CS Tumor Size CS Extension CS Tumor Size/Ext Eval CS Lymph Nodes CS Lymph Nodes Eval Reg LN Pos Reg LN Exam CS Mets at DX CS Mets Eval

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C70.0, C71.0-C71.9 C70.0 Cerebral meninges C71.0 Cerebrum C71.1 Frontal lobe C71.2 Temporal lobe C71.3 Parietal lobe C71.4 Occipital lobe C71.5 Ventricle, NOS C71.6 Cerebellum, NOS C71.7 Brain stem C71.8 Overlapping lesion of brain C71.9 Brain, NOS Note 1: This scheme is compatible with the AJCC fourth edition scheme TNM for brain. The AJCC opted not to recommend a TNM scheme in the sixth or seventh editions. Note 2: AJCC does not define TNM staging for this site. CS Tumor Size CS Extension CS Tumor Size/Ext Eval CS Lymph Nodes CS Lymph Nodes Eval Reg LN Pos Reg LN Exam CS Mets at DX CS Mets Eval CS Site-Specific Factor 1 WHO Grade Classification CS Site-Specific Factor 2 Proliferative Fraction- Ki-67/MIB-1 CS Site-Specific Factor 3 Function neurological status- The Karnofsky Performance Score/Scale CS Site-Specific Factor 4 MGMT - Methylation of MGMT CS Site-Specific Factor 5 Chromosome 1p: Loss of Heterozygosity (LOH) CS Site-Specific Factor 6 Chromosome 19q: Loss of Heterozygosity (LOH) CS Site-Specific Factor 7 Surgical Resection CS Site-Specific Factor 8 Focus of Primary Tumor CS Site-Specific Factor 9 CS Site-Specific Factor 10 CS Site-Specific Factor 11 CS Site-Specific Factor 12 CS Site-Specific Factor 13 CS Site-Specific Factor 14 CS Site-Specific Factor 15 CS Site-Specific Factor 16 CS Site-Specific Factor 17 CS Site-Specific Factor 18 CS Site-Specific Factor 19 CS Site-Specific Factor 20 CS Site-Specific Factor 21 CS Site-Specific Factor 22 CS Site-Specific Factor 23 CS Site-Specific Factor 24 CS Site-Specific Factor 25 The following tables are available at the collaborative staging website: Histology Inclusion Table AJCC 7th ed. Histology Exclusion Table AJCC 6th ed. AJCC TNM 7 Stage AJCC TNM 6 Stage Summary Stage 29 April 2010 Part II - Central Nervous System - 1 Version 02.02.00

CS Tumor Size (Revised: 10/07/2009) 000 No mass/tumor found 001-988 001-988 millimeters (code exact size in millimeters) 989 989 millimeters or larger 990 Microscopic focus or foci only, no size of focus given 991 Described as "less than 1 cm" 992 Described as "less than 2 cm," or "greater than 1 cm," or "between 1 cm and 2 cm" 993 Described as "less than 3 cm," or "greater than 2 cm," or "between 2 cm and 3 cm" 994 Described as "less than 4 cm," or "greater than 3 cm," or "between 3 cm and 4 cm" 995 Described as "less than 5 cm," or "greater than 4 cm," or "between 4 cm and 5 cm" 999 Unknown; size not stated CS Extension (Revised: 11/25/2009) Note 1: C71.0 is SUPRAtentorial, except the following subsites coded to C71.0 are INFRAtentorial: hypothalamus, pallium, thalamus. C71.1-C71.5 are SUPRAtentorial. C71.6-C71.7 are INFRAtentorial. The following subsites coded to C71.8 are SUPRAtentorial: corpus callosum, tapetum. The following sites coded to C71.9 are SUPRAtentorial: anterior cranial fossa, middle cranial fossa, suprasellar. The following subsites coded to C71.9 are INFRAtentorial: posterior cranial fossa. Note 2: Tumors affecting the central nervous system rarely develop extraneural metastases, probably because of inherent biologic characteristics of these tumors, and also because the brain does not have a well-developed drainage system. Tumors do spread through cerebrospinal fluid (CSF) pathways, and such spread has a major impact on survival. It is important to differentiate between extraneural metastasis and metastaisis within the CNS and CSF pathways and record distant spread in the Mets at DX table. TNM 7 TNM 6 SS77 SS2000 050 Benign or borderline brain tumors NA NA NA NA 100 Supratentorial tumor confined to: CEREBRAL HEMISPHERE (cerebrum) or MENINGES of CEREBRAL HEMI-SPHERE on one side: Frontal lobe Occipital lobe Parietal lobe Temporal lobe NA NA L L 29 April 2010 Part II - Central Nervous System - 2 Version 02.02.00

