Data Collection of Primary Intracranial and Central Nervous ...

Data Collection of Primary Intracranial and Central Nervous ...

Data Collection of Primary Central Nervous System (CNS) Tumors DEPARTMENT OF HEALTH AND HUMAN SERVICES CENTERS FOR DISEASE CONTROL AND PREVENTION Atlanta, Georgia, USA 1 Portions of this presentation are based on non-malignant CNS tumor data collection rules adopted by the North American Association of Central Cancer Registries (NAACCR) Uniform Data Standards Committee - June 2003.

2 Part I Rationale History Definition of Reportable Cases Casefinding Anticipated Impact on Registries 3 Rationale for Non-malignant CNS Tumor Surveillance and

Registration Non-malignant CNS tumors cause disruption in normal function similar to that caused by malignant CNS tumors. Location of a CNS tumor is as important as tumor behavior (benign or malignant) to morbidity and mortality. 4 History 1992 -1996 1992 Central Brain Tumor Registry of the United States (CBTRUS) formed to report population-based data on primary benign, borderline, and

malignant CNS tumors. 1996 National Coordinating Council on Cancer Surveillance (NCCCS) formed Brain Tumor Working Group (BTWG) to explore the feasibility of registering nonmalignant CNS tumors 5 History 1998 BTWG forwarded four recommendations to the NCCCS

NCCCS Accepted recommendations 1 and 2 Deferred recommendations 3 and 4 6 BTWG Recommendations (1) 1. The following standard definition is to be used for collecting precise data for all primary intracranial and CNS tumors: Primary intracranial and CNS tumors are all primary tumors occurring in the following sites, irrespective of histologic type or behavior:

Brain Meninges Spinal cord Cauda Pituitary gland equina Craniopharyngeal Pineal gland duct Cranial nerves and other parts of the CNS. 7

BTWG Recommendations (2) 2. Develop a standard site and histology definition for tabulating estimates of CNS tumors to allow comparability of information across registries. 3. All registries, both hospital- and population-based, should collect data on primary CNS tumors. 8 BTWG Recommendations (3) 4. Develop training for reporting and tabulating primary intracranial and CNS

tumors, and develop computerized edit- checking procedures. 9 History 2000 International Classification of Diseases for Oncology 3rd Edition (ICD-O-3) and World Health Organization (WHO) 2000 Brain Tumor Classification are compatible. November Consensus conference on brain tumor definition convened. Group agrees to:

Site definition as in Recommendation 1. Need to develop a standard site and histology definition based on the SEER site and histology validation list. 10 History 2001-2002 2001 NCCCS Accepted Recommendations 1 and 2 as completed. Reconvened the BTWG to work on Recommendations 3 and 4. 2002 NAACCR established subcommittee of Registry Operations Committee to: Identify changes needed in registry

operations for inclusion of nonmalignant CNS tumors. October: Benign Brain Tumor Cancer Registries Amendment Act (Public Law 107-260) signed by President Bush. 11 Reportable Brain-Related Tumors (1) Public Law 107-260 requires reporting of brain-related tumors. The term brain-related tumor means a listed primary tumor (whether malignant or benign) occurring in any of the following sites: (I) The brain, meninges, spinal cord,

cauda equina, a cranial nerve or nerves, or any other part of the CNS. (II) The pituitary gland, pineal gland, or craniopharyngeal duct. 12 Reportable Brain-Related Tumors (2) Brain Cerebrum (C71.0) Frontal lobe (C71.1) Temporal lobe (C71.2) Parietal lobe (C71.3) Occipital lobe

(C71.4). 13 Reportable Brain-Related Tumors (3) Brain (continued) Ventricle (C71.5) Cerebellum (C71.6) Brain stem (C71.7) Overlapping lesion of the brain (C71.8) Brain NOS (C71.9) 14

Reportable Brain-Related Tumors (4) Meninges Cerebral meninges (C70.0) Spinal meninges (C70.1) Meninges NOS (C70.9) Spinal cord (C72.0) Cauda equina (C72.1) 15 Reportable Brain-Related Tumors

(5) Cranial nerves Olfactory nerve (C72.2) Optic nerve (C72.3) Acoustic nerve (C72.4) Cranial nerve NOS (C72.5) 16 Reportable Brain-Related Tumors (6) Other CNS (C72.8, C72.9) Pituitary gland (C75.1) Craniopharyngeal duct (C75.2)

