Collection and Coding Clarifications for Central Nervous System (CNS) Tumors
Date of clarification: March 27, 2006
Is it possible for a benign schwannoma to arise on the spinal cord or does one always arise from nerve roots coming off the cord? Example: The medical record indicates a C2-C3 schwannoma. Is this a reportable neoplasm?
Anatomically, schwannomas must be derived from Schwann cells which are not a part of the CNS; therefore, they must all come from peripheral nerves, and as such come from nerve roots. The definition of reportable non-malignant sites includes the spinal cord (C72). It does not include "spinal nerves" (C47); consequently, schwannomas of the spinal cord area are not reportable.
If the surgeon states "resection of intradural extramedullary schwannoma," does this mean that the tumor is outside the cord but within the dura?
Yes, but it also means it is on a peripheral nerve root and therefore is not reportable.
Are vestibular schwannomas reportable?
Vestibular schwannomas, also known as acoustic neuromas, arise from the VIIIth cranial nerve (C72.4). Tumors of the cranial nerves are reportable. Code these tumors to acoustic nerve C72.4 M-9560/0.
Are cerebellopontine angle tumors reportable?
Cerebellopontine angle schwannomas are VIIIth cranial nerve schwannomas, and are reportable. However, cerebellopontine angle tumors also include meningiomas and epidermoids, so you must have histologic verification to code them correctly.
Is a schwannoma of the face reportable?
Subcutaneous or intramuscular schwannomas, including those on the face or neck, arise from a peripheral nerve root; therefore, they are not reportable.
Collection and Coding of Blood Vessel Tumors Occurring in CNS Sites
Date of clarification: July 2004
Are non-malignant blood vessel tumors occurring in CNS sites reportable?
Yes. The CNS site/histology listing includes blood vessel tumors under several categories, including meninges C70.0–C70.9, brain C71.0–C71.4 and C71.7–C71.9, (excluding ventricle), spinal cord C72.0, cauda equina C72.1, cranial nerves C72.2–C72.5, cerebellum C71.6, and other nervous system C72.8–C72.9.
These tumors include—
- 9120/0 Hemangioma, NOS
- 9121/0 Cavernous hemangioma
- 9150/0 Hemangiopericytoma, benign
- 9150/1 Hemangiopericytoma, NOS
- 9161/1 Hemangioblastoma
- 9120/3 Angiosarcoma
- 9130/3 Hemangioendothelioma
- 9150/3 Hemangiopericytoma
Should blood vessel tumors occurring in CNS sites be coded to blood vessel or CNS?
They should be coded to the CNS site in which they occur. This follows the same rationale that if you have a lymphoma of the brain, it is coded to "brain," not lymph node.
Example: Patient is diagnosed with a hemangioblastoma of the right temporal lobe of the brain. Site code: C71.2, histology code: 9161/1 Laterality: Right only.
Angiosarcomas are exceedingly rare and occur in the "brain" parenchyma. Hemangiopericytomas are not rare tumors and occur almost entirely attached to the dura (meninges). There have been rare case reports of hemangioendotheliomas of the brain. They occur anywhere in the brain and dura.
Coding Multiple Meningiomas
Date of clarification: March 2004
- Cerebral meningiomas are coded to C70.0; spinal meningiomas are coded to C70.1.
- Cerebral meninges is listed as a lateral site, so you code left and/or right cerebral meninges when possible.
Example: Patient has a meningioma over the right temporal lobe and a second meningioma over the left frontal area. Code the laterality of the first tumor to "right" cerebral meninges and the laterality of the second tumor to "left" cerebral meninges.
- The morphology code for benign meningioma, NOS is M9530/0 (other benign codes are in the 953-series).
- Use of the code for "multiple meningiomas," M9530/1.
This has a behavior code of "1" and does not apply to multiple or sequential benign meningiomas that have a behavior code of /0 OR malignant meningiomas that have a behavior code of /3.
Do not use this code for multiple meningiomas except in rare circumstances (about 1%–2% of all meningiomas). Multiple meningiomas (also called meningiomatosis), is a rare condition strongly associated with neurofibromatosis type 2 and other genetic disorders, but occasionally occurs as sporadic or idiopathic. Occasionally multiple meningiomas may arise that are not associated with a genetic disorder. However, multiple malignant meningiomas are rare.
Multiple Primary Rules for Meningiomas
- Laterality is coded on cerebral meninges. The benign CNS tumor rules indicate that if there are multiple tumors in "different sites" defined as cerebral (C70.0) versus spinal (C70.1) or "different laterality" within cerebral meninges, they are separate tumors.
- Patient has a benign cerebral meningioma, NOS over the left parietal lobe. Site code: C70.0; Laterality: Left only, M9530/0. The patient is subsequently diagnosed with a benign cerebral meningioma, NOS on the right side of the brain. Site code: C70.0; Laterality: Right only; M9530/0. Because these benign tumors are located on different sides of the brain, they are considered separate primary tumors. Two abstracts should be completed.
- Patient has a benign cerebral meningioma, NOS over the left parietal lobe. Site code: C70.0; Laterality: Left only, M9530/0. The patient is subsequently diagnosed with a benign spinal meningioma, NOS. Site code: C70.1; Laterality: Not a paired site; M9530/0. Because these tumors are located in different subsites, they are considered separate primary tumors. Two abstracts should be completed.
- Conversely, if they are in the "same site" (subsite and laterality), they are the same tumor regardless of the timing.
Example: Patient has a benign cerebral meningioma, NOS over the left parietal lobe. Site code: C70.0; Laterality: Left only, M9530/0. The patient is subsequently diagnosed with a benign cerebral meningioma, NOS also on the left side of the brain. Site code: C70.0; Laterality: Left only; M9530/0. Because these tumors are in the same site (C70.0), same histology (M9530/0), and located on the same side of the brain, they are considered the same primary tumor regardless of the timing of the diagnosis. Only one abstract should be completed.
- If one meningioma is benign (/0 or /1) and one is malignant (/3), they are always considered separate tumors and two abstracts are completed.