </ref><ref name="pmid24039013">{{cite journal| author=Mirow C, Pietsch T, Berkefeld S, Kwiecien R, Warmuth-Metz M, Falkenstein F et al.| title=Children <1 year show an inferior outcome when treated according to the traditional LGG treatment strategy: a report from the German multicenter trial HIT-LGG 1996 for children with low grade glioma (LGG). | journal=Pediatr Blood Cancer | year= 2014 | volume= 61 | issue= 3 | pages= 457-63 | pmid=24039013 | doi=10.1002/pbc.24729 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=24039013  }} </ref>
:* Young age
:* Young age
:* Fibrillary histology
:* Fibrillary histology
:* Inability to obtain a complete [[resection]]
:* Inability to obtain a complete [[resection]]
* In patients with [[pilocytic astrocytoma]], elevated MIB-1 labeling index, a marker of cellular =activity, is associated with shortened PFS.<ref name="pmid21653594">{{cite journal| author=Margraf LR, Gargan L, Butt Y, Raghunathan N, Bowers DC| title=Proliferative and metabolic markers in incompletely excised pediatric pilocytic astrocytomas--an assessment of 3 new variables in predicting clinical outcome. | journal=Neuro Oncol | year= 2011 | volume= 13 | issue= 7 | pages= 767-74 | pmid=21653594 | doi=10.1093/neuonc/nor041 | pmc=PMC3129272 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21653594  }} </ref><ref name="pmid21610142">{{cite journal| author=Hawkins C, Walker E, Mohamed N, Zhang C, Jacob K, Shirinian M et al.| title=BRAF-KIAA1549 fusion predicts better clinical outcome in pediatric low-grade astrocytoma. | journal=Clin Cancer Res | year= 2011 | volume= 17 | issue= 14 | pages= 4790-8 | pmid=21610142 | doi=10.1158/1078-0432.CCR-11-0034 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21610142  }} </ref> A [[BRAF]]-KIAA fusion, found in [[Pilocytic astrocytoma|pilocytic tumors]], confers a better clinical outcome.
* In patients with [[pilocytic astrocytoma]], elevated MIB-1 labeling index, a marker of cellular activity, is associated with reduced progression-free survival.<ref name="pmid21653594">{{cite journal| author=Margraf LR, Gargan L, Butt Y, Raghunathan N, Bowers DC| title=Proliferative and metabolic markers in incompletely excised pediatric pilocytic astrocytomas--an assessment of 3 new variables in predicting clinical outcome. | journal=Neuro Oncol | year= 2011 | volume= 13 | issue= 7 | pages= 767-74 | pmid=21653594 | doi=10.1093/neuonc/nor041 | pmc=PMC3129272 | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21653594  }} </ref><ref name="pmid21610142">{{cite journal| author=Hawkins C, Walker E, Mohamed N, Zhang C, Jacob K, Shirinian M et al.| title=BRAF-KIAA1549 fusion predicts better clinical outcome in pediatric low-grade astrocytoma. | journal=Clin Cancer Res | year= 2011 | volume= 17 | issue= 14 | pages= 4790-8 | pmid=21610142 | doi=10.1158/1078-0432.CCR-11-0034 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=21610142  }} </ref>
* Children with isolated [[optic nerve]] [[tumor]]s have a better [[prognosis]] than those with [[lesion]]s that involve the [[Chiasma|chiasm]] or that extend along the optic pathway.<ref name="pmid8832464">{{cite journal| author=Campbell JW, Pollack IF| title=Cerebellar astrocytomas in children. | journal=J Neurooncol | year= 1996 | volume= 28 | issue= 2-3 | pages= 223-31 | pmid=8832464 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8832464  }} </ref><ref name="pmid1738456">{{cite journal| author=Schneider JH, Raffel C, McComb JG| title=Benign cerebellar astrocytomas of childhood. | journal=Neurosurgery | year= 1992 | volume= 30 | issue= 1 | pages= 58-62; discussion 62-3 | pmid=1738456 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1738456  }} </ref>; Children with [[NF1]] also have a better [[prognosis]], especially when the [[tumor]] is found in asymptomatic patients at the time of [[screening]].
* A [[BRAF]]-KIAA fusion, found in [[Pilocytic astrocytoma|pilocytic tumors]], confers a better clinical outcome.