Pineal gland (C75.3) For the sites described, benign, borderline, and malignant tumors are reportable for cases diagnosed on or after January 1, 2004. 17 History 2003 2003 SEER-supported registries and COC-approved hospital cancer registries will also report nonmalignant CNS tumors diagnosed on or after January 1, 2004. 18

Impact of Collecting Data on Non-malignant CNS Tumors (1) Annual increase in number of cases estimated by doubling the number of malignant CNS cases diagnosed in the same year Increase in hospital registry case load will depend on the type of hospital: Community hospitals with small or no neurology service will likely experience a small increase in case load. Hospitals with a large neurology service will likely experience a larger increase. 19

Impact of Collecting Data on Non-malignant CNS Tumors (2) Central registry case load is estimated to increase by 1%. In 2002, 21 state cancer registries collected data on non-malignant CNS tumors: Minimal impact if registrys definition for brain-related sites does not change. 20 Impact of Collecting Data on Non-malignant CNS Tumors (3) Central registries adding non-malignant

CNS tumors to reportable case definition may have to change state reporting law if law does not allow for collection of data on non-malignant cases. 21 Impact of Collecting Data on Non-malignant CNS Tumors (4) All cancer registries must: Have the same definition for brain-related tumors. Implement data edits created for nonmalignant CNS tumors. Report rates for these tumors.

22 Case-finding (1) Additional or expanded case-finding mechanisms: Pathology Radiology Treatment facilities: Radiation oncology centers and departments Gamma or cyber knife center. 23 Case-finding (2)

Disease indices Surgery logs Diagnostic imaging Radiation oncology Neurology clinics Medical oncology Autopsy reports.

24 Case-finding Sources Free-standing radiation therapy centers Free-standing Magnetic Resonance Imaging (MRI) centers Free-standing gamma or cyber knife centers Free-standing oncology centers Data exchange with other central registries Death clearance process 25

ICD-9-CM Codes for Casefinding Table 1: ICD-9-CM Casefinding Codes for Benign and Borderline Intracranial and CNS Tumors ICD-9-CM Description of Neoplasm Code 225.0 Benign neoplasm of brain 225.1 Benign neoplasm of cranial nerves 225.2 Benign neoplasm of cerebral meninges; cerebral meningioma 225.3 Benign neoplasm of spinal cord, cauda equina 225.4

Benign neoplasm of spinal meninges; spinal meningioma 225.8 Benign neoplasm of other specified sites of nervous sytem 225.9 Benign neoplasm of nervous system, part unspecified 227.3 Benign neoplasm of pituitary, craniopharyngeal duct, craniobuccal pouch, hypophysis, Rathkes pouch, sella turcica 227.4 Benign neoplasm of pineal gland, pineal body 237.0 Neoplasm of uncertain behavior of pituitary gland and craniopharyngeal duct 237.1 Neoplasm of uncertain behavior of pineal gland

237.5 Neoplasm of uncertain behavior of brain and spinal cord 237.6 Neoplasm of uncertain behavior of meninges: NOS, cerebral, spinal 237.70 Neurofibromatosis, Unspecified von Recklinghausens Disease 237.71 Neurofibromatosis, Type One von Recklinghausens Disease 237.72 Neurofibromatosis, Type Two von Recklinghausens Disease 237.9 Neoplasm of uncertain behavior of other and unspecified parts of nervous system; cranial nerves 26

Unusual and Ambiguous Terminology If the final pathologic diagnosis is a CNS neoplasm or mass, an ICD-O3 histology code must exist for the case to be reportable. Hypodense mass or cystic neoplasm are not reportable, even for CNS sites. A benign meningioma with a skull site should be coded to the cerebral meninges (C70.1). 27 Part II CNS Anatomy and Function

Histologies and Primary Sites Grading Systems and Coding Grade 28 CNS Functional Anatomy Source: URL: www.solinas.com/solinas/brain.html accessed 7/18/03. 29 CNS Anatomy C71.0

C71.6 C71 C75.3 C75.1 C71.7 C71.7 C72.0 Source: URL: www.universalpeace.ca/principles.htm accessed 7/18/03. 30

Intracranial Sites Parietal lobe C41.0 C71.0 Frontal lobe C71.6 C71.7 C72.0 Source: URL: mscenter.ucsf.edu/faq.htm accessed 7/18/03. 31

Cerebrum C71.0 C71.3 C71.1 C71.4 C71.2 C71.6 C71.7 Source: URL: www.sciencebob.com/lab/bodyzone/brainprint.html Accessed 7/18/03.