TNM 7 TNM 6 SS77 SS2000 110 Infratentorial tumor confined to: CEREBELLUM or MENINGES of CEREBELLUM on one side: Vermis: Lateral lobes Median lobe of cerebellum 120 Infratentorial tumor confined to: BRAIN STEM or MENINGES of BRAIN STEM on one side: Medulla oblongata Midbrain (mesencephalon) Pons Hypothalamus Thalamus 150 Confined to brain, NOS Confined to meninges, NOS 200 Infratentorial tumor: Both cerebellum and brain stem involved with tumor on one side 300 Confined to ventricles Tumor invades or encroaches upon ventricular system 400 Tumor crosses the midline Tumor involves contralateral hemisphere Tumor involves corpus callosum (including splenium) 500 Supratentorial tumor extends infratentorially to involve cerebellum or brain stem 510 Infratentorial tumor extends supratentorially to involve cerebrum (cerebral hemisphere) 600 Tumor invades: Bone (skull) Major blood vessel(s) Meninges (dura) Nerves, NOS Cranial nerves Spinal cord/canal 700 Circulating cells in cerebral spinal fluid (CSF) Nasal cavity Nasopharynx Posterior pharynx NA NA L L NA NA L L NA NA L L NA NA L L NA NA L L NA NA RNOS RNOS NA NA RNOS RNOS NA NA RNOS RNOS NA NA RNOS RNOS NA NA D D Outside central nervous system (CNS) 750 Extension to: Nasal cavity Nasopharynx Posterior pharyn NA NA D D 29 April 2010 Part II - Central Nervous System - 3 Version 02.02.00

TNM 7 TNM 6 SS77 SS2000 800 Further contiguous extension NA NA D D 950 No evidence of primary tumor NA NA U U 999 Unknown extension Primary tumor cannot be assessed NA NA U U CS Tumor Size/Ext Eval (Revised: 11/03/2009) Note: The staging basis for this schema is blank because AJCC stage is not applicable. Staging Basis 9 Not applicable for this schema CS Lymph Nodes (Revised: 01/13/2010) TNM 7 TNM 6 SS77 SS2000 888 OBSOLETE DATA CONVERTED V0200 See code 988 ERROR ERROR ERROR ERROR Not applicable 988 Not applicable: Information not collected for this schema NA NA U U CS Lymph Nodes Eval (Revised: 11/03/2009) Note: The staging basis for this schema is blank because AJCC stage is not applicable. Staging Basis 9 Not applicable for this schema Reg LN Pos (Revised: 06/30/2008) 99 Not applicable 29 April 2010 Part II - Central Nervous System - 4 Version 02.02.00

Reg LN Exam (Revised: 06/30/2008) 99 Not applicable CS Mets at DX (Revised: 10/07/2009) TNM 7 TNM 6 SS77 SS2000 00 No; none NA NA NONE NONE 10 Distant metastases NA NA D D 85 "Drop" metastases NA NA D D 99 Unknown Distant metastasis cannot be assessed NA NA U U CS Mets Eval (Revised: 11/03/2009) Note: The staging basis for this schema is blank because AJCC stage is not applicable. Staging Basis 9 Not applicable for this schema CS Site-Specific Factor 1 WHO Grade Classification (Revised: 10/07/2009) Note: the WHO Grade Classification as documented in the medical record. 010 Grade I 020 Grade II 030 Grade III 040 Grade IV 999 Clinically diagnosed/grade unknown Not documented in medical record Grade unknown, NOS 29 April 2010 Part II - Central Nervous System - 5 Version 02.02.00

CS Site-Specific Factor 2 Proliferative Fraction- Ki-67/MIB-1 (Revised: 12/23/2009) Note 1: Ki-67 and MIB-1 are labeling indexes used as a complementary method to differentiate better and worse prognostic groups of certain brain tumors. They evaluate the cell kinetics in brain tumors. the labeling index fraction percentage using Ki-67 or MIB-1 (monoclonal antibody) when it is available on the final diagnosis of the pathology report of the primary brain tumor. Note 2: Record the Labeling Index (LI) percentage as documented by the pathologist as an exact number (001-100). For instance, if the LI level is recorded by the pathologist as 15%, assign code 015. If percentage is documented as 13.7% round up to 14% and assign code 014. If percentage is documented as 13.2%,, round down to 13% and assign code 013. 001-100 1-100% (code exact Labeling Index percentage) Examples: 001 = 1% 010 = 10% 014 = 13.7% 055 = 55.2% 200 Labeling Index normal 300 Labeling Index stated as slightly elevated and no percentage provided 400 Labeling Index stated as elevated and no percentage provided 888 OBSOLETE DATA CONVERTED V0200 See code 988. Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 997 Test ordered, results not in chart 998 Test not done (test was not ordered and was not performed) 999 Unknown or no information 29 April 2010 Part II - Central Nervous System - 6 Version 02.02.00