* Grade 2 astrocytomas are defined as being [[invasive]] [[gliomas]], meaning that the [[tumor]] [[cell]]s penetrate into the surrounding normal [[brain]]. People with [[oligodendrogliomas]] (which might share common [[cell]]s of origin have better [[prognosis]] than those with mixed [[Oligoastrocytoma|oligoastrocytomas]], who in turn have better [[prognosis]] than patients with (pure) [[Low grade astrocytoma|low-grade astrocytomas]]. Individuals with grade 2 astrocytoma have a 5-year survival rate of about 34% without treatment and about 70% with [[radiation therapy]]. The median survival time is 4 years.<ref name="pmid12145515">{{cite journal| author=Due-Tønnessen BJ, Helseth E, Scheie D, Skullerud K, Aamodt G, Lundar T| title=Long-term outcome after resection of benign cerebellar astrocytomas in children and young adults (0-19 years): report of 110 consecutive cases. | journal=Pediatr Neurosurg | year= 2002 | volume= 37 | issue= 2 | pages= 71-80 | pmid=12145515 | doi=65108 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12145515  }} </ref>
* Children with isolated [[optic nerve]] [[tumor]]s have a better [[prognosis]] than those with [[lesion]]s that involve the [[Chiasma|chiasm]] or that extend along the optic pathway.<ref name="pmid8832464">{{cite journal| author=Campbell JW, Pollack IF| title=Cerebellar astrocytomas in children. | journal=J Neurooncol | year= 1996 | volume= 28 | issue= 2-3 | pages= 223-31 | pmid=8832464 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8832464  }} </ref><ref name="pmid1738456">{{cite journal| author=Schneider JH, Raffel C, McComb JG| title=Benign cerebellar astrocytomas of childhood. | journal=Neurosurgery | year= 1992 | volume= 30 | issue= 1 | pages= 58-62; discussion 62-3 | pmid=1738456 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1738456  }} </ref>
High-grade astrocytomas:
* Children with [[NF1]] also have a better [[prognosis]], especially when the [[tumor]] is found in asymptomatic patients at the time of [[screening]].
* Biologic markers, such as [[p53]] overexpression and [[mutation]] status, may be useful predictors of outcome in patients with high-grade [[gliomas]]. MIB-1 labeling index is predictive of outcome in childhood [[malignant]] brain [[tumors]].<ref name="pmidPMID: 16195804">{{cite journal| author=Rood BR, MacDonald TJ| title=Pediatric high-grade glioma: molecular genetic clues for innovative therapeutic approaches. | journal=J Neurooncol | year= 2005 | volume= 75 | issue= 3 | pages= 267-72 | pmid=PMID: 16195804 | doi=10.1007/s11060-005-6749-5 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16195804  }} </ref> Both histologic classification and proliferative activity evaluation have been shown to be independently associated with survival.<ref name="pmid12015841">{{cite journal| author=Pollack IF, Hamilton RL, Burnham J, Holmes EJ, Finkelstein SD, Sposto R et al.| title=Impact of proliferation index on outcome in childhood malignant gliomas: results in a multi-institutional cohort. | journal=Neurosurgery | year= 2002 | volume= 50 | issue= 6 | pages= 1238-44; discussion 1244-5 | pmid=12015841 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12015841  }} </ref>
* Grade 2 astrocytomas are defined as being [[invasive]] [[gliomas]], meaning that the [[tumor]] [[cell]]s penetrate into the surrounding normal [[brain]].
* Although [[High grade astrocytoma|high-grade astrocytomas]] generally carry a poor [[prognosis]] in younger patients, those with [[Anaplastic astrocytoma|anaplastic astrocytomas]] in whom a gross-total [[resection]] is possible may fare better.
* People with [[oligodendrogliomas]] (which might share common [[cell]]s of origin) have better [[prognosis]] than those with mixed [[Oligoastrocytoma|oligoastrocytomas]], who in turn have better [[prognosis]] than patients with (pure) [[Low grade astrocytoma|low-grade astrocytomas]].
* Individuals with grade 2 astrocytoma have a 5-year survival rate of about 34% without treatment and about 70% with [[radiation therapy]]. The median survival time is 4 years.<ref name="pmid12145515">{{cite journal| author=Due-Tønnessen BJ, Helseth E, Scheie D, Skullerud K, Aamodt G, Lundar T| title=Long-term outcome after resection of benign cerebellar astrocytomas in children and young adults (0-19 years): report of 110 consecutive cases. | journal=Pediatr Neurosurg | year= 2002 | volume= 37 | issue= 2 | pages= 71-80 | pmid=12145515 | doi=65108 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12145515  }} </ref>
 
