# Presentation: 844475

## Malignant Meninges, Brain, Spinal Cord, Cranial Nerves, Pituitary Gland, Craniopharyngeal Duct and Pineal Gland

**Notes:**
These rules were reviewed by the physician member of the Benign Brain Tumor Committee, the Central Brain Tumor Registry of the United States (CBTRUS), and the AJCC site team.

## Equivalent Terms, Definitions, Charts and Illustrations

- Benign and borderline intracranial and CNS tumors have a separate set of rules.

**Notes:**
Do NOT use for benign reportable cases.

## Equivalent Terms, Definitions, Charts and Illustrations

- PNET (Primitive neuroectodermal tumor)
- Central PNET
- Supratentorial PNET
- pPNET –not brain primary

**Notes:**
Central and supratentorial refer to the brain subsite where tumor(s) are located.

PNET tumors include medulloblastoma, pineoblastoma, ependymoblastoma, retinoblastoma, neuroblastoma, esthesioneuroblastoma, medulloepithelioma and ganglioneuroblastoma 

## Chart 1Neuroepithelial Brain CNS

*WHO Classification of Tumors *of the brain and central nervous system
- Not complete listing

**Notes:**
The charts are based on the World Health Organization (WHO) classification of Tumors – the “blue books.”

Although this is not a listing of every tumor that could possibly arise in the brain or central nervous system, other histologies would be rare.

## Slide 5

**Notes:**
The chart reads from top down – less specific to more specific.
The circles show classifications of brain tumors. These classifications do not have ICD-O-3 codes, but will frequently be a part of the documentation in the medical record.  Notice that there are 8 different types of neuroepithelial histologies.

Note the group of glial tumors. The branch decends to glioblastoma, NOS and glioblastoma multiforme. Glioblastoma: A malignant rapidly growing Astrocytoma of the central nervous system.  These neoplasms grow rapidly, invade extensively, and occur most frequently in the cerebrum of adults. Glioblastoma multiforme is an undifferentiated glioblastoma. Any of the glial tumors can recur as a glioblastoma or glioblastoma multiforme. Remember, if the original tumor was glial, a subsequent glial tumor of any type is a recurrence, not a new primary.
Therefore, for a lifetime, if a patient has any of these glial tumors, then subsequently has glioblastoma multiforme, it is not a new primary. It is a recurrence. These tumors may recur in a more differentiated state. In brain there is no timing rule! 

## Chart 2Non-Neuroepithelial Brain CNS

*WHO Classification of Tumors *of the brain and central nervous system
- Not complete listing

**Notes:**
The charts are based on the World Health Organization (WHO) classification of Tumors – the “blue books.”

Although this is not a listing of every tumor that could possibly arise in the brain or central nervous system, other histologies would be rare.

## Slide 7

**Notes:**
The chart reads from top down – less specific to more specific.

The circles show classifications of brain tumors. These classifications do not have ICD-O-3 codes, but will frequently be a part of the documentation in the medical record.

## Multiple Primary Rules

## Unknown if Single or Multiple Tumors

**Notes:**
Use this module when:

Central registry gets a pathology report of a biopsy followed by a hospital report of a resection. Central registry is not sure if there was a single tumor or multiple tumors

Hospital registry has an H&P documenting a biopsy in the physician’s office. Patient has another biopsy or resection. Registrar cannot confirm whether the patient had a single tumor or multiple tumors.

## Slide 10

| Rule | Site |  |  | Behavior | Notes/
Examples | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| UNKNOWN IF SINGLE OR MULTIPLE TUMORS |  |  |  |  | Tumor(s) not described as metastasis |  |
| M1 | Brain |  |  | Invasive (/3) and either a benign (/0) or uncertain/borderline (1) tumor |  | Multiple |

**Notes:**
Note:  The matrix format has been condensed to fit on the slides.
If it is not possible to determine if there is a single tumor or multiple tumors, but there is invasive and a benign or borderline abstract as a multiple primary.
This rule says if you have more than one tumor, invasive and either benign or borderline, that is multiple primaries; don’t treat that as a single primary. Benign and malignant tumors present at the same time are always multiple tumors. Don’t group benign/borderline with malignant tumors. 

## Slide 11

| Rule |  |  |  |  | Notes/Examples | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| UNKNOWN IF SINGLE OR MULTIPLE TUMOR |  |  |  |  | Tumor(s) not described as metastasis |  |
| M2 |  |  |  |  | Use this rule only after all information sources have been exhausted. | Single |

**Notes:**
This is a default so everyone will handle the cases in a standardized manner. 

The note is a warning not to default to a single tumor until all sources of information have been exhausted.

## Single Tumor

## Slide 13

| Rule | Site |  |  |  | Notes/Examples | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| SINGLE TUMOR |  |  |  |  | Tumor not described as metastasis |  |
| M3 | Single |  |  |  | The tumor may overlap onto or extend into adjacent/contiguous site or subsite | Single |

**Notes:**
A single tumor is always a single primary no matter how large it becomes or how many regional sites it may involve.