CS Site-Specific Factor 3 Function neurological status- The Karnofsky Performance Score/Scale (Revised: 01/21/2010) Note 1: The Karnofsky Performance Scale (KPS) Index allows patients to be classified as to their functional impairment. This can be used to compare effectiveness of different therapies and to assess the prognosis in individual patients. The lower the Karnofsky score, the worse the survival for most serious illnesses. Note 2: Record the KPS using information in the patient's record or record the KPS as documented in the patient's record by the physician. 100 Normal no complaints; no evidence of disease 90 Able to carry on normal activity; minor signs or symptoms of disease 80 Normal activity with effort; some signs or symptoms of disease 70 Cares for self; unable to carry on normal activity normal activity or to do active work 60 Requires occasional assistance, but is able to care for most of his personal needs 50 Requires considerable assistance and frequent medical care 40 Disabled; requires special care and assistance 30 Severely disables; hospital admission is indicated although death not imminent 20 Very; hospital admission necessary; active supportive treatment necessary 10 Moribund; fatal processes progressing rapidly 0 Dead 000-100 Karnosky Performance Score (KPS) KPS Score 40 = 040 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 999 Unknown CS Site-Specific Factor 4 MGMT - Methylation of MGMT (Revised: 01/17/2010) Note 1: MGMT (O6-methylguanine-methyltransferase) is an enzyme in cells that repairs DNA. DNA repair is undesirable in tumors, because they may be able to overcome the DNA damage done by chemotherapy. With methylation, less MGMT enzyme is produced, which may lead to prolonged survival compared to unmethylated MGMT. Note 2: the methylation status of the MGMT gene (also termed MGMT promoter) as stated in the medical record. A positive test result may be termed "methylated," "hypermethylated," or "high." A negative result may be termed "unmethylated" or "low." This molecular test is not part of a routine pathology report. 010 Yes - Methylated Hypermethylated High levels 020 No - Unmethylated Low levels 29 April 2010 Part II - Central Nervous System - 7 Version 02.02.00

888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 998 No histologic examination of primary site 999 Unknown CS Site-Specific Factor 5 Chromosome 1p: Loss of Heterozygosity (LOH) (Revised: 01/21/2010) Note 1: This is a special molecular diagnostic test performed on tumor tissue to identify loss of genetic material normally found on the short arm of one of the patient's two copies of chromosome 1. A normal cell will contain two complete copies of each chromosome, one from each parent, and this normal state is termed heterozygous. Loss of heterozygosity (LOH) is an abnormal state reflecting damage to the chromosome. Note 2: Other terms for loss of heterozygosity include gene deletion and allelic loss. Note 3: For brain tumors, tests for LOH of chromosomes 1p and 19q may be performed at the same time and reported on a single report. See also Site-Specific Factor 6. 010 Test positive for loss of heterozygosity 020 Test negative for loss of heterozygosity 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 997 Test ordered, results not in chart 998 Test not done (test was not ordered and was not performed) 999 Unknown or no information 29 April 2010 Part II - Central Nervous System - 8 Version 02.02.00

CS Site-Specific Factor 6 Chromosome 19q: Loss of Heterozygosity (LOH) (Revised: 01/17/2010) Note 1: This is a special molecular diagnostic test performed on tumor tissue to identify loss of genetic material normally found on the long arm of one of the patient's two copies of chromosome 19. A normal cell will contain two complete copies of each chromosome, one from each parent, and this normal state is termed heterozygous. Loss of heterozygosity (LOH) is an abnormal state reflecting damage to the chromosome. Note 2: Other terms for loss of heterozygosity include gene deletion and allelic loss. Note 3: For brain tumors, tests for LOH of chromosomes 1p and 19q may be performed at the same time and reported on a single report. See also Site-Specific Factor 5. 010 Test positive for loss of heterozygosity 020 Test negative for loss of heterozygosity 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 997 Test ordered, results not in chart 998 Test not done (test was not ordered and was not performed) 999 Unknown or no information CS Site-Specific Factor 7 Surgical Resection (Revised: 01/21/2010) Note 1: Record the details of the surgical resection here even if previously recorded within abstract. Note 2: If case was clinically diagnosed and there was no surgical resection, code 000. Note 3: Do not code laminectomies for spinal cord primaries. Note 4: Do not record stereotactic radiosurgery as tumor destruction. It should be recorded in the radiation treatment items. 000 None; no surgery of primary site; autopsy ONLY 010 Tumor destruction, NOS No specimen sent to pathology from surgical event 10. 020 Local excision (biopsy) of tumor, lesion or mass 021 Subtotal resection of tumor, lesion or mass of brain 022 Resection of tumor of spinal cord or nerve 030 Radical, total, gross resection of tumor, lesion or mass in brain Less than half of lobe involved with tumor for 21 and 30. Specimen sent to pathology from surgical event 20, 21, 22 29 April 2010 Part II - Central Nervous System - 9 Version 02.02.00

040 Partial resection of lobe of brain 055 Gross total resection of lobe of brain (lobectomy) Tumor involves more than half of lobe for 40, 55; not used for nerve or spinal cord primary. 090 Surgery, NOS 988 Not applicable: Information not collected for this case 999 Unknown if surgery performed; death certificate ONLY CS Site-Specific Factor 8 Focus of Primary Tumor (Revised: 01/21/2010) Note 1: Record tumor focus at initial diagnosis on the resectable and unresectable cases. Unresectable cases can be determined using information recorded in the medical record (radiological imaging studies or managing physician documentation. Note 2: Use code 001 when tumor is described as unifocal; arising from or occurring in a single focus or location. Note 3: Use code 002 when tumor described as multifocal without measurement of foci, or when multiple foci of tumor are measured. 001 Unifocal/Solitary tumor 002 Multifocal tumor Tumor described as multifocal or multicentric, multiplicity counter > 01 or 99 988 Not applicable: Information not collected for this case 999 Unknown CS Site-Specific Factor 9 (Revised: 06/30/2008) CS Site-Specific Factor 10 (Revised: 06/30/2008) 29 April 2010 Part II - Central Nervous System - 10 Version 02.02.00