==== High-grade astrocytomas ====
* Biologic markers, such as [[p53]] [[overexpression]] and [[mutation]] status, may be useful predictors of outcome in patients with high-grade [[gliomas]].
** MIB-1 labeling index is predictive of outcome in childhood [[malignant]] brain [[tumors]].<ref name="pmidPMID: 16195804">{{cite journal| author=Rood BR, MacDonald TJ| title=Pediatric high-grade glioma: molecular genetic clues for innovative therapeutic approaches. | journal=J Neurooncol | year= 2005 | volume= 75 | issue= 3 | pages= 267-72 | pmid=PMID: 16195804 | doi=10.1007/s11060-005-6749-5 | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=16195804  }} </ref>
** Both histologic classification and proliferative activity evaluation have been shown to be independently associated with survival.<ref name="pmid12015841">{{cite journal| author=Pollack IF, Hamilton RL, Burnham J, Holmes EJ, Finkelstein SD, Sposto R et al.| title=Impact of proliferation index on outcome in childhood malignant gliomas: results in a multi-institutional cohort. | journal=Neurosurgery | year= 2002 | volume= 50 | issue= 6 | pages= 1238-44; discussion 1244-5 | pmid=12015841 | doi= | pmc= | url=http://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12015841  }} </ref>
* Although, [[High grade astrocytoma|high-grade astrocytomas]] generally carry a poor [[prognosis]] in younger patients, those with [[Anaplastic astrocytoma|anaplastic astrocytomas]] in whom a gross-total [[resection]] is possible may fare better.
* For [[Low grade astrocytoma|low grade astrocytomas]], removal of the [[tumor]] will generally allow functional survival for many years.
* For [[Low grade astrocytoma|low grade astrocytomas]], removal of the [[tumor]] will generally allow functional survival for many years.
* In some reports, the five-year survival has been over 90% with well resected [[tumor]]s.  
* In some reports, the five-year survival has been over 90% with well resected [[tumor]]s.  
Line 45: Line 54:
* [[Radiation therapy]] has been shown to prolong survival and is a standard component of treatment of [[anaplastic astrocytoma]].
* [[Radiation therapy]] has been shown to prolong survival and is a standard component of treatment of [[anaplastic astrocytoma]].
* Individuals with grade 3 astrocytoma have a [[median]] survival time of 18 months without treatment ([[radiation]] and [[chemotherapy]]).  
* Individuals with grade 3 astrocytoma have a [[median]] survival time of 18 months without treatment ([[radiation]] and [[chemotherapy]]).  
