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Malignant Meninges, Brain, Spinal Cord, Cranial Nerves, Pituitary Gland,

Craniopharyngeal Duct and Pineal Gland

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Equivalent Terms, Definitions, Charts and Illustrations

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

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Equivalent Terms, Definitions, Charts and Illustrations

• PNET (Primitive neuroectodermal tumor)– Central PNET– Supratentorial PNET

• pPNET –not brain primary

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Chart 1Neuroepithelial Brain CNS

• WHO Classification of Tumors of the brain and central nervous system

• Not complete listing

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Pilocyticastrocytoma(9421)

Giant cell glioblastoma (9441)Gliosarcoma (9442)

Glial tumors ofun certain

origin

M ixed glioma(9382)

Oligodendroglialtumors

Astrocytictumors

Oligodendroglioma NOS (9450)Oligodendrogliomaanaplastic (9451)

Oligodendroblastoma (9460)

Astroblastoma (9430)Gliomatosis cerebri (9381)Polar spongioblastoma (9423)

Neuroepithelial(9503)

Choroid plexustumors

Neuronal and mixedneuronal-glial tumors

Pinealtumors

Neuroblastictumors

Ependymoma,NOS (9391)

Ependymaltumors

Choroid plexuscarcinoma(9390)

Pineoblastoma(9362)

Olfactory neuroblastoma (9522)Olfactory neurocytoma (9521) Olfactory neuroepithlioma (9523)

Supratentorial primitiv eneuroectodermal tumor (PNET) (9473)

Neuroblastoma (9500)Ganglioneuroblastoma (9490)

Atypicaltetratoid/rhabdoidtumor (9508)

Demoplastic (9471)Large cell (9474)M edullomyoblastoma (9472)

Embryonaltumors

M edulloblastoma(9470)

Anasplastic ependymoma (9392)Papillary ependymoma(9393)

M edulloepithelioma (9501)

Ependymoblastoma (9392)

Astrocytoma,NOS (9400)

Anaplastic astrocytoma (9401)Fibrillary astrocytoma (9420)Gemistocytic

astrocytoma (9411)Protoplasmic

astromytoma (9410)

Glioblastoma, NOSand Glioblastomamultiforme (9440)

Pleomorphicxanthoastrocytoma

(9424)

Glialtumors

Ganglioglioma, anaplastic (9505Ganglioglioma, malignant (9505)

Teratoidmedulloepthelioma(9502)

Glioma, NOS (9380)

Key:The ovals( )represent group term s.

Chart Ins truc tions : Use this chart to code histology. The tree is arrangedin descending order. Each branch is a histology group, starting at the topwith the least specific terms and descending into more specific terms.

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Chart 2Non-Neuroepithelial Brain CNS

• WHO Classification of Tumors of the brain and central nervous system

• Not complete listing

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P eripheralNerve

G erm CellTum ors

M en ing iom a,m alignan t

Non-Neuroep ithelial

M alignan t peripheral nerve sheath tum or (9540)M alignan t peripheral nerve sheath tum or with rhabdom yob lastic d ifferen tiation (M P NS T) (9561)Neurilem om a, m alignan t (9560)P erineuriom a, m alignan t (9571)

Choriocarcinom a (9100)E m bryonal carcionom a (9070)G erm inom a (9064)Im m ature teratom a (9080)M ixed germ cell tum or (9085)Teratom a with m alignan t transform ation (9084)Yolk sac tum or (9071)

M en ingeal sarcom atosis (9539)P ap illary m en ing iom a, rhadboid

m en ing iom a (9538)

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Multiple Primary Rules

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Unknown if Single or Multiple Tumors

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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

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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

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Single Tumor

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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

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Multiple Tumors

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Rule Site Behavior Notes/Examples

Primary

MULTIPLE TUMORSMultiple 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

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Rule Site Primary

M5 Tumors with topography codes different at the second (Cxxx) and/or third (Cxxx) character

Multiple

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Rule Histology Primary

M6 Glioblastoma or glioblastoma multiforme (9440) following a glial tumor

(See Chart 1)

Single

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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

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Rule Histology Primary

M8 Tumors with histology codes on different branches in Chart 1 or Chart 2

Multiple

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Rule Histology Primary

M9 Tumors with histology codes different at the first (xxxx), second (xxxx), or third (xxxx) number

Multiple

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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

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Histology Rules

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Single Tumor

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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 histologyDocumentation in the medical record that refers to pathologic or cytologic findingsPhysician’s reference to type of cancer (histology) in the medical recordCT or MRI scans

The histology documented by the physician

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Rule Pathology/ Cytology

Notes/Examples Code

SINGLE TUMOR

H1Continued

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

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Rule Pathology/Cytology Specimen

Notes/ Examples

Code

H2 None from primary site

Code the behavior /3

The histology from metastatic site

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Rule Histology Code

H3 At least two of the following cells and/or differentiation are present:AstrocytomaOligodendrogliomaEpendymal

Code 9382/3 (mixed glioma)

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Rule Histology Code

SINGLE TUMOR

H4 One type The histology

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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

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Rule Code

H6 None of the above conditions are met

The histology with the numerically higher ICD-O-3 code

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Multiple Tumors Abstracted as a Single Primary

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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 histologyDocumentation in the medical record that refers to pathologic or cytologic findingsPhysician’s reference to type of cancer (histology) in the medical recordCT or MRI scans

The histology documented by the physician

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Rule Pathology/ Cytology

Notes/Examples Code

MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY

H7Continued

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

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Rule Pathology/Cytology Specimen

Notes/ Examples

Code

H8 None from primary site

Code the behavior /3

The histology from metastatic site

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Rule Histology Code

MULTIPLE TUMORS ABSTRACTED AS A SINGLE PRIMARY

H9 One type The histology

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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

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Rule Code

H11 None of the above conditions are met

The histology with the numerically higher ICD-O-3 code

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MP/H Task Force