32 Cerebellum and Brain Stem C71.0 C71.3 C71.1 C71.4 C71.6 C71.2 C71.7

URL: www.sciencebob.com/lab/bodyzone/brain.html 7/18/03 33 The Ventricular System http://www.abta.org/primer2.htm 34 Pineal and Pituitary Glands C75.3 C71.6

C75.1 C71.7 C72.0 Source: URL: training.seer.cancer.gov/module_anatomy/unit6_3_endo_gl Accessed 7/18/03. 35 Cranial Nerves I=C72.2, II=C72.3, VIII=C72.4, Others=C72.5 Source: URL: faculty.washington.edu/chudler/cranial.html Accessed

7/18/03. 36 Meninges C71.0 C70.0 C70.0 Source: URL: 37 www.cardioliving.com/consumer/Stroke/Hemorrhagic_Stroke.sht Accessed

Tentorium C70.0 C70.0 Source: URL: neurosurgery.mgh.harvard.edu/abta/primer.htm Accessed 7/18/03. 38 Spinal Cord C70.1 C72.0

Source: URL: www.merck.com/pubs/mmanual/figures/182fig1.htm Accessed 7/18/03 39 Cellular Classification Neuroepithelial tumors Astrocytomas

Oligodendrogliomas Ependymomas Pineal parenchymal tumors Other CNS tumors Sellar tumors Hematopoetic tumors Germ cell tumors Meningiomas

Tumors of cranial nerves 40 Glial Tumors (1) Glial tissue: supportive tissue of brain made up of astrocytes and oligodendrocytes Glial tumors assigned ICD-O-3 histology codes from glioma series: Codes 938 through 948. 41 Glial Tumors (2) Astrocytic tumors

Noninfiltrating Juvenile pilocytic (M9421) Subependymal (M9383) Infiltrating Well-differentiated mildly and moderately anaplastic astrocytomas (M9401) Anaplastic astrocytomas Glioblastoma multiforme (M9440) Brain stem gliomas (M9380) 42 Glial Tumors (3) Ependymal tumors Myxopapillary and well-differentiated

ependymomas (M9394) Anaplastic ependymomas (M9392) Ependymoblastomas (M9392) Oligodendroglial tumors Well-differentiated oligodendrogliomas (M9450) Anaplastic oligodendrogliomas (M9451) 43 Glial Tumors (4) Mixed tumors Mixed astrocytoma-ependymomas Mixed astrocytoma-oligodendrogliomas Mixed astrocytoma-ependymomaoligodendrogliomas

Other gliomas Ganglioneuromas (M9490) Optic nerve gliomas 44 Non-Glial Tumors (1) Pineal region tumors Parenchymal tumors Pineocytomas (M9361) Pineoblastomas (M9362) Pineal astrocytomas (M9400) Germ cell tumors

Germinomas (M9064) Embryonal carcinomas (M9070) Choriocarcinomas (M9100) Teratomas (M9080) 45 Non-Glial Tumors (2) Meningiomas Meningioma: Benign (M953_) Malignant meningiomas Anaplastic meningioma Hemangiopericytoma (M9150) Papillary meningioma (M9538) Choroid plexus tumors

Choroid plexus papilloma (M9390) Choroid plexus carcinoma Choroid plexus meningioma (M9538) 46 Other CNS Tumors (1) Craniopharyngiomas (M9350) Rathke pouch tumors Chordomas (M9370) Schwannomas (M9560) Acoustic schwannomas/neuromas 47

Other CNS Tumors (2) Embryonal tumors Retinoblastomas (M9510) Primitive neuroectodermal tumors (PNETs) Meduloblastomas (M9470) Neuroblastomas (M9500) 48 Other CNS Tumors (3) Lymphomas (M9590) Arise from Indigenous brain histiocytes (microglia) Rare lymphocytes in meninges

High incidence in patients with AIDS Vascular tumors Rare, non-malignant tumors Arise from blood vessels of brain and spinal cord Hemangioblastoma (M9161) most common vascular tumor 49 Other CNS Tumors (4) Cysts and tumor-like lesions Reportable

