CNS5:Oligodendroglioma, IDH-mutant and 1p/19q-codeleted: Difference between revisions
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- Oligodendrogliomas are most often low-grade, slow growing tumors | - Oligodendrogliomas are most often low-grade, slow growing tumors | ||
* Tumors are frequently asymptomatic and are increasingly found incidentally on imaging for other indications<ref>{{Cite journal|last=Wijnenga|first=Maarten M. J.|last2=French|first2=Pim J.|last3=Dubbink|first3=Hendrikus J.|last4=Dinjens|first4=Winand N. M.|last5=Atmodimedjo|first5=Peggy N.|last6=Kros|first6=Johan M.|last7=Smits|first7=Marion|last8=Gahrmann|first8=Renske|last9=Rutten|first9=Geert-Jan|date=2018-01-10|title=The impact of surgery in molecularly defined low-grade glioma: an integrated clinical, radiological, and molecular analysis|url=https://pubmed.ncbi.nlm.nih.gov/29016833|journal=Neuro-Oncology|volume=20|issue=1|pages=103–112|doi=10.1093/neuonc/nox176|issn=1523-5866|pmc=5761503|pmid=29016833}}</ref> | * Tumors are frequently asymptomatic and are increasingly found incidentally on imaging for other indications<ref name=":20">{{Cite journal|last=Wijnenga|first=Maarten M. J.|last2=French|first2=Pim J.|last3=Dubbink|first3=Hendrikus J.|last4=Dinjens|first4=Winand N. M.|last5=Atmodimedjo|first5=Peggy N.|last6=Kros|first6=Johan M.|last7=Smits|first7=Marion|last8=Gahrmann|first8=Renske|last9=Rutten|first9=Geert-Jan|date=2018-01-10|title=The impact of surgery in molecularly defined low-grade glioma: an integrated clinical, radiological, and molecular analysis|url=https://pubmed.ncbi.nlm.nih.gov/29016833|journal=Neuro-Oncology|volume=20|issue=1|pages=103–112|doi=10.1093/neuonc/nox176|issn=1523-5866|pmc=5761503|pmid=29016833}}</ref> | ||
- Most commonly present with seizures<ref name=":4">{{Cite journal|last=Zetterling|first=Maria|last2=Berhane|first2=Luwam|last3=Alafuzoff|first3=Irina|last4=Jakola|first4=Asgeir S.|last5=Smits|first5=Anja|date=2017|title=Prognostic markers for survival in patients with oligodendroglial tumors; a single-institution review of 214 cases|url=https://pubmed.ncbi.nlm.nih.gov/29186201|journal=PloS One|volume=12|issue=11|pages=e0188419|doi=10.1371/journal.pone.0188419|issn=1932-6203|pmc=5706698|pmid=29186201}}</ref> | - Most commonly present with seizures<ref name=":4">{{Cite journal|last=Zetterling|first=Maria|last2=Berhane|first2=Luwam|last3=Alafuzoff|first3=Irina|last4=Jakola|first4=Asgeir S.|last5=Smits|first5=Anja|date=2017|title=Prognostic markers for survival in patients with oligodendroglial tumors; a single-institution review of 214 cases|url=https://pubmed.ncbi.nlm.nih.gov/29186201|journal=PloS One|volume=12|issue=11|pages=e0188419|doi=10.1371/journal.pone.0188419|issn=1932-6203|pmc=5706698|pmid=29186201}}</ref> | ||
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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" /> . | *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. | *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). | *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. | *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 | **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" /> | *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" />) | **Slight male preponderance (M:F = 1.2:1<ref name=":1" />) | ||
* Low incidence worldwide | *Low incidence worldwide | ||
** Incidence is changing over time due to refined molecular definition | **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 (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" />) | ***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 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" /> | **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: | ||
Oligodendrogliomas are most often low-grade, slow growing tumors | |||
Laboratory findings - | * Tumors are frequently asymptomatic and are increasingly found incidentally on imaging for other indications<ref name=":20" /> | ||
* Most commonly present with seizures<ref name=":4" /> | |||
* Can present with focal neurologic deficits or cognitive changes secondary to increased cranial pressure, especially in the high-grade setting<ref name=":4" /> | |||
* Signs and symptoms - Seizures<ref name=":4" />; Headache; Signs of increased intracranial pressure (Focal neurologic deficits, Cognitive changes); Asymptomatic (increasingly an incidental finding on neuroimaging<ref name=":4" />) | |||
* Laboratory findings - Not applicable | |||
The <u>sites of involvement</u> of this disease are detailed below: | The <u>sites of involvement</u> of this disease are detailed below: | ||