CNS5:Oligodendroglioma, IDH-mutant and 1p/19q-codeleted: Difference between revisions
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- Germline mutations in ''POT1'' have been associated with familial oligodendroglioma<ref>{{Cite journal|last=Bainbridge|first=Matthew N.|last2=Armstrong|first2=Georgina N.|last3=Gramatges|first3=M. Monica|last4=Bertuch|first4=Alison A.|last5=Jhangiani|first5=Shalini N.|last6=Doddapaneni|first6=Harsha|last7=Lewis|first7=Lora|last8=Tombrello|first8=Joseph|last9=Tsavachidis|first9=Spyros|date=2015-01|title=Germline mutations in shelterin complex genes are associated with familial glioma|url=https://pubmed.ncbi.nlm.nih.gov/25482530|journal=Journal of the National Cancer Institute|volume=107|issue=1|pages=384|doi=10.1093/jnci/dju384|issn=1460-2105|pmc=4296199|pmid=25482530}}</ref> | - Germline mutations in ''POT1'' have been associated with familial oligodendroglioma<ref>{{Cite journal|last=Bainbridge|first=Matthew N.|last2=Armstrong|first2=Georgina N.|last3=Gramatges|first3=M. Monica|last4=Bertuch|first4=Alison A.|last5=Jhangiani|first5=Shalini N.|last6=Doddapaneni|first6=Harsha|last7=Lewis|first7=Lora|last8=Tombrello|first8=Joseph|last9=Tsavachidis|first9=Spyros|date=2015-01|title=Germline mutations in shelterin complex genes are associated with familial glioma|url=https://pubmed.ncbi.nlm.nih.gov/25482530|journal=Journal of the National Cancer Institute|volume=107|issue=1|pages=384|doi=10.1093/jnci/dju384|issn=1460-2105|pmc=4296199|pmid=25482530}}</ref> | ||
== Additional Information == | ==Additional Information== | ||
This disease is <u>defined/characterized</u> as detailed below: | This disease is <u>defined/characterized</u> as detailed below: | ||
* A molecularly defined diffusely infiltrating glioma with IDH1 or IDH2 mutation and codeletion of chromosome arms 1p and 19q<ref name=":0" /> . | |||
* Oligodendrogliomas are graded morphologically as either CNS WHO grade 2 or CNS WHO grade 3. | |||
* In rare cases where molecular studies are unable to be completed or have failed, tumors can be histologically diagnosed as Oligodendroglioma, NOS (not otherwise specified). | |||
The <u>epidemiology/prevalence</u> of this disease is detailed below: | The <u>epidemiology/prevalence</u> of this disease is detailed below: | ||
* Epidemiological statistics should be interpreted with caution as oligodendroglioma is now molecularly defined. | |||
** A subset of tumor historically diagnosed as oligodendroglioma on morphological grounds may therefore not meet current definition | |||
* Oligodendrogliomas occur primarily in adults (median age 43 years for CNS WHO grade 2 and 50 years for CNS WHO grade 3)<ref name=":1" /> | |||
** Slight male preponderance (M:F = 1.2:1<ref name=":1" />) | |||
* Low incidence worldwide | |||
** Incidence is changing over time due to refined molecular definition | |||
*** Incidence rate (cases per 100,000 person-years) for histologically defined oligodendroglioma – 0.10% (Republic of Korea; <ref name=":2" />), 0.50 (France <ref name=":3" />), 0.23 (USA 31675094<ref name=":1" />) | |||
*** Incidence rate for histologically defined CNS WHO Grade 3 oligodendroglioma – 0.06% (Republic of Korea<ref name=":2" />), 0.39 (France <ref name=":3" />), 0.11 (USA<ref name=":1" />) | |||
** CNS WHO grade 2 oligodendrogliomas account for 0.9% of primary brain tumors in US (PMID: 34608945)<ref name=":1" /> | |||
** CNS WHO grade 3 oligodendrogliomas account of primary brain tumors in the US(PMID: 34608945)<ref name=":1" /> | |||
The <u>clinical features</u> of this disease are detailed below: | The <u>clinical features</u> of this disease are detailed below: | ||