Dermoid cysts (M9084) Granular cell tumors (M9580) Rathke pouch tumors (M9350) Not reportable Epidermoid cysts Colloid cysts Enterogenous cysts Neuroglial cysts Plasma cell granulomas Nasal glial herterotopias Rathke cleft cysts 50

Childhood versus Adult Tumors CNS tumor histology and location are different in adult and children. Tumor location and extent of spread affect treatment and prognosis. Most common solid tumor in childhood. 51 Childhood Brain Tumors Meduloblastomas are the most common CNS histology in children. 50% are infratentorial. Common infratentorial tumors:

Cerebellar astrocytomas Meduloblastomas Ependymomas Brain stem gliomas Atypical teratoid tumors 52 Cellular Classification Childhood Brain Tumors (1) Supratentorial tumors in children Craniopharyngiomas Germ cell tumors Diencephalic and hypothalamic gliomas Low grade

astrocytomas Mixed gliomas Anaplastic astrocytomas Oligodendrogliomas PNETs Meningiomas Glioblastoma multiforme Low-grade or anaplastic ependymomas Choroid plexus tumors Pineal parenchymal tumors

Gangliogliomas Desmoplastic infantile gangliogliomas Dysembryoplastic neuroepithelial tumors 53 Cellular Classification Childhood Brain Tumors (2) The histopathology of childhood spinal tumors is the same as for childhood brain tumors. Primary spinal cord tumors comprise approximately 1% to 2% of all childhood CNS tumors.

54 Cellular Classification Childhood CNS Tumors Cause of childhood CNS tumors remains unknown. American Academy of Pediatrics has outlined guidelines for pediatric cancer centers and their role in the treatment of pediatric cancer patients. 55 ICD-O-3 Coding Issues (1) Some histologies may be difficult to

determine if the primary site is intracranial or the skull (C41.0). Non-malignant tumors of the skull are not reportable. Chondroma (M9220/0) must originate in a brain-related site to be reportable. Chordoma (M9370/3) and chondrosarcoma (M9220/3) are malignant. Tumors in brain-related sites are analyzed separately from those in the skull. 56

ICD-O-3 Coding Issues (2) Continue to assign histology code M9421/3 to pilocytic astrocytoma. When the primary site for intracranial schwannoma (9560/0) is not documented in source documents, the site should be coded to cranial nerves NOS (C72.5). 57 Grade for CNS Tumors Sixth digit of ICD-O-3 histology code Describes tumor differentiation or grade. Is not usually specified for CNS tumors. Is always assigned code 9 for nonmalignant CNS tumors:

Not determined, not stated, or not applicable. Per ICD-O-3, page 30, Rule G, paragraph 1 Only malignant tumors are graded. Not the same as WHO grade. 58 WHO Grade (1) WHO grade coded in Collaborative Stage data field: Site-specific factor 1 for Brain. Four-category tumor grading system Grade I

Slow growing Non-malignant tumors Patients have long-term survival. 59 WHO Grade (2) Grade II Relatively slow growing Sometimes recur as higher grade tumors May be non-malignant or malignant . Grade III

Malignant tumors Often recur as higher grade tumors. Grade IV Highly malignant and aggressive. 60 Kernohan Grade Defines progressive malignancy for astrocytoma Grade Grade Grade Grade

1: 2: 3: 4: benign astrocytomas low-grade astrocytomas anaplastic astrocytomas glioblastoma multiforme No NAACCR data field for Kernohan grade. 61 St. Anne-Mayo Grade (1)

Used for astrocytomas. Uses four morphologic criteria: Nuclear atypia Mitosis Endothelial proliferation Necrosis No NAACCR data field for the St. Anne-Mayo grade. 62 St. Anne-Mayo Grade (2) Grade 1: No criteria Grade 2: One criterion, usually nuclear

atypia Grade 3: Two criteria, usually nuclear atypia and mitosis Grade 4: Three or four criteria 63 Grade for CNS Tumors Do not record WHO grade, Kernohan grade, or St. Anne/Mayo grade in the sixth digit histology code data field

64 Part III Laterality Multiple Primaries Malignant Transformation Sequence Numbers Date of Diagnosis 65 Determining Multiple Primaries: Laterality

Brain is not a paired organ. Laterality collected on both nonmalignant and malignant tumors. Used to determine if multiple nonmalignant CNS tumors are counted as multiple primary tumors. Not used to determine if multiple malignant tumors of the same intracranial or CNS site are multiple primary tumors. 66 Coding Laterality (1) CNS sites to be coded with laterality: Cerebral meninges, NOS (C70.0)