## Multiple Tumors

## Slide 15

| Rule | Site |  |  | Behavior | Notes/
Examples | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| MULTIPLE TUMORS
Multiple tumors may be a single primary or multiple primaries |  |  |  |  | Tumors not described as metastases |  |
| M4 | Brain |  |  | Invasive (/3) and either a benign (/0) or uncertain/border- line (1) tumor |  | Multiple |

**Notes:**
Same as M1.  Rule placed in both modules to make sure it is not overlooked.
Invasive tumors and benign or borderline are always multiple primaries.

## Slide 16

| Rule | Site |  |  |  |  | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| M5 | Tumors with topography codes different at the second (Cxxx) and/or third (Cxxx) character |  |  |  |  | Multiple |

**Notes:**
Subsites of the brain are not separate primaries. 

A  tumor in the brain or CNS and a separate tumor in any other site are multiple primaries
Notice we are saying if they differ at the 2nd or 3rd character, not the 4th character. Brain is treated as one organ, not right and left lobes, for example. 

## Slide 17

| Rule |  | Histology
 |  |  |  | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| M6 |  | Glioblastoma or glioblastoma multiforme (9440) following a glial tumor 
(See Chart 1)  |  |  |  | Single |

**Notes:**
As previously mentioned,
Glioblastoma: A malignant rapidly growing Astrocytoma of the central nervous system.  These neoplasms grow rapidly, invade extensively, and occur most frequently in the cerebrum of adults. Glioblastoma multiforme is an undifferentiated glioblastoma.  If a patient has a glial tumor, then subsequently has glioblastoma multiforme, it is not a new primary. It is a recurrence. These tumors may recur in a more differentiated state. In brain there is no timing rule! 

## Slide 18

| Rule |  | Histology |  |  | Notes/Examples | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| M7 |  | Tumors with histology codes on the same branch in Chart 1 or Chart 2 |  |  | Recurrence, progression or any reappearance of histologies on the same branch in Chart 1 or Chart 2 is always the same disease process.
Example:  Patient has astrocytoma. Ten years later the patient is diagnosed with glioblastoma multiforme.  This is a progression or recurrence of the earlier astrocytoma. | Single |

**Notes:**
There is no time limit on recurrences. The brain/CNS rules do NOT have a timing rule.  

Use Chart 1 or Chart 2 to determine whether the subsequent tumor is in the same branch of the histology tree.

## Slide 19

| Rule |  | Histology |  |  |  | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| M8 |  | Tumors with histology codes on different branches in Chart 1 or Chart 2 |  |  |  | Multiple |

**Notes:**
Use Chart 1 or Chart 2 to determine whether the subsequent tumor is in the same branch of the histology tree.

## Slide 20

| Rule |  | Histology |  |  |  | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| M9 |  | Tumors with histology codes different at the first (xxxx), second (xxxx), or third (xxxx) number |  |  |  | Multiple |

**Notes:**
This is a default rule. If one of the tumors is not on either Chart 1 or Chart 2, use the ICD-O-3 histology codes to determine whether they are different primaries or the same primary.

## Slide 21

| Rule |  |  |  |  | Notes/Examples | Primary |
| --- | --- | --- | --- | --- | --- | --- |
| M10 | Does not meet any of the above criteria |  |  |  | 1:  Neither timing nor laterality is used to determine multiple primaries for malignant intracranial and CNS tumors.
  Example: The patient is treated for an anaplastic astrocytoma (9401) in the right parietal lobe.  Three months later the patient is diagnosed with a separate anaplastic astrocytoma in the left parietal lobe.  This is one primary because laterality is not used to determine multiple primary status.
2:  Multi-centric brain tumors which involve different lobes of the brain that do not meet any of the above criteria are the same disease process. | Single |

**Notes:**
This is a default rule. We do not have to write multiple rules saying that these conditions are single primaries. If the case did not meet the criteria for any of the previous rules, it is a single primary.

## Histology Rules

**Notes:**
Code the histology at initial diagnosis.  Do not change the histology code when there is recurrence, reappearance, or progression.

## Single Tumor

## Slide 24

| Rule | Pathology/
Cytology |  |  | Notes/Examples | Code |
| --- | --- | --- | --- | --- | --- |
| SINGLE TUMOR |  |  |  |  |  |
| H1 | No pathology/
cytology specimen or the pathology/
cytology report is not available |  |  | 1:  Priority for using documents to code the histology
Documentation in the medical record that refers to pathologic or cytologic findings
Physician’s reference to type of cancer (histology) in the medical record
CT or MRI scans | The histology documented by the physician |

**Notes:**
Used when there was no pathology or cytology specimen and when the abstractor does not have access to the pathology or cytology report.

Gives a priority for coding histology.

