As of 1/22/2019 the following is an excerpt from the Solid Tumor Manual. Please review the manual for complete instructions and details.
C470-479, C700, C701, C709, C710-C719, C720-C725, 728, C729, C751-C753
- Non-Malignant intracranial and CNS tumors are NOT included. They have a separate set of rules.
- The 2007 rules said a glioblastoma multiforme (GBM) following an astrocytic or glial tumor was a single primary. In the 2018 Solid Tumor Rules GBM subsequent to an astrocytic or glial tumor is a multiple primary. GBM is now being collected as a new primary so it is possible to analyze the frequency with which these tumors recur in a more aggressive form (GBM).
- The following meningiomas are reportable:
- Intraosseous
- Cavernous sinus
- Sphenoid wing
- Multiple cerebral meningiomas are a single primary
- Multiple brain tumors of same histology are single primary
- Laterality is not used to determine multiple primaries
- Timing is not used to determine multiple primaries
- The brain C710-C719 is a single primary site
- Neurofibromatosis NOS, Neurofibromatosis 1 (NF1), Neurofibromatosis 2 (NF2), and schwannomatosis are genetic syndromes and not reportable neoplasms. People with this genetic syndrome do have a high risk of developing:
- Non-reportable non-malignant tumors occurring in skin and sites other than CNS AND
- Reportable malignant tumors
The 2016 WHO (World Health Organization) Classification of Tumors of the Central Nervous System contains newly recognized histologies and subtypes/variants. New codes/terms are identified by asterisks (*) in Table 3 in Terms and Definitions
- CNS neoplasms must meet all three of the conditions below to be reported as malignant /3
1.The behavior must be malignant /3
-
- The tumor is a WHO Grade 3 or 4 (See Section 1: Table 1
- Note that WHO Grade 2 tumors may be non-malignant or malignant
- Always code the behavior as designated by the pathologist
- Never report a malignant /3 behavior code for a meningioma based on tumor extension to brain, skin of scalp, or other regional organs/tissue. Non-malignant CNS tumors can extend to the regional tissue and bone
- The tumor is a WHO Grade 3 or 4 (See Section 1: Table 1
2.The primary site must be reportable (See Section 2: Table 2) AND
3.The histology must be reportable (See Section 2: Table 3)
Behavior determines which set of CNS rules should be used: malignant vs non malignant.
Priority Order for Using Documentation to Assign Behavior: Start with Instruction 1 and STOP when you reach the instruction that applies to the case being abstracted:
1. Pathology: Tissue from resection
- Use pathologist’s description of malignant/invasive behavior
- Cases are reportable as malignant when pathology states WHO Grade 3 or 4
- WHO Grade 2 may be either non-malignant or malignant. Use the pathologist’s description of behavior
- Never change behavior described by pathologist
2.Pathology: Tissue from biopsy
3.Cytology (usually cerebrospinal fluid)
4.Physician’s documentation (no pathology report) in the following priority order
- Tumor Board
- Documentation of original pathologic diagnosis and behavior
- Documentation of behavior, no mention of original diagnosis
5.Scan, use behavior information from radiography in the following priority order
- MRI
- CT
- PET
- Angiogram
When instructions 1-5 do not apply use Table 1 to determine behavior
After determining behavior code, the next step is to confirm both the primary and the histology are reportable:
- All malignant tumors are reportable including peripheral nerve tumors AND
- The Malignant CNS and Peripheral Nerve rules contain the correct histologies and coding rules for tumors of peripheral nerves and meninges/dura
- Peripheral nerves are extracranial or extradural
- The following malignant meningiomas are reportable:
- Intraosseous. The primary site for intraosseous meningioma is cranial meninges C700
- Sphenoid Wing. Sphenoid wing meningiomas arise in the cranial meninges C700 which covers the bony structure called the sphenoid wing
- Cavernous sinus. There is no ICD-O site code for cavernous sinus because tumors do not arise in the sinus itself; the primary sites are:
- The cranial nerves passing through the sinus (trochlear, abducent C725) OR
- The cerebral meninges/dura C700 covering the cranial nerve
The classification of brain tumors is a subjective matter because definitive criteria have not been established/accepted. Pathologist may disagree on the histology or behavior. Follow the steps below for coding the histology and/or behavior of tumor when there are discrepancies:
- When possible, get advice from the pathologist
- When possible, contact attending physician
- When possible, consult with registry advisor
- If none of these options are available, code the histology and behavior from the most dependable source (see Priority List for Coding Histology)
Use Table 5 to identify sites for which laterality must be coded. Do NOT use this table to determine multiple primaries:
Paired Sites and Codes:
- Acoustic Nerve C724
- Cerebral Meninges C700
- Cerebrum C710
- Cranial Nerves C725
- Frontal Lobe C711
- Occipital Lobe C714
- Olfactory Nerve C722
- Optic Nerve C723
- Parietal Lobe C713
- Temporal Lobe C712
Midline tumors are common for glioblastoma multiforme and meningiomas
Code the most specific histology when any one of the following criteria are met:
1.Code the histology when the exact term is documented
2.Code the histology when described as:
-
- Subtype
- Type
- Variant
- Note: The most specific histology may be described as component, majority/majority of, or predominantly
3.Code the histology described as differentiation or features/features of ONLY when there is a specific ICD-O code for the “NOS with ____ features” or “NOS with ____ differentiation”
-
- Note: Do not code differentiation or features when there is no specific ICD-O code
4.Code the histology described by ambiguous terminology ONLY when:
-
- Histology is clinically confirmed by a physician (attending, pathologist, oncologist, etc)
- Patient is receiving treatment based on the histology described by an ambiguous term
- Case is accessioned based on ambiguous terminology and no other histology information is available/documented
Do Not Code histology when described as:
- Architecture
- Foci, focus, focal
- Pattern