Malignant Meninges, Brain, Spinal Cord, Cranial Nerves, 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 nervous system • 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 Choroid plexus Neuronal and mixed Neuroblastic Glial Oligodendroglial tumors tumors tumors tumors neuronal-glial tumors tumors tumors tumors Ependymoma, Pineoblastoma Choroid plexus Olfactory neuroblastoma Oligodendroglioma NOS (9391) (9362) carcinoma Ganglioglioma, anaplastic (9522) NOS (9450) (9390) (9505 Olfactory neurocytoma Oligodendroglioma Ganglioglioma, malignant (9521) anaplastic (9451) Anasplastic ependymoma (9505) Olfactory neuroepithlioma Oligodendroblastoma (9392) (9523) (9460) Papillary ependymoma (9393) Glioma, NOS (9380) Atypical Ependymoblastoma Medulloepithelioma Medulloblastoma Supratentorial primitive tetratoid/rhabdoid (9392) (9501) (9470) neuroectodermal tumor tumor (9508) (PNET) (9473) Teratoid Demoplastic (9471) Neuroblastoma (9500) medulloepthelioma Large cell (9474) Ganglioneuroblastoma (9502) Medullomyoblastoma (9490) (9472) Mixed glioma Glial tumors of (9382) Astrocytic un certain tumors origin Astroblastoma (9430) Pleomorphic Gliomatosis cerebri (9381) Astrocytoma, Pilocytic xanthoastrocytoma Polar spongioblastoma NOS (9400) astrocytoma (9423) (9421) (9424) Anaplastic astrocytoma Glioblastoma, NOS (9401) and Glioblastoma Fibrillary astrocytoma multiforme (9440) (9420) Gemistocytic Giant cell glioblastoma astrocytoma (9411) (9441) Key:The ovals( )represent Protoplasmic Gliosarcoma (9442) group terms. astromytoma (9410) 5 Chart 2 Non-Neuroepithelial Brain CNS • WHO Classification of Tumors of the brain and central nervous system • Not complete listing 6 Non- Neuroepithelial Peripheral Germ Cell Meningioma, Nerve Tumors malignant Malignant peripheral Choriocarcinoma (9100) Meningeal sarcomatosis (9539) nerve sheath tumor (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 metastasis 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 anaplastic astrocytoma (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 cancer (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 neoplasm, 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 38.