--If the medical record references a pathology or cytology report. 
--Use the physician’s documentation in the medical record giving the histology. Example: H&P with a diagnosis of meduloblastoma.
--Reference to histologic type in CT or MRI scan

## Slide 25

| Rule | Pathology/ Cytology |  |  | Notes/Examples | Code |
| --- | --- | --- | --- | --- | --- |
| SINGLE TUMOR |  |  |  |  |  |
| H1
Continued | No pathology/
cytology specimen or the pathology/
cytology report is not available |  |  | 2:  Code the specific histology when documented.
3:  Code the histology to 8000 (cancer/malignant neoplasm, NOS) as stated by the physician when nothing more specific is documented | The histology documented by the physician |

**Notes:**
If any of the documentation sources mentions a specific histology, code as documented.

If the only mention is that the patient has cancer of the brain code 8000.

## Slide 26

| Rule | Pathology/Cytology Specimen |  |  | Notes/ Examples | Code |
| --- | --- | --- | --- | --- | --- |
| H2 | None from primary site  |  |  | Code the behavior /3 | The histology from metastatic site |

**Notes:**
If there is no pathology or cytology specimen from the primary site, code the histology from a biopsy of a metastatic site.

## Slide 27

| Rule |  | Histology |  |  | Code |
| --- | --- | --- | --- | --- | --- |
| H3 |  | At least two of the following cells and/or differentiation are present:
Astrocytoma
Oligodendroglioma
Ependymal |  |  | Code 9382/3 (mixed glioma) |

**Notes:**
A mixed glioma contains at least two of the three histologies
Astrocytoma
Oligodendroglioma
Ependymal

The code in this rule for mixed glioma, 9382/3 is one people have historically found problematic. Cite this rule which gives specific guidelines on the only cells or differentiation (astrocytoma, oligodendroglioma or ependymal) that can be present for this code to be used.

## Slide 28

| Rule |  | Histology |  |  | Code |
| --- | --- | --- | --- | --- | --- |
| SINGLE TUMOR |  |  |  |  |  |
| H4 |  | One type |  |  | The histology |

**Notes:**
Used when diagnosis is simply astrocytoma or glioblastoma, for example.

## Slide 29

| Rule |  | Histology |  |  | Code |
| --- | --- | --- | --- | --- | --- |
| H5 |  | Diagnosis includes a non-specific term and a specific term or type on the same branch in Chart 1 or Chart 2 |  |  | The specific type |

**Notes:**
This is a version of the previous NOS and more specific rule. You have to be careful to code the more specific ONLY when the two terms are on the same branch of the histology tree.

## Slide 30

| Rule |  |  |  |  | Code |
| --- | --- | --- | --- | --- | --- |
| H6 | None of the above conditions are met |  |  |  | The histology with the numerically higher ICD-O-3 code |

**Notes:**
This is a default rule for any cases did not meet the criteria of the first 5 rules

## Multiple Tumors Abstracted as a Single Primary

## Slide 32

| Rule | Pathology/
Cytology |  |  | Notes/Examples | Code |
| --- | --- | --- | --- | --- | --- |
| MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY |  |  |  |  |  |
| H7 | No pathology/
cytology specimen or the pathology/
cytology report is not available |  |  | 1:  Priority for using documents to code the histology
Documentation in the medical record that refers to pathologic or cytologic findings
Physician’s reference to type of cancer (histology) in the medical record
CT or MRI scans | The histology documented by the physician |

**Notes:**
Same as H1

## Slide 33

| Rule | Pathology/ Cytology |  |  | Notes/Examples | Code |
| --- | --- | --- | --- | --- | --- |
| MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY |  |  |  |  |  |
| H7
Continued | No pathology/
cytology specimen or the pathology/
cytology report is not available |  |  | 2:  Code the specific histology when documented.
3:  Code the histology to 8000 (cancer/malignant neoplasm, NOS) as stated by the physician when nothing more specific is documented | The histology documented by the physician |

**Notes:**
Same as H1

## Slide 34

| Rule | Pathology/Cytology Specimen |  |  | Notes/ Examples | Code |
| --- | --- | --- | --- | --- | --- |
| H8 | None from primary site  |  |  | Code the behavior /3 | The histology from metastatic site |

**Notes:**
Same as H2

## Slide 35

| Rule |  | Histology |  |  | Code |
| --- | --- | --- | --- | --- | --- |
| MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY |  |  |  |  |  |
| H9 |  | One type |  |  | The histology |

**Notes:**
Same as H4

## Slide 36

| Rule |  | Histology |  |  | Code |
| --- | --- | --- | --- | --- | --- |
| H10 |  | Diagnosis includes a non-specific term and a specific term or type on the same branch in Chart 1 or Chart 2 |  |  | The specific type |

**Notes:**
Same as H5

## Slide 37

| Rule |  |  |  |  | Code |
| --- | --- | --- | --- | --- | --- |
| H11 | None of the above conditions are met |  |  |  | The histology with the numerically higher ICD-O-3 code |

**Notes:**
Same as H6

## MP/H Task Force