Cerebrum (C71.0) Frontal lobe (C71.1) Temporal lobe (C71.2) Parietal lobe (C71.3) Occipital lobe (C71.4). 67 Coding Laterality (2) CNS sites to be coded with laterality (continued): Olfactory nerve (C72.2) Optic nerve (C72.3) Acoustic nerve (C72.4) Cranial nerve, NOS (C72.5)

68 Determining Multiple Primaries: Definitions Non-malignant tumor Tumor with ICD-O-3 behavior code 0 (benign) or 1 (borderline). CNS Includes intracranial and central nervous system topographic sites. 69

Determining Multiple Primaries Malignant (1) NO CHANGES (at this time) Site Rule: Each category (first three characters) as delineated in ICD-O-3 is considered to be a separate site. Multiple tumors are: Same: C71.0 Cerebrum, C71.2 Temporal lobe Different: C70.0 Cerebral Meninges,

C71.0 Cerebrum 70 Determining Multiple Primaries: Malignant (2) Histology Rule: Differences in histologic type refer to differences in the FIRST THREE digits of the morphology code. Multiple tumors in the same site are:

Same: Choroid plexus carcinoma (M9390), Ependymoma (M9391) Different: Astrocytoma (M9400), Gemistocytic astrocytoma (M9411) 71 Determining Multiple Primaries Non-malignant (1) NEW RULES Site Rule: Each sub-site (fourth-digit level) as delineated in ICD-O-3 is considered a

separate site. Same site if separate tumors with the same histology are in the same sub-site. Different site if separate tumors have the same histology in different sub-site C71.1 Frontal lobe, C71.4 Occipital lobe C70.0 Cerebral Meninges, C70.1 Spinal meninges. 72 Determining Multiple Primaries Non-malignant (2) Site (cont) EXCEPT NOS (C_ _.9) with specific

four-digit site code in same rubric Example: meninges, NOS (C70.9) with spinal meninges (C70.1) or cerebral meninges (C70.0). 73 Determining Multiple Primaries Non-malignant (3) Site (cont)

Laterality: For non-malignant cases only If multiple tumors of the same site and same histologic type are identified and both sides of a site listed as lateral are involved, tumors should be counted as separate primaries. Different: Right temporal lobe (C71.2) and left temporal lobe (C71.2) 74 Determining Multiple Primaries: Non-malignant

(4) Histology Table 2: Histologic Groupings to Determine Same Histology for Non-malignant Brain Tumors Choroid plexus neoplasms 9390/0, 9390/1 Ependymomas 9393, 9394, 9444 Neuronal and neuronal-glial 9384, 9412, 9413, 9442, 9505/1, neoplasms 9506 Neurofibromas 9540/0,9540/1, 9541, 9550, 9560/0

Neurinomatosis 9560/1 Neurothekeoma 9562 Neuroma 9570 Perineurioma, NOS 9571/0 75 Determining Multiple Primaries: Non-malignant (5)

Histology If multiple tumors are in the same site, refer to Table 2, and use the following rules in priority order: A-1: If the first three digits are the same but the codes are not found in Table 2, then the histology is considered to be the SAME. A-2: If the first three digits are different but the codes are not found in Table 2, then the histology is considered to be DIFFERENT. 76

Determining Multiple Primaries: Non-malignant (6) Histology (cont.) B. If all histologies are listed in the same histologic group in Table 2, then the histology is considered to be the SAME. * Example: Ependymomas: M9394, Myxopapillary ependymoma and M9444, Chordoid glioma have the same histology *Note: If two histologies are in the same group in Table 2, code the first or more specific histology.

