Anaplastic pilocytic astrocytoma
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Anaplastic pilocytic astrocytoma | |
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Diagnosis in short | |
Anaplastic pilocytic astrocytoma. H&E stain. | |
LM DDx | astrocytoma, oligodendroglioma, glioblastoma |
Stains | PAS-D +ve (eosinophilic granular bodies) |
IHC | GFAP +ve |
Gross | usually cerebellar |
Site | brain - usu. cerebellum |
| |
Prevalence | very rare - esp. in children |
Prognosis | poor (analog to WHO Grade III) |
Anaplastic pilocytic astrocytoma, abbreviated APA, is a rare high-grade astrocytoma. It can develop from a pilocytic astrocytoma.
General
- High-grade astrocytoma - behaviour corresponds to WHO Grade III.
- Some authors prefer the designation pilocytic astrocytoma with anaplastic features.
- Less than 2% of all pilocytic astrocytomas.[1]
- De novo cases in the setting of neurofibromatosis 1 reported.
- Many tumors have history of previous radiation.
Microscopic
Features:[2]
- At least 4 mitoses/10 high power fields - see HPFitis.
- Hypercellularity.
- Necrosis +/-.
- Pilocytic like (biphasic) - most common.
- Eosinophilic granular bodies +/-.
- Small cell (32%)
- Epithelioid cells (15%)
- Fibrillary areas resembling diffuse astrocytoma.
DDx of APA (brief):
- Ependymoma
- Oligodendroglioma.
- Glioblastoma including small-cell GBM (important).
Images
Vacular proliferations and necrosis in APA mimicking GBM (WC).
IHC
Features:[3]
- GFAP +ve (fibres).
- p53: +ve.
- KI-67: high 25%.
- ATRX loss may be present.[4]
- IDH1 (R132H) -ve.
- H3F3A (K27M) -ve.
Molecular
Prognosis
- Poor, but better than conventional glioblastoma.[6]
- Prognostic unfavourable paramters include: Necrosis, Mitoses and previous radiation.
See also
References
- ↑ Rodriguez, FJ.; Scheithauer, BW.; Burger, PC.; Jenkins, S.; Giannini, C. (Feb 2010). "Anaplasia in pilocytic astrocytoma predicts aggressive behavior.". Am J Surg Pathol 34 (2): 147-60. doi:10.1097/PAS.0b013e3181c75238. PMID 20061938.
- ↑ Rodriguez, FJ.; Scheithauer, BW.; Burger, PC.; Jenkins, S.; Giannini, C. (Feb 2010). "Anaplasia in pilocytic astrocytoma predicts aggressive behavior.". Am J Surg Pathol 34 (2): 147-60. doi:10.1097/PAS.0b013e3181c75238. PMID 20061938.
- ↑ Rodriguez, FJ.; Scheithauer, BW.; Burger, PC.; Jenkins, S.; Giannini, C. (Feb 2010). "Anaplasia in pilocytic astrocytoma predicts aggressive behavior.". Am J Surg Pathol 34 (2): 147-60. doi:10.1097/PAS.0b013e3181c75238. PMID 20061938.
- ↑ Ebrahimi, A.; Skardelly, M.; Bonzheim, I.; Ott, I.; Mühleisen, H.; Eckert, F.; Tabatabai, G.; Schittenhelm, J. (Jun 2016). "ATRX immunostaining predicts IDH and H3F3A status in gliomas.". Acta Neuropathol Commun 4 (1): 60. doi:10.1186/s40478-016-0331-6. PMID 27311324.
- ↑ Jones, DT.; Hutter, B.; Jäger, N.; Korshunov, A.; Kool, M.; Warnatz, HJ.; Zichner, T.; Lambert, SR. et al. (Aug 2013). "Recurrent somatic alterations of FGFR1 and NTRK2 in pilocytic astrocytoma.". Nat Genet 45 (8): 927-32. doi:10.1038/ng.2682. PMID 23817572.
- ↑ Rodriguez, FJ.; Scheithauer, BW.; Burger, PC.; Jenkins, S.; Giannini, C. (Feb 2010). "Anaplasia in pilocytic astrocytoma predicts aggressive behavior.". Am J Surg Pathol 34 (2): 147-60. doi:10.1097/PAS.0b013e3181c75238. PMID 20061938.