CS Site-Specific Factor 11 (Revised: 06/30/2008) CS Site-Specific Factor 12 (Revised: 06/30/2008) CS Site-Specific Factor 13 (Revised: 06/30/2008) CS Site-Specific Factor 14 (Revised: 06/30/2008) CS Site-Specific Factor 15 (Revised: 06/30/2008) CS Site-Specific Factor 16 (Revised: 02/23/2009) CS Site-Specific Factor 17 (Revised: 02/23/2009) 29 April 2010 Part II - Central Nervous System - 11 Version 02.02.00

CS Site-Specific Factor 18 (Revised: 02/23/2009) CS Site-Specific Factor 19 (Revised: 02/23/2009) CS Site-Specific Factor 20 (Revised: 02/23/2009) CS Site-Specific Factor 21 (Revised: 02/23/2009) CS Site-Specific Factor 22 (Revised: 02/23/2009) CS Site-Specific Factor 23 (Revised: 02/23/2009) CS Site-Specific Factor 24 (Revised: 02/23/2009) 29 April 2010 Part II - Central Nervous System - 12 Version 02.02.00

CS Site-Specific Factor 25 (Revised: 02/23/2009) 29 April 2010 Part II - Central Nervous System - 13 Version 02.02.00

C70.1, C70.9, C72.0-C72.5, C72.8-C72.9 C70.1 Spinal meninges C70.9 Meninges, NOS C72.0 Spinal cord C72.1 Cauda equina C72.2 Olfactory nerve C72.3 Optic nerve C72.4 Acoustic nerve C72.5 Cranial nerve, NOS C72.8 Overlapping lesion of brain and central nervous system C72.9 Nervous system, NOS Note: This schema is compatible with the AJCC fourth edition TNM for spinal cord. AJCC does not define TNM staging for this site in the sixth or seventh editions. CS Tumor Size CS Extension CS Tumor Size/Ext Eval CS Lymph Nodes CS Lymph Nodes Eval Reg LN Pos Reg LN Exam CS Mets at DX CS Mets Eval CS Site-Specific Factor 1 WHO Grade Classification CS Site-Specific Factor 2 Proliferative Fraction- Ki-67/MIB-1 CS Site-Specific Factor 3 Function neurological status- The Karnofsky Performance Score/Scale CS Site-Specific Factor 4 MGMT - Methylation of MGMT CS Site-Specific Factor 5 Chromosome 1p: Loss of Heterozygosity (LOH) CS Site-Specific Factor 6 Chromosome 19q: Loss of Heterozygosity (LOH) CS Site-Specific Factor 7 Surgical Resection CS Site-Specific Factor 8 Focus of Primary Tumor CS Site-Specific Factor 9 CS Site-Specific Factor 10 CS Site-Specific Factor 11 CS Site-Specific Factor 12 CS Site-Specific Factor 13 CS Site-Specific Factor 14 CS Site-Specific Factor 15 CS Site-Specific Factor 16 CS Site-Specific Factor 17 CS Site-Specific Factor 18 CS Site-Specific Factor 19 CS Site-Specific Factor 20 CS Site-Specific Factor 21 CS Site-Specific Factor 22 CS Site-Specific Factor 23 CS Site-Specific Factor 24 CS Site-Specific Factor 25 The following tables are available at the collaborative staging website: Histology Inclusion Table AJCC 7th ed. Histology Exclusion Table AJCC 6th ed. AJCC TNM 7 Stage AJCC TNM 6 Stage Summary Stage CS Tumor Size (Revised: 10/07/2009) 000 No mass/tumor found 29 April 2010 Part II - Central Nervous System - 14 Version 02.02.00

001-988 001-988 millimeters (code exact size in millimeters) 989 989 millimeters or larger 990 Microscopic focus or foci only, no size of focus given 991 Described as "less than 1 cm" 992 Described as "less than 2 cm," or "greater than 1 cm," or "between 1 cm and 2 cm" 993 Described as "less than 3 cm," or "greater than 2 cm," or "between 2 cm and 3 cm" 994 Described as "less than 4 cm," or "greater than 3 cm," or "between 3 cm and 4 cm" 995 Described as "less than 5 cm," or "greater than 4 cm," or "between 4 cm and 5 cm" 999 Unknown; size not stated CS Extension (Revised: 10/07/2009) TNM 7 TNM 6 SS77 SS2000 050 Benign or borderline brain and other parts of the CSN tumors NA NA NA NA 100 Tumor confined to tissue or site of origin NA NA L L 300 Localized, NOS NA NA L L 400 Meningeal tumor infiltrates nerve Nerve tumor infiltrates meninges (dura) 500 Adjacent connective/soft tissue Adjacent muscle 600 Brain, for cranial nerve tumors Major blood vessel(s) Sphenoid and frontal sinuses (skull) 700 Brain except for cranial nerve tumors Bone, other than skull Eye NA NA RNOS RNOS NA NA RNOS RNOS NA NA RNOS RNOS NA NA D D 800 Further contiguous extension NA NA D D 950 No evidence of primary tumor NA NA U U 999 Unknown extension Primary tumor cannot be assessed NA NA U U 29 April 2010 Part II - Central Nervous System - 15 Version 02.02.00

