Pilocytic astrocytoma: Difference between revisions
Jump to navigation
Jump to search
Jensflorian (talk | contribs) →Molecular: update |
Jensflorian (talk | contribs) →IHC: update |
||
| (3 intermediate revisions by the same user not shown) | |||
| Line 36: | Line 36: | ||
*Classically in the cerebellum in children; most common glioma in children.<ref name=Ref_PSNP82>{{Ref PSNP|82}}</ref> | *Classically in the cerebellum in children; most common glioma in children.<ref name=Ref_PSNP82>{{Ref PSNP|82}}</ref> | ||
*The ''optic glioma'' is associated with neurofibromatosis 1. | *The ''optic glioma'' is associated with neurofibromatosis 1. | ||
* | *Rare variants include [[Pilomyxoid astrocytoma]] and [[Anaplastic pilocytic astrocytoma]]. | ||
==Imaging== | |||
*Well-defined, T2-hyperintense. | |||
*Strong CM enhancement. | |||
*May contain cysts. | |||
*Associated with midline structures. | |||
==Gross== | ==Gross== | ||
Features:<ref name=Ref_PSNP82>{{Ref PSNP|82}}</ref> | Features:<ref name=Ref_PSNP82>{{Ref PSNP|82}}</ref> | ||
*Usually well-circumscribed. | *Usually well-circumscribed, soft. | ||
* | *Can be cystic with mural nodule. | ||
*Optic gliomas may present as fusiform mass. | |||
*Occ. calcium deposits and hemosiderin. | |||
==Microscopic== | ==Microscopic== | ||
| Line 103: | Line 111: | ||
*KI-67: may be "high" (~20% ???). | *KI-67: may be "high" (~20% ???). | ||
*Olig 2: Usually strongly present.<ref name=pmid21193945>{{Cite journal | last1 = Otero | first1 = JJ. | last2 = Rowitch | first2 = D. | last3 = Vandenberg | first3 = S. | title = OLIG2 is differentially expressed in pediatric astrocytic and in ependymal neoplasms. | journal = J Neurooncol | volume = 104 | issue = 2 | pages = 423-38 | month = Sep | year = 2011 | doi = 10.1007/s11060-010-0509-x | PMID = 21193945 }}</ref> | *Olig 2: Usually strongly present.<ref name=pmid21193945>{{Cite journal | last1 = Otero | first1 = JJ. | last2 = Rowitch | first2 = D. | last3 = Vandenberg | first3 = S. | title = OLIG2 is differentially expressed in pediatric astrocytic and in ependymal neoplasms. | journal = J Neurooncol | volume = 104 | issue = 2 | pages = 423-38 | month = Sep | year = 2011 | doi = 10.1007/s11060-010-0509-x | PMID = 21193945 }}</ref> | ||
*[[IDH1]] (R132H) -ve. | |||
*[[H3F3A]] (K27M) -ve. | |||
==Molecular== | ==Molecular== | ||
| Line 111: | Line 121: | ||
*Up to 15% of all [[NF1]] patients develop a PA ("optic glioma" as predilection).<ref>{{Cite journal | last1 = Friedrich | first1 = RE. | last2 = Nuding | first2 = MA. | title = Optic Pathway Glioma and Cerebral Focal Abnormal Signal Intensity in Patients with Neurofibromatosis Type 1: Characteristics, Treatment Choices and Follow-up in 134 Affected Individuals and a Brief Review of the Literature. | journal = Anticancer Res | volume = 36 | issue = 8 | pages = 4095-121 | month = Aug | year = 2016 | doi = | PMID = 27466519 }}</ref> | *Up to 15% of all [[NF1]] patients develop a PA ("optic glioma" as predilection).<ref>{{Cite journal | last1 = Friedrich | first1 = RE. | last2 = Nuding | first2 = MA. | title = Optic Pathway Glioma and Cerebral Focal Abnormal Signal Intensity in Patients with Neurofibromatosis Type 1: Characteristics, Treatment Choices and Follow-up in 134 Affected Individuals and a Brief Review of the Literature. | journal = Anticancer Res | volume = 36 | issue = 8 | pages = 4095-121 | month = Aug | year = 2016 | doi = | PMID = 27466519 }}</ref> | ||
*Rare reports of PA in Noonan-Syndrome (PTPN11 mutation).<ref>{{Cite journal | last1 = Jones | first1 = DT. | last2 = Hutter | first2 = B. | last3 = Jäger | first3 = N. | last4 = Korshunov | first4 = A. | last5 = Kool | first5 = M. | last6 = Warnatz | first6 = HJ. | last7 = Zichner | first7 = T. | last8 = Lambert | first8 = SR. | last9 = Ryzhova | first9 = M. | title = Recurrent somatic alterations of FGFR1 and NTRK2 in pilocytic astrocytoma. | journal = Nat Genet | volume = 45 | issue = 8 | pages = 927-32 | month = Aug | year = 2013 | doi = 10.1038/ng.2682 | PMID = 23817572 }}</ref> | *Rare reports of PA in Noonan-Syndrome (PTPN11 mutation).<ref>{{Cite journal | last1 = Jones | first1 = DT. | last2 = Hutter | first2 = B. | last3 = Jäger | first3 = N. | last4 = Korshunov | first4 = A. | last5 = Kool | first5 = M. | last6 = Warnatz | first6 = HJ. | last7 = Zichner | first7 = T. | last8 = Lambert | first8 = SR. | last9 = Ryzhova | first9 = M. | title = Recurrent somatic alterations of FGFR1 and NTRK2 in pilocytic astrocytoma. | journal = Nat Genet | volume = 45 | issue = 8 | pages = 927-32 | month = Aug | year = 2013 | doi = 10.1038/ng.2682 | PMID = 23817572 }}</ref> | ||
==Prognosis== | |||
*Excellent (10-year OS: 90%) | |||
*In thalamic/chiasmatic region not so good (incomplete resection, often [[Pilomyxoid astrocytoma]]). | |||
*Primary treatment: surgery. Incomplete resection: RT has to be considered. | |||
**Chx is given in rare cases that are still progredient<ref>{{Cite journal | last1 = Metts | first1 = RD. | last2 = Bartynski | first2 = W. | last3 = Welsh | first3 = CT. | last4 = Kinsman | first4 = S. | last5 = Bredlau | first5 = AL. | title = Bevacizumab Therapy for Pilomyxoid Astrocytoma. | journal = J Pediatr Hematol Oncol | volume = | issue = | pages = | month = Mar | year = 2017 | doi = 10.1097/MPH.0000000000000824 | PMID = 28338567 }}</ref> | |||
==See also== | ==See also== | ||