* Although [[radiotherapy]] rarely cures [[glioblastoma multiforme]], studies show that it doubles the median survival of patients, compared to supportive care alone. The [[prognosis]] is worst for these grade 4 [[glioma]]s. Few patients survive beyond 3 years. Individuals with grade 4 astrocytoma have a median survival time of 17 weeks without treatment, 30 weeks with [[radiation]], and 37 weeks with surgical removal of most of the [[tumors]].<ref name="pmid15497115">{{cite journal |vauthors=See SJ, Gilbert MR |title=Anaplastic astrocytoma: diagnosis, prognosis, and management |journal=Semin. Oncol. |volume=31 |issue=5 |pages=618–34 |date=October 2004 |pmid=15497115 |doi= |url=}}</ref><ref name="pmid12187956">{{cite journal |vauthors=Korshunov A, Golanov A, Sycheva R |title=Immunohistochemical markers for prognosis of anaplastic astrocytomas |journal=J. Neurooncol. |volume=58 |issue=3 |pages=203–15 |date=July 2002 |pmid=12187956 |doi= |url=}}</ref><ref name="pmid2990664">{{cite journal |vauthors=Burger PC, Vogel FS, Green SB, Strike TA |title=Glioblastoma multiforme and anaplastic astrocytoma. Pathologic criteria and prognostic implications |journal=Cancer |volume=56 |issue=5 |pages=1106–11 |date=September 1985 |pmid=2990664 |doi= |url=}}</ref>
* Although [[radiotherapy]] rarely cures [[glioblastoma multiforme]], studies show that it doubles the median survival of patients, compared to supportive care alone.
* The [[prognosis]] is worst for these grade 4 [[glioma]]s.<ref name="pmid15497115">{{cite journal |vauthors=See SJ, Gilbert MR |title=Anaplastic astrocytoma: diagnosis, prognosis, and management |journal=Semin. Oncol. |volume=31 |issue=5 |pages=618–34 |date=October 2004 |pmid=15497115 |doi= |url=}}</ref><ref name="pmid12187956">{{cite journal |vauthors=Korshunov A, Golanov A, Sycheva R |title=Immunohistochemical markers for prognosis of anaplastic astrocytomas |journal=J. Neurooncol. |volume=58 |issue=3 |pages=203–15 |date=July 2002 |pmid=12187956 |doi= |url=}}</ref><ref name="pmid2990664">{{cite journal |vauthors=Burger PC, Vogel FS, Green SB, Strike TA |title=Glioblastoma multiforme and anaplastic astrocytoma. Pathologic criteria and prognostic implications |journal=Cancer |volume=56 |issue=5 |pages=1106–11 |date=September 1985 |pmid=2990664 |doi= |url=}}</ref>
** Few patients survive beyond 3 years.
** Individuals with grade 4 astrocytoma have a median survival time of:
*** 17 weeks without treatment,
*** 30 weeks with [[radiation]]
*** 37 weeks with surgical removal of most of the [[tumors]].