CS Tumor Size/Ext Eval (Revised: 11/03/2009) Note: The staging basis for this schema is blank because AJCC stage is not applicable. Staging Basis 9 Not applicable for this schema CS Lymph Nodes (Revised: 01/13/2010) TNM 7 TNM 6 SS77 SS2000 888 OBSOLETE DATA CONVERTED V0200 See code 988 ERROR ERROR ERROR ERROR Not applicable 988 Not applicable: Information not collected for this schema NA NA U U CS Lymph Nodes Eval (Revised: 11/03/2009) Note: The staging basis for this schema is blank because AJCC stage is not applicable. Staging Basis 9 Not applicable for this schema Reg LN Pos (Revised: 06/30/2008) 99 Not applicable Reg LN Exam (Revised: 06/30/2008) 99 Not applicable CS Mets at DX (Revised: 11/19/2009) TNM 7 TNM 6 SS77 SS2000 00 No; none NA NA NONE NONE 29 April 2010 Part II - Central Nervous System - 16 Version 02.02.00

TNM 7 TNM 6 SS77 SS2000 10 Distant lymph node(s), NOS NA NA D D 40 Distant metastases except distant lymph node(s) (code 10) Carcinomatosis 50 40 + 10 NA NA D D NA NA D D Other distant metastases plus distant lymph node(s) 60 Distant metastasis, NOS NA NA D D 99 Unknown if distant metastasis Distant metastasis cannot be assessed NA NA U U CS Mets Eval (Revised: 11/03/2009) Note: The staging basis for this schema is blank because AJCC stage is not applicable. Staging Basis 9 Not applicable for this schema CS Site-Specific Factor 1 WHO Grade Classification (Revised: 10/07/2009) Note: the WHO Grade Classification as documented in the medical record. 010 Grade I 020 Grade II 030 Grade III 040 Grade IV 999 Clinically diagnosed/grade unknown Not documented in medical record Grade unknown, NOS 29 April 2010 Part II - Central Nervous System - 17 Version 02.02.00

CS Site-Specific Factor 2 Proliferative Fraction- Ki-67/MIB-1 (Revised: 12/23/2009) Note 1: Ki-67 and MIB-1 are labeling indexes used as a complementary method to differentiate better and worse prognostic groups of certain brain tumors. They evaluate the cell kinetics in brain tumors. the labeling index fraction percentage using Ki-67 or MIB-1 (monoclonal antibody) when it is available on the final diagnosis of the pathology report of the primary brain tumor. Note 2: Record the Labeling Index (LI) percentage as documented by the pathologist as an exact number (001-100). For instance, if the LI level is recorded by the pathologist as 15%, assign code 015. If percentage is documented as 13.7% round up to 14% and assign code 014. If percentage is documented as 13.2%,, round down to 13% and assign code 013. 001-100 1-100% (code exact Labeling Index percentage) Examples: 001 = 1% 010 = 10% 014 = 13.7% 055 = 55.2% 200 Labeling Index normal 300 Labeling Index stated as slightly elevated and no percentage provided 400 Labeling Index stated as elevated and no percentage provided 888 OBSOLETE DATA CONVERTED V0200 See code 988. Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 997 Test ordered, results not in chart 998 Test not done (test was not ordered and was not performed) 999 Unknown or no information 29 April 2010 Part II - Central Nervous System - 18 Version 02.02.00

CS Site-Specific Factor 3 Function neurological status- The Karnofsky Performance Score/Scale (Revised: 01/21/2010) Note 1: The Karnofsky Performance Scale (KPS) Index allows patients to be classified as to their functional impairment. This can be used to compare effectiveness of different therapies and to assess the prognosis in individual patients. The lower the Karnofsky score, the worse the survival for most serious illnesses. Note 2: Record the KPS using information in the patient's record or record the KPS as documented in the patient's record by the physician. 100 Normal no complaints; no evidence of disease 90 Able to carry on normal activity; minor signs or symptoms of disease 80 Normal activity with effort; some signs or symptoms of disease 70 Cares for self; unable to carry on normal activity normal activity or to do active work 60 Requires occasional assistance, but is able to care for most of his personal needs 50 Requires considerable assistance and frequent medical care 40 Disabled; requires special care and assistance 30 Severely disables; hospital admission is indicated although death not imminent 20 Very; hospital admission necessary; active supportive treatment necessary 10 Moribund; fatal processes progressing rapidly 0 Dead 000-100 Karnosky Performance Score (KPS) KPS Score 40 = 040 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 999 Unknown CS Site-Specific Factor 4 MGMT - Methylation of MGMT (Revised: 01/17/2010) Note 1: MGMT (O6-methylguanine-methyltransferase) is an enzyme in cells that repairs DNA. DNA repair is undesirable in tumors, because they may be able to overcome the DNA damage done by chemotherapy. With methylation, less MGMT enzyme is produced, which may lead to prolonged survival compared to unmethylated MGMT. Note 2: the methylation status of the MGMT gene (also termed MGMT promoter) as stated in the medical record. A positive test result may be termed "methylated," "hypermethylated," or "high." A negative result may be termed "unmethylated" or "low." This molecular test is not part of a routine pathology report. 010 Yes - Methylated Hypermethylated High levels 020 No - Unmethylated Low levels 29 April 2010 Part II - Central Nervous System - 19 Version 02.02.00

