Astrocytoma natural history
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. ; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D., Ammu Susheela, M.D. 
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
- Low grade astrocytoma:
- The natural history of all low grade astrocytomas is not the same.
- Most of the patients experience almost no progression of symptoms for initial 5 to 7 years.
- If left untreated, all the patients with low grade astrocytomas will have rapid growth of tumor similar to high grade astrocytoma.
- High grade astrocytoma:
- If left untreated, all of the patients become symptomatic and deteriorate.
- Recurrence is more common in high grade astrocytoma compared to low grade astrocytoma.
- Astrocytoma being a space occupying lesion may have following complications depending on the location of the tumor:
- Increased intracranial pressure
- Cognitive dysfunction
- Emotional disturbances
- Behavioral complications
- Visual defects
- Muscle weakness
- Local neurological deficit
- Low-grade astrocytomas (grade I [pilocytic] and grade II) have a relatively favorable prognosis, particularly for circumscribed, grade I lesions where complete excision is possible.
- Tumor spread, when it occurs, is usually by contiguous extension; dissemination to other CNS sites may occur, although uncommon.
- Although, metastasis is uncommon, tumors may be of multi-focal origin, especially when associated with neurofibromatosis 1 (NF1).
- Unfavorable prognostic features for childhood low-grade astrocytomas include:
- Young age
- Fibrillary histology
- Inability to obtain a complete resection
- In patients with pilocytic astrocytoma, elevated MIB-1 labeling index, a marker of cellular activity, is associated with reduced progression-free survival.
- A BRAF-KIAA fusion, found in pilocytic tumors, confers a better clinical outcome.
- Children with isolated optic nerve tumors have a better prognosis than those with lesions that involve the chiasm or that extend along the optic pathway.
- Children with NF1 also have a better prognosis, especially when the tumor is found in asymptomatic patients at the time of screening.
- Grade 2 astrocytomas are defined as being invasive gliomas, meaning that the tumor cells penetrate into the surrounding normal brain.
- People with oligodendrogliomas (which might share common cells of origin) have better prognosis than those with mixed oligoastrocytomas, who in turn have better prognosis than patients with (pure) 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.
- Biologic markers, such as p53 overexpression and mutation status, may be useful predictors of outcome in patients with high-grade gliomas.
- Although, high-grade astrocytomas generally carry a poor prognosis in younger patients, those with anaplastic astrocytomas in whom a gross-total resection is possible may fare better.
- For 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 tumors.
- To date, complete resection of high grade astrocytomas is impossible because of the diffuse infiltration of tumor cells.
- 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).
- 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 gliomas.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Pollack IF (1994). "Brain tumors in children". N Engl J Med. 331 (22): 1500–7. doi:10.1056/NEJM199412013312207. PMID 7969301.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ 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.
- ↑ Campbell JW, Pollack IF (1996). "Cerebellar astrocytomas in children". J Neurooncol. 28 (2–3): 223–31. PMID 8832464.
- ↑ Schneider JH, Raffel C, McComb JG (1992). "Benign cerebellar astrocytomas of childhood". Neurosurgery. 30 (1): 58–62, discussion 62-3. PMID 1738456.
- ↑ 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.
- ↑ 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
- ↑ 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.
- ↑ See SJ, Gilbert MR (October 2004). "Anaplastic astrocytoma: diagnosis, prognosis, and management". Semin. Oncol. 31 (5): 618–34. PMID 15497115.
- ↑ Korshunov A, Golanov A, Sycheva R (July 2002). "Immunohistochemical markers for prognosis of anaplastic astrocytomas". J. Neurooncol. 58 (3): 203–15. PMID 12187956.
- ↑ 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.