Data Last Updated: May 31, 2024

NAACCR Item #3816: Brain Molecular Markers

Item # Length Source of Standard Section Name Year Implemented Version Implemented XML NAACCR ID Parent XML Element
3816 2 NAACCR Stage/Prognostic Factors 2018 18 brainMolecularMarkers Tumor
Required Status:

NPCR Collect: Required, site specific
CoC Collect: No recommendation
SEER Collect: Required, site specific
CCCR Collect: No recommendation

Description
Multiple brain molecular markers have become standard pathology components necessary for diagnosis. This data item captures clinically important brain cancer subtypes identified by molecular markers that are not distinguishable by ICD-O-3 codes.
Rationale
Collection of these clinically important brain cancer subtypes has been recommended by CBTRUS.
Notes
**Definition** If a mutation or alteration is in the name of the histopathology, it is required for diagnosis as it helps distinguish among clinically important subtypes within ICD-O-3.  * _IDH mutation status_ distinguishes between clinically important subtypes within ICD-O-3 9400/3, Diffuse astrocytoma and 9401/3, Anaplastic astrocytoma. * _IDH mutant and 1p/19q co-deletion_ distinguishes between clinically important subtypes within ICD-O-3 code 9450/3, Oligodendroglioma and 9451/3, Anaplastic Oligodendroglioma. * _IDH-wildtype_ distinguishes clinically important subtypes within ICD-O-3 9400/3, Diffuse astrocytoma, 9401/3, Anaplastic astrocytoma and 9440/3, Glioblastoma, Epithelioid glioblastoma and Glioblastoma, NOS (note that the new ICD-O-3 code 9445/3 applies to Glioblastoma, IDH-mutant; information regarding this subtype is not collected using this data item). * _SHH-activation and TP53-wildtype_ distinguishes between clinically important subtypes within ICD-O-3 histology code 9471/3, Medulloblastoma. * _C19MC alteration_ status distinguishes a clinically important highly aggressive subtype within ICD-O-3 9478/3, Embryonal tumor with multilayered rosettes. * _Pediatric-type diffuse low-grade gliomas_: 9385/3 Diffuse hemispheric glioma, H3-G34-mutant, Diffuse midline glioma, H3 K27-altered, Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype, and Infant-type hemispheric glioma. * _Ependymal tumors_ are distinguished by mutations required for diagnosis among clinically important subtypes: 9396/3 Posterior fossa group A (PFA) ependymoma, Posterior fossa group B (PFB) ependymoma, Spinal ependymoma, \_MYCN\_-amplified, Supratentorial ependymoma, _YAPI_ fusion-positive, and Supratentorial ependymoma, _ZFTA_ fusion-positive. * _Pediatric-type diffuse low-grade gliomas_ are distinguished among subtypes by mutations required for diagnosis: 9421/1 Diffuse astrocytoma, \_MYB\_-or \_MYBL1\_-altered, Diffuse low-grade glioma, MAPK pathway-altered, and Pilocytic astrocytoma. * _Circumscribed astrocytic tumors_ are distinguished among subtypes by mutations required for diagnosis: 9430/3 Astroblastoma, MN1-altered and Astroblastoma. * _Other CNS embryonal tumors_ are distinguished among subtypes by mutations required for diagnosis: 9500/3 CNS neuroblastoma\_, FOXR2\_-activated, CNS tumor with _BCOR_ internal tandem duplication, and Neuroblastoma, NOS.
Allowable Values
01-23, 85-88, 99
Codes
Code Description
01 Astrocytoma, IDH-mutant, grade 2 (9400/3)
02 Diffuse astrocytoma, IDH-wildtype (9400/3)
03 Astrocytoma, IDH-mutant, grade 3 (9401/3)
04 Anaplastic astrocytoma, IDH-wildtype (9401/3)
05 Glioblastoma, IDH-wildtype (9440/3)
06 Oligodendroglioma, IDH-mutant and 1 p/19q co-deleted (9450/3)
07 Oligodendroglioma, IDH-mutant and 1p/19q co-deleted, grade 3  (9451/3)
08 Medulloblastoma, SHH-activated and TP53-wildtype (9471/3)
09 Embryonal tumor with multilayered rosettes, C19MC-altered (9478/3)
10 Diffuse hemispheric glioma, H3-G34 mutant (9385/3)
11 Diffuse midline glioma, H3 K27-altered (9385/3)
12 Diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype (9385/3)
13 Infant-type hemispheric glioma (9385/3)
14 Posterior fossa group A (PFA) ependymoma (9396/3)
15 Posterior fossa group B (PFB) ependymoma (9396/3)
16 Spinal ependymoma, MYCN-amplified (9396/3)
17 Supratentorial ependymoma, YAP1 fusion-positive (9396/3)
18 Supratentorial ependymoma, ZFTA fusion-positive (9396/3)
19 Diffuse astrocytoma, MYB- or MYBL1-altered (9421/1)
20 Diffuse low-grade glioma, MAPK pathway-altered (9421/1)
21 Astroblastoma, MN1-altered (9430/3)
22 CNS neuroblastoma, FOXR2-activated (9500/3)
23 CNS tumor with _BCOR_ internal tandem duplication (9500/3)
85 Not applicable: Histology not 9385/3, 9396/3, 9400/3, 9401/3, 9430/3, 9440/3, 9450/3, 9451/3, 9471/3, 9478/3, 9421/1, 9430/3, 9500/3
86 Benign or borderline tumor  Excludes: 9421/1 (codes 19-20)
87 Test ordered, results not in chart
88 Not applicable: Information not collected for this case (If this item is required by your standard setter, use of code 88 will result in an edit error.)
99 Not documented in patient record No microscopic confirmation Brain molecular markers not assessed or unknown if assessed
Code Notes
Each Site-Specific Data Item (SSDI) applies only to selected primary sites, histologies, and years of diagnosis. Depending on applicability and standard-setter requirements, SSDIs may be left blank.