888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 998 No histologic examination of primary site 999 Unknown CS Site-Specific Factor 5 Chromosome 1p: Loss of Heterozygosity (LOH) (Revised: 01/21/2010) Note 1: This is a special molecular diagnostic test performed on tumor tissue to identify loss of genetic material normally found on the short arm of one of the patient's two copies of chromosome 1. A normal cell will contain two complete copies of each chromosome, one from each parent, and this normal state is termed heterozygous. Loss of heterozygosity (LOH) is an abnormal state reflecting damage to the chromosome. Note 2: Other terms for loss of heterozygosity include gene deletion and allelic loss. Note 3: For brain tumors, tests for LOH of chromosomes 1p and 19q may be performed at the same time and reported on a single report. See also Site-Specific Factor 6. 010 Test positive for loss of heterozygosity 020 Test negative for loss of heterozygosity 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 997 Test ordered, results not in chart 998 Test not done (test was not ordered and was not performed) 999 Unknown or no information 29 April 2010 Part II - Central Nervous System - 20 Version 02.02.00

CS Site-Specific Factor 6 Chromosome 19q: Loss of Heterozygosity (LOH) (Revised: 01/17/2010) Note 1: This is a special molecular diagnostic test performed on tumor tissue to identify loss of genetic material normally found on the long arm of one of the patient's two copies of chromosome 19. A normal cell will contain two complete copies of each chromosome, one from each parent, and this normal state is termed heterozygous. Loss of heterozygosity (LOH) is an abnormal state reflecting damage to the chromosome. Note 2: Other terms for loss of heterozygosity include gene deletion and allelic loss. Note 3: For brain tumors, tests for LOH of chromosomes 1p and 19q may be performed at the same time and reported on a single report. See also Site-Specific Factor 5. 010 Test positive for loss of heterozygosity 020 Test negative for loss of heterozygosity 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 997 Test ordered, results not in chart 998 Test not done (test was not ordered and was not performed) 999 Unknown or no information CS Site-Specific Factor 7 Surgical Resection (Revised: 01/21/2010) Note 1: Record the details of the surgical resection here even if previously recorded within abstract. Note 2: If case was clinically diagnosed and there was no surgical resection, code 000. Note 3: Do not code laminectomies for spinal cord primaries. Note 4: Do not record stereotactic radiosurgery as tumor destruction. It should be recorded in the radiation treatment items. 000 None; no surgery of primary site; autopsy ONLY 010 Tumor destruction, NOS No specimen sent to pathology from surgical event 10. 020 Local excision (biopsy) of tumor, lesion or mass 021 Subtotal resection of tumor, lesion or mass of brain 022 Resection of tumor of spinal cord or nerve 030 Radical, total, gross resection of tumor, lesion or mass in brain Less than half of lobe involved with tumor for 21 and 30. Specimen sent to pathology from surgical event 20, 21, 22 29 April 2010 Part II - Central Nervous System - 21 Version 02.02.00

040 Partial resection of lobe of brain 055 Gross total resection of lobe of brain (lobectomy) Tumor involves more than half of lobe for 40, 55; not used for nerve or spinal cord primary. 090 Surgery, NOS 988 Not applicable: Information not collected for this case 999 Unknown if surgery performed; death certificate ONLY CS Site-Specific Factor 8 Focus of Primary Tumor (Revised: 01/21/2010) Note 1: Record tumor focus at initial diagnosis on the resectable and unresectable cases. Unresectable cases can be determined using information recorded in the medical record (radiological imaging studies or managing physician documentation. Note 2: Use code 001 when tumor is described as unifocal; arising from or occurring in a single focus or location. Note 3: Use code 002 when tumor described as multifocal without measurement of foci, or when multiple foci of tumor are measured. 001 Unifocal/Solitary tumor 002 Multifocal tumor Tumor described as multifocal or multicentric, multiplicity counter > 01 or 99 988 Not applicable: Information not collected for this case 999 Unknown CS Site-Specific Factor 9 (Revised: 06/30/2008) CS Site-Specific Factor 10 (Revised: 06/30/2008) 29 April 2010 Part II - Central Nervous System - 22 Version 02.02.00

CS Site-Specific Factor 11 (Revised: 06/30/2008) CS Site-Specific Factor 12 (Revised: 06/30/2008) CS Site-Specific Factor 13 (Revised: 06/30/2008) CS Site-Specific Factor 14 (Revised: 06/30/2008) CS Site-Specific Factor 15 (Revised: 06/30/2008) CS Site-Specific Factor 16 (Revised: 02/23/2009) CS Site-Specific Factor 17 (Revised: 02/23/2009) 29 April 2010 Part II - Central Nervous System - 23 Version 02.02.00

CS Site-Specific Factor 18 (Revised: 02/23/2009) CS Site-Specific Factor 19 (Revised: 02/23/2009) CS Site-Specific Factor 20 (Revised: 02/23/2009) CS Site-Specific Factor 21 (Revised: 02/23/2009) CS Site-Specific Factor 22 (Revised: 02/23/2009) CS Site-Specific Factor 23 (Revised: 02/23/2009) CS Site-Specific Factor 24 (Revised: 02/23/2009) 29 April 2010 Part II - Central Nervous System - 24 Version 02.02.00

