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Home , Fibrillary astrocytoma, Medulloepithelioma, Nerve sheath tumor

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

1 Equivalent Terms, Definitions, Charts and Illustrations • Benign and borderline intracranial and CNS tumors have a separate set of rules.

2 Equivalent Terms, Definitions, Charts and Illustrations • PNET (Primitive neuroectodermal tumor) – Central PNET – Supratentorial PNET • pPNET –not brain primary

3 Chart 1 Neuroepithelial Brain CNS • WHO Classification of Tumors of the brain and central • Not complete listing

4 Chart Instructions:Use this chart to code histology. The tree is arranged Neuroepithelial in descending order. Each branch is a histology group, starting at the top (9503) with the least specific terms and descending into more specific terms.

Embryonal Ependymal Pineal Neuronal and mixed Neuroblastic Glial Oligodendroglial tumors tumors tumors tumors neuronal-glial tumors tumors tumors tumors

Ependymoma, Pineoblastoma Choroid plexus Olfactory NOS (9391) (9362) carcinoma , anaplastic (9522) NOS (9450) Oligodendroglioma (9390) (9505 Olfactory Ganglioglioma, malignant (9521) anaplastic (9451) Anasplastic (9505) Olfactory neuroepithlioma Oligodendroblastoma (9392) (9523) (9460) Papillary ependymoma (9393)

Glioma, NOS (9380) Atypical Ependymoblastoma Supratentorial primitive tetratoid/rhabdoid (9392) (9501) (9470) neuroectodermal tumor tumor (9508) (PNET) (9473)

Teratoid Demoplastic (9471) Neuroblastoma (9500) medulloepthelioma Large (9474) (9502) Medullomyoblastoma (9490) (9472)

Mixed Glial tumors of (9382) Astrocytic un certain tumors origin

Astroblastoma (9430) (9381) Pleomorphic , Pilocytic Polar spongioblastoma xanthoastrocytoma NOS (9400) astrocytoma (9423) (9421) (9424)

Anaplastic astrocytoma , NOS (9401) and Glioblastoma multiforme (9440) (9420) Gemistocytic Giant cell glioblastoma astrocytoma (9411) (9441) Protoplasmic (9442) Key:The ovals( )represent astromytoma (9410) group terms.

5 Chart 2 Non-Neuroepithelial Brain CNS • WHO Classification of Tumors of the brain and • Not complete listing

6 Non- Neuroepithelial

Peripheral Germ Cell , Tumors malignant

Malignant peripheral Choriocarcinoma (9100) Meningeal sarcomatosis (9539) (9540) Embryonal carcionoma Papillary meningioma, rhadboid Malignant peripheral nerve sheath (9070) meningioma (9538) tumor with rhabdomyoblastic Germinoma (9064) differentiation (MPNST) (9561) Immature teratoma (9080) Neurilemoma, malignant Mixed germ cell tumor (9085) (9560) Teratoma with malignant Perineurioma, malignant (9571) transformation (9084) Yolk sac tumor (9071)

7 Multiple Primary Rules

8 Unknown if Single or Multiple Tumors

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

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

11 Single Tumor

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

13 Multiple Tumors

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

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

16 Rule Histology Primary

M6 Glioblastoma or glioblastoma Single multiforme (9440) following a glial tumor (See Chart 1)

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

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

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

20 Rule Notes/Examples Primary M10 Does 1: Neither timing nor laterality is used Single not to determine multiple primaries for meet malignant intracranial and CNS tumors. any of Example: The patient is treated for an the (9401) in the above right parietal lobe. Three months later criteria 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. 21 Histology Rules

22 Single Tumor

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

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

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

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

27 Rule Histology Code SINGLE TUMOR H4 One type The histology

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

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

30 Multiple Tumors Abstracted as a Single Primary

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

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

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

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

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

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

37 MP/H Task Force

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