==References==
==References==

Revision as of 18:41, 31 January 2019

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D., Ammu Susheela, M.D. [2]

Overview

If left untreated, all of patients with low grade astrocytomas will have a rapid growth of the tumor similar to high grade astrocytoma and all of the patients with high grade astrocytoma will become symptomatic and deteriorate. Astrocytoma being a space occupying lesion may have following complications depending on the location of the tumor including increased intracranial pressure, cognitive dysfunction, emotional disturbances, behavioral complications, visual defects and muscle weakness. Low-grade astrocytomas (grade I [pilocytic] and grade II) have a relatively favorable prognosis, particularly for circumscribed, grade I lesions where complete excision may be possible. High-grade astrocytomas generally carry a poor prognosis in younger patients.


Natural History, Complications, and Prognosis

Natural History

Complications

  • Astrocytoma being a space occupying lesion may have following complications depending on the location of the tumor:[6][7][8]

Prognosis

Low-grade astrocytomas

  • Young age
  • Fibrillary histology
  • Inability to obtain a complete resection

High-grade astrocytomas

References

  1. Recht LD, Lew R, Smith TW (April 1992). "Suspected low-grade glioma: is deferring treatment safe?". Ann. Neurol. 31 (4): 431–6. doi:10.1002/ana.410310413. PMID 1586143.
  2. Olson JD, Riedel E, DeAngelis LM (April 2000). "Long-term outcome of low-grade oligodendroglioma and mixed glioma". Neurology. 54 (7): 1442–8. PMID 10751254.
  3. Bauman G, Fisher B, Watling C, Cairncross JG, Macdonald D (December 2009). "Adult supratentorial low-grade glioma: long-term experience at a single institution". Int. J. Radiat. Oncol. Biol. Phys. 75 (5): 1401–7. doi:10.1016/j.ijrobp.2009.01.010. PMID 19395201.
  4. Claus EB, Black PM (March 2006). "Survival rates and patterns of care for patients diagnosed with supratentorial low-grade gliomas: data from the SEER program, 1973-2001". Cancer. 106 (6): 1358–63. doi:10.1002/cncr.21733. PMID 16470608.
  5. Piepmeier J, Christopher S, Spencer D, Byrne T, Kim J, Knisel JP; et al. (1996). "Variations in the natural history and survival of patients with supratentorial low-grade astrocytomas". Neurosurgery. 38 (5): 872–8, discussion 878-9. PMID 8727811.
  6. Ansell P, Johnston T, Simpson J, Crouch S, Roman E, Picton S (January 2010). "Brain tumor signs and symptoms: analysis of primary health care records from the UKCCS". Pediatrics. 125 (1): 112–9. doi:10.1542/peds.2009-0254. PMID 20026498.
  7. Wilne SH, Ferris RC, Nathwani A, Kennedy CR (June 2006). "The presenting features of brain tumours: a review of 200 cases". Arch. Dis. Child. 91 (6): 502–6. doi:10.1136/adc.2005.090266. PMC 2082784. PMID 16547083.
  8. Wilne S, Collier J, Kennedy C, Koller K, Grundy R, Walker D (August 2007). "Presentation of childhood CNS tumours: a systematic review and meta-analysis". Lancet Oncol. 8 (8): 685–95. doi:10.1016/S1470-2045(07)70207-3. PMID 17644483.
  9. Pollack IF (1994). "Brain tumors in children". N Engl J Med. 331 (22): 1500–7. doi:10.1056/NEJM199412013312207. PMID 7969301.
  10. Pfister S, Witt O (2009). "Pediatric gliomas". Recent Results Cancer Res. 171: 67–81. doi:10.1007/978-3-540-31206-2_4. PMID 19322538.
  11. Fisher PG, Tihan T, Goldthwaite PT, Wharam MD, Carson BS, Weingart JD; et al. (2008). "Outcome analysis of childhood low-grade astrocytomas". Pediatr Blood Cancer. 51 (2): 245–50. doi:10.1002/pbc.21563. PMID 18386785.
