CNS Anatomy and ICD-O-3
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For Primary Sites, use ICD-O-3 (Topography section): ICD-O-3.1 or the Solid Tumor Rules
Priority order for coding primary site
- ICD-O
- SEER Program Coding Manual (Including Coding Guidelines in Appendix C)
- Solid Tumor Rules (Malignant CNS, Benign CNS)
NAACCR Item #400: Primary Site (NAACCR Item #2580 Text-Primary Site Title)
For primary sites C710-C719, an “S” for supratentorial, or an “I” for infratentorial will be included after the term. This classification is important for EOD Primary Tumor and Summary Stage.
See Solid Tumor Rules for additional information on location.
| ICD-O-3 Codes | ICD-O-3 Preferred Term | Synonyms | Location |
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| Peripheral Nerve and Autonomic Nervous System | |||
| C470 | Peripheral nerves and autonomic nervous system of head, face, and neck |
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| C471 | Peripheral nerves and autonomic nervous system of upper limb and shoulder |
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| C472 | Peripheral nerves and autonomic nervous system of lower limb and hip |
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| C473 | Peripheral nerves and autonomic nervous system of thorax |
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| C474 | Peripheral nerves and autonomic nervous system of abdomen |
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| C475 | Peripheral nerves and autonomic nervous system of pelvis |
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| C476 | Peripheral nerves and autonomic nervous system of trunk, NOS |
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| C478 | Overlapping lesion of peripheral nerves and autonomic nervous system | ||
| C479 | Autonomic nervous system, NOS |
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| Meninges | |||
| C700 | Cerebral meninges |
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| C701 | Spinal meninges |
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| C709 | Meninges, NOS |
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| Brain | |||
| C710 | Cerebrum (S) |
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| C711 | Frontal lobe (S) |
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| C712 | Temporal Lobe (S) |
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| C713 | Parietal Lobe (S) | Located near the top and center of the brain, situated behind the frontal lobe and above the temporal lobe | |
| C714 | Occipital Lobe (S) |
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| C715 | Ventricle (NOS) (S) |
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| C716 | Cerebellum (I) |
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| C717 | Brain stem (I) |
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| C718 | Overlapping lesion of the Brain |
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| C719 | Brain, NOS (S) |
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| Spinal Sites | |||
| C720 | Spinal cord |
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| C721 | Cauda equina |
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| Cranial Nerves | |||
| C722 | Olfactory nerve |
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| C723 | Optic nerve |
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| C724 | Acoustic nerve |
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| C725 | Cranial nerves, NOS |
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| Ill-Defined Sites Central Nervous System | |||
| C728 | Overlapping lesion of brain and central nervous system | ||
| C729 | Nervous system, NOS |
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| Intracranial Duct and Glands | |||
| C751 | Pituitary gland |
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Located at the base of the brain |
| C752 | Craniopharyngeal duct | ||
| C753 | Pineal gland | Small endocrine gland in the brain, situated beneath the back part of the corpus callosum. | |
Behavior
Per the Solid Tumor Rules, there is a priority order for assigning behavior for CNS tumors
- Pathology-Tissue from resection
- Pathology: Tissue from biopsy
- Cytology (usually cerebrospinal fluid)
- Physician’s documentation (no pathology report)
- Scans with a priority order: MRI, CT, PET, Angiogram
- CNS CAP Protocol
Primary Site Coding Issues
For some histologic types, it is difficult to determine if the primary site is intracranial or the skull (C410). Tumors that originate in the skull are not intracranial. The malignant tumors are reportable regardless of origin, but non-malignant tumors that originate in the skull are not reportable.
- Chondroma (9220/0) is a benign tumor of cartilage cells. Review the record carefully to determine if the tumor originated in bone or in an intracranial site. Because chondroma is a benign tumor, only complete an abstract if the primary tumor is in an intracranial site. A chondroma of the skull is not reportable.
- Chordoma (9370/3) is a malignant tumor arising from the embryonic notochord, while a chondrosarcoma (9220/3) is a malignant tumor of cartilage cells. These tumor histologies are reportable, but you must determine if the primary site is bone or an intracranial site. Intracranial tumors are analyzed separately for research purposes.
Pilocytic Astrocytoma (9421/1) vs High-grade astrocytoma with piloid features (HGAP) (9421/3)
When ICD-O-3 was published, the code for pilocytic astrocytoma changed from malignant behavior (/3) to borderline behavior (/1). Registrars were instructed to continue to assign the code Pilocytic astrocytoma as /3 for malignant behavior.
In 2023, a new definition for 9421/3 was added, High-grade astrocytoma with piloid features (HGAP), which is a very aggressive tumor. So, starting in 2023, registrars were instructed to code Pilocytic astrocytoma as 9421/1.
Intracranial schwannoma (9560/0) is reportable for cases diagnosed January 1, 2004, and later. It can be difficult to determine the intracranial site of a schwannoma. Assign the primary site for intracranial schwannoma to cranial nerves NOS (C725) when the site is not documented in the health record.
See the current version of the SEER Program Coding Manual, Section IV: Description of this Neoplasm and Appendix C for complete coding instructions for primary site.
Updated: September 23, 2026
Suggested Citation
SEER Training Modules: CNS Anatomy and ICD-O-3. U.S. National Institutes of Health, National Cancer Institute. Cited 24 September 2026. Available from: https://training.seer.cancer.gov.