CS Site-Specific Factor 25 (Revised: 02/23/2009) 29 April 2010 Part II - Central Nervous System - 25 Version 02.02.00

C75.1, C75.2, C75.3 C75.1 Pituitary gland C75.2 Cranipharyngeal duct C75.3 Pineal Gland Note 1: AJCC does not define TNM staging for this schema. CS Tumor Size CS Extension CS Tumor Size/Ext Eval CS Lymph Nodes CS Lymph Nodes Eval Reg LN Pos Reg LN Exam CS Mets at DX CS Mets Eval CS Site-Specific Factor 1 WHO Grade Classification CS Site-Specific Factor 2 Proliferative Fraction- Ki-67/MIB-1 CS Site-Specific Factor 3 CS Site-Specific Factor 4 CS Site-Specific Factor 5 CS Site-Specific Factor 6 CS Site-Specific Factor 7 CS Site-Specific Factor 8 CS Site-Specific Factor 9 CS Site-Specific Factor 10 CS Site-Specific Factor 11 CS Site-Specific Factor 12 CS Site-Specific Factor 13 CS Site-Specific Factor 14 CS Site-Specific Factor 15 CS Site-Specific Factor 16 CS Site-Specific Factor 17 CS Site-Specific Factor 18 CS Site-Specific Factor 19 CS Site-Specific Factor 20 CS Site-Specific Factor 21 CS Site-Specific Factor 22 CS Site-Specific Factor 23 CS Site-Specific Factor 24 CS Site-Specific Factor 25 The following tables are available at the collaborative staging website: Histology Inclusion Table AJCC 7th ed. Histology Exclusion Table AJCC 6th ed. AJCC TNM 7 Stage AJCC TNM 6 Stage Summary Stage CS Tumor Size (Revised: 02/03/2010) 000 No mass/tumor found 001-988 001-988 millimeters (code exact size in millimeters) 989 989 millimeters or larger 990 Microscopic focus or foci only and no size of focus given 991 Described as "less than 1 cm" 992 Described as "less than 2 cm," or "greater than 1 cm," or "between 1 cm and 2 cm" 993 Described as "less than 3 cm," or "greater than 2 cm," or "between 2 cm and 3 cm" 994 Described as "less than 4 cm," or "greater than 3 cm," or "between 3 cm and 4 cm" 29 April 2010 Part II - Central Nervous System - 26 Version 02.02.00

995 Described as "less than 5 cm," or "greater than 4 cm," or "between 4 cm and 5 cm" 999 Unknown; size not stated CS Extension (Revised: 12/10/2009) TNM 7 TNM 6 SS77 SS2000 000 In situ; non-invasive; intraepithelial NA NA IS IS 050 Benign or borderline tumors NA NA NA NA 100 Invasive carcinoma confined to gland of origin NA NA L L 300 Localized, NOS NA NA L L 400 Adjacent connective tissue (See definition in General Instructions) NA NA RE RE 600 Adjacent organs/structures NA NA RE RE 800 Further contiguous extension NA NA D D 950 No evidence of primary tumor NA NA U U 999 Unknown extension Primary tumor cannot be assessed NA NA U U CS Tumor Size/Ext Eval (Revised: 11/03/2009) Note: The staging basis for this schema is blank because AJCC stage is not applicable. Staging Basis 9 Not applicable for this schema CS Lymph Nodes (Revised: 01/19/2010) Note: 988 for Lymph nodes. TNM 7 TNM 6 SS77 SS2000 000 OBSOLETE DATA RETAINED V0200 None; no regional lymph node involvement 100 OBSOLETE DATA RETAINED V0200 Regional lymph nodes Cervical for carotid body and parathyroid only Mediastinal for aortic body and thymus only NA NA NONE NONE NA NA RN RN 29 April 2010 Part II - Central Nervous System - 27 Version 02.02.00

TNM 7 TNM 6 SS77 SS2000 800 OBSOLETE DATA RETAINED V0200 Lymph nodes, NOS NA NA RN RN 988 Not applicable NA NA U U 999 OBSOLETE DATA RETAINED V0200 Unknown; not stated Regional lymph nodes cannot be assessed NA NA U U CS Lymph Nodes Eval (Revised: 11/03/2009) Note: The staging basis for this schema is blank because AJCC stage is not applicable. Staging Basis 9 Not applicable for this schema Reg LN Pos (Revised: 12/11/2009) Note 1: Record this field even if there has been preoperative treatment. Note 2: Use code 99, not applicable, for the following sites: Pituitary gland (C75.1), Craniopharyngeal duct (C75.2), and Pineal gland (C75.3) 00 All nodes examined negative. 01-89 1-89 nodes positive (code exact number of nodes positive) 90 90 or more nodes positive 95 Positive aspiration or core biopsy of lymph node(s) 97 Positive nodes - number unspecified 98 No nodes examined 99 Unknown if nodes are positive; not applicable Reg LN Exam (Revised: 12/11/2009) Note: Use code 99, not applicable, for the following sites: Pituitary gland (C75.1), Craniopharyngeal duct (C75.2), and Pineal gland (C75.3). 00 No nodes examined 01-89 1-89 nodes examined (code exact number of regional lymph nodes examined) 29 April 2010 Part II - Central Nervous System - 28 Version 02.02.00