  12. Bandopadhayay P, Bergthold G, London WB, Goumnerova LC, Morales La Madrid A, Marcus KJ; et al. (2014). "Long-term outcome of 4,040 children diagnosed with pediatric low-grade gliomas: an analysis of the Surveillance Epidemiology and End Results (SEER) database". Pediatr Blood Cancer. 61 (7): 1173–9. doi:10.1002/pbc.24958. PMID 24482038.
  13. von Hornstein S, Kortmann RD, Pietsch T, Emser A, Warmuth-Metz M, Soerensen N; et al. (2011). "Impact of chemotherapy on disseminated low-grade glioma in children and adolescents: report from the HIT-LGG 1996 trial". Pediatr Blood Cancer. 56 (7): 1046–54. doi:10.1002/pbc.23006. PMID 21319282.
  14. Mazloom A, Hodges JC, Teh BS, Chintagumpala M, Paulino AC (2012). "Outcome of patients with pilocytic astrocytoma and leptomeningeal dissemination". Int J Radiat Oncol Biol Phys. 84 (2): 350–4. doi:10.1016/j.ijrobp.2011.12.044. PMID 22401918.
  15. Stokland T, Liu JF, Ironside JW, Ellison DW, Taylor R, Robinson KJ; et al. (2010). "A multivariate analysis of factors determining tumor progression in childhood low-grade glioma: a population-based cohort study (CCLG CNS9702)". Neuro Oncol. 12 (12): 1257–68. doi:10.1093/neuonc/noq092. PMC 3018938. PMID 20861086.
  16. Mirow C, Pietsch T, Berkefeld S, Kwiecien R, Warmuth-Metz M, Falkenstein F; et al. (2014). "Children <1 year show an inferior outcome when treated according to the traditional LGG treatment strategy: a report from the German multicenter trial HIT-LGG 1996 for children with low grade glioma (LGG)". Pediatr Blood Cancer. 61 (3): 457–63. doi:10.1002/pbc.24729. PMID 24039013.
  17. Margraf LR, Gargan L, Butt Y, Raghunathan N, Bowers DC (2011). "Proliferative and metabolic markers in incompletely excised pediatric pilocytic astrocytomas--an assessment of 3 new variables in predicting clinical outcome". Neuro Oncol. 13 (7): 767–74. doi:10.1093/neuonc/nor041. PMC 3129272. PMID 21653594.
  18. Hawkins C, Walker E, Mohamed N, Zhang C, Jacob K, Shirinian M; et al. (2011). "BRAF-KIAA1549 fusion predicts better clinical outcome in pediatric low-grade astrocytoma". Clin Cancer Res. 17 (14): 4790–8. doi:10.1158/1078-0432.CCR-11-0034. PMID 21610142.
  19. Campbell JW, Pollack IF (1996). "Cerebellar astrocytomas in children". J Neurooncol. 28 (2–3): 223–31. PMID 8832464.
  20. Schneider JH, Raffel C, McComb JG (1992). "Benign cerebellar astrocytomas of childhood". Neurosurgery. 30 (1): 58–62, discussion 62-3. PMID 1738456.
  21. Due-Tønnessen BJ, Helseth E, Scheie D, Skullerud K, Aamodt G, Lundar T (2002). "Long-term outcome after resection of benign cerebellar astrocytomas in children and young adults (0-19 years): report of 110 consecutive cases". Pediatr Neurosurg. 37 (2): 71–80. doi:65108 Check |doi= value (help). PMID 12145515.
  22. Rood BR, MacDonald TJ (2005). "Pediatric high-grade glioma: molecular genetic clues for innovative therapeutic approaches". J Neurooncol. 75 (3): 267–72. doi:10.1007/s11060-005-6749-5. PMID 16195804 PMID: 16195804 Check |pmid= value (help).
  23. Pollack IF, Hamilton RL, Burnham J, Holmes EJ, Finkelstein SD, Sposto R; et al. (2002). "Impact of proliferation index on outcome in childhood malignant gliomas: results in a multi-institutional cohort". Neurosurgery. 50 (6): 1238–44, discussion 1244-5. PMID 12015841.
  24. See SJ, Gilbert MR (October 2004). "Anaplastic astrocytoma: diagnosis, prognosis, and management". Semin. Oncol. 31 (5): 618–34. PMID 15497115.
  25. Korshunov A, Golanov A, Sycheva R (July 2002). "Immunohistochemical markers for prognosis of anaplastic astrocytomas". J. Neurooncol. 58 (3): 203–15. PMID 12187956.
  26. Burger PC, Vogel FS, Green SB, Strike TA (September 1985). "Glioblastoma multiforme and anaplastic astrocytoma. Pathologic criteria and prognostic implications". Cancer. 56 (5): 1106–11. PMID 2990664.

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