90 90 or more nodes examined 95 No regional nodes removed, but aspiration or core biopsy of regional nodes performed 96 Regional lymph node removal documented as sampling and number of nodes unknown/not stated 97 Regional lymph node removal documented as dissection and number of nodes unknown/not stated 98 Regional lymph nodes surgically removed but number of lymph nodes unknown/not stated and not documented as sampling or dissection; nodes examined, but number unknown 99 Unknown if nodes were examined; not applicable or negative CS Mets at DX (Revised: 11/19/2009) TNM 7 TNM 6 SS77 SS2000 00 No; none NA NA NONE NONE 10 Distant lymph node(s), NOS NA NA D D 40 Distant metastases except distant lymph node(s) (code 10) Carcinomatosis 50 40 + 10 NA NA D D NA NA D D Other distant metastases plus distant lymph node(s) 60 Distant metastasis, NOS NA NA D D 99 Unknown if distant metastasis Distant metastasis cannot be assessed NA NA U U CS Mets Eval (Revised: 11/03/2009) Note: The staging basis for this schema is blank because AJCC stage is not applicable. Staging Basis 9 Not applicable for this schema CS Site-Specific Factor 1 WHO Grade Classification (Revised: 12/10/2009) Note: the WHO Grade Classification as documented in the medical record. 010 Grade I 29 April 2010 Part II - Central Nervous System - 29 Version 02.02.00

020 Grade II 030 Grade III 040 Grade IV 999 Clinically diagnosed/grade unknown Not documented in medical record Grade unknown, NOS CS Site-Specific Factor 2 Proliferative Fraction- Ki-67/MIB-1 (Revised: 12/23/2009) Note 1: Ki-67 and MIB-1 are labeling indexes used as a complementary method to differentiate better and worse prognostic groups of certain brain tumors. They evaluate the cell kinetics in brain tumors. the labeling index fraction percentage using Ki-67 or MIB-1 (monoclonal antibody) when it is available on the final diagnosis of the pathology report of the primary brain tumor. Note 2: Record the Labeling Index (LI) percentage as documented by the pathologist as an exact number (001-100). For instance, if the LI level is recorded by the pathologist as 15%, assign code 015. If percentage is documented as 13.7% round up to 14% and assign code 014. If percentage is documented as 13.2%,, round down to 13% and assign code 013. 001-100 1-100% (code exact Labeling Index percentage) Examples: 001 = 1% 010 = 10% 014 = 13.7% 055 = 55.2% 200 Labeling Index normal 300 Labeling Index stated as slightly elevated and no percentage provided 400 Labeling Index stated as elevated and no percentage provided 888 OBSOLETE DATA CONVERTED V0200 See code 988. Not applicable for this schema 988 Not applicable: Information not collected for this case (May include cases converted from code 888 used in CSv1 for "Not applicable" or when the item was not collected. If this item is required to derive T, N, M, or any stage, use of code 988 may result in an error.) 997 Test ordered, results not in chart 998 Test not done (test was not ordered and was not performed) 999 Unknown or no information 29 April 2010 Part II - Central Nervous System - 30 Version 02.02.00

CS Site-Specific Factor 3 (Revised: 06/30/2008) 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this site CS Site-Specific Factor 4 (Revised: 06/30/2008) 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this site CS Site-Specific Factor 5 (Revised: 06/30/2008) 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this site CS Site-Specific Factor 6 (Revised: 06/30/2008) 888 OBSOLETE DATA CONVERTED V0200 See code 988 Not applicable for this site CS Site-Specific Factor 7 (Revised: 06/30/2008) 29 April 2010 Part II - Central Nervous System - 31 Version 02.02.00

CS Site-Specific Factor 8 (Revised: 06/30/2008) CS Site-Specific Factor 9 (Revised: 06/30/2008) CS Site-Specific Factor 10 (Revised: 06/30/2008) CS Site-Specific Factor 11 (Revised: 06/30/2008) CS Site-Specific Factor 12 (Revised: 06/30/2008) CS Site-Specific Factor 13 (Revised: 06/30/2008) CS Site-Specific Factor 14 (Revised: 06/30/2008) 29 April 2010 Part II - Central Nervous System - 32 Version 02.02.00

CS Site-Specific Factor 15 (Revised: 06/30/2008) CS Site-Specific Factor 16 (Revised: 02/23/2009) CS Site-Specific Factor 17 (Revised: 02/23/2009) CS Site-Specific Factor 18 (Revised: 02/23/2009) CS Site-Specific Factor 19 (Revised: 02/23/2009) CS Site-Specific Factor 20 (Revised: 02/23/2009) CS Site-Specific Factor 21 (Revised: 02/23/2009) 29 April 2010 Part II - Central Nervous System - 33 Version 02.02.00

CS Site-Specific Factor 22 (Revised: 02/23/2009) CS Site-Specific Factor 23 (Revised: 02/23/2009) CS Site-Specific Factor 24 (Revised: 02/23/2009) CS Site-Specific Factor 25 (Revised: 02/23/2009) 29 April 2010 Part II - Central Nervous System - 34 Version 02.02.00