77 Determining Multiple Primaries: Non-malignant (7) Histology (cont) C: If the first three digits are the same as the first three digits for any histology in one of the groupings in Table 2 , then the histology is considered to be the SAME.* Example: On table: Neuronal and neurol-glial neoplasm: M9505, ganglioglioma, Not on

table: M9507, Pacinian tumor * Note: If two histologies are in the same group in Table 2, code the first or more specific histology. 78 Determining Multiple Primaries: Non-malignant (8) Histology (cont) D: If the first three digits are the same and the histologies are from two different groups in the histologic groupings table,

the histologies are considered to be DIFFERENT. Example: Gliomas: M9442, Gliofibroma; Ependymoma: M9444, Chordoid glioma 79 Determining Multiple Primaries: Timing (1) Primary malignant CNS tumors NO CHANGE Malignant tumors of the same site and same histology, diagnosed within 2

months: Tumors are counted as the SAME primary. Malignant tumors of the same site and same histology, diagnosed more than 2 months apart: Tumors are counted as DIFFERENT primary sites. 80 Determining Multiple Primaries: Timing (2) Primary non-malignant CNS

tumors NEW No timing rule If a new non-malignant tumor of the same histology as an earlier tumor that had been diagnosed in the same site is diagnosed subsequently at any time, it is considered to be the SAME primary tumor. 81 General Rules for Determining Multiple Primaries of CNS Sites (1) Multiple lesions: all non-malignant

1. If different sites, then DIFFERENT primaries. 2. If different histologies, then DIFFERENT primaries. 82 General Rules for Determining Multiple Primaries of CNS Sites (2) Multiple lesions: all non-malignant (cont.) 3. If same site and same histology: a. Laterality is same side, one side unknown or not applicable, then

SAME primary. b. Laterality is both sides, then DIFFERENT primaries. 83 General Rules for Determining Multiple Primaries of CNS Sites (3) B. Multiple tumors: One nonmalignant and one malignant 1. Non-malignant tumor followed by malignant tumor: DIFFERENT primaries, regardless of timing. 2. Malignant tumor followed by a nonmalignant tumor: DIFFERENT primaries, regardless of timing. 84

Histologic Transformation (1) Histologic transformation or progression to a higher grade: Determined by pathological review. Final diagnosis made by review of previous biopsies or excisions and comparison to newly biopsied or resected brain tumor Non-malignant tumor transforms to malignant tumor. Malignant tumors transforms to higher grade tumor. 85

Histologic Transformation (2) If a malignant CNS tumor recurs (transforms) as a higher grade tumor, SAME tumor. Do not change the histology or grade. Do not abstract as new primary. Example: Astrocytoma (M9400) transforms to glioblastoma multiforme (M9440). 86 Histologic Transformation (3) Transformation of a non-malignant tumor to a malignant tumor is rare. Malignant transformations include:

Changes from WHO grade I to WHO grade II, III, or IV. Changes from behavior code 0 or 1 to code 2 or 3. Complete two abstracts: One for the non-malignant tumor One for the malignant tumor 87 Histologic Transformation (4) Sequence Numbers Non-malignant tumors: assigned sequence numbers from the reportable-by-agreement series.

Malignant tumors: assigned sequence numbers from the malignant series. Example: Patient has a non-malignant CNS tumor that progressed into a malignant CNS tumor: Non-malignant tumor is sequenced as 60. Malignant tumor is sequenced as 00. 88 Histologic Transformation (5) Date of Diagnosis Non-malignant tumors: First date that a medical practitioner diagnosed the non-malignant tumor either

clinically or histologically. Malignant tumors: First date that a medical practitioner diagnosed the malignant transformation either clinically or histologically. 89 Coding Sequence Numbers (1) Indicates the sequence of all reportable neoplasms over the lifetime of the person. Codes 00 35: Malignant and in situ reportable neoplasms. Codes 60 87: Reportable-byagreement including non-malignant

tumors diagnosed after January1, 2004. 90 Coding Sequence Numbers (2) Reportable-by-agreement neoplasms are defined by each facility and/or central cancer registry: Non-malignant CNS tumors are assigned reportable-by-agreement sequence numbers even when they are reportable. Codes 60 87 91

Coding Sequence Numbers (3) Sequence numbers for non-malignant CNS tumors are assigned over the lifetime of the person. Example: Patient diagnosed with a nonmalignant CNS tumor in January, 2003 (not reportable by state or hospital reporting rules) and diagnosed with second non-malignant CNS tumor in 2004: Second is sequence number 62. Complete abstract for the second tumor only. 92 Assigning Diagnosis Date

Rules for assigning diagnosis date are the same for malignant and non-malignant tumors. Review source records carefully to determine initial diagnosis date, regardless of whether it is a clinical or histological diagnosis. 93 Part IV Staging Risk Factors Genetic Syndromes Diagnostic Tools Treatment

Edits Data Analysis 94 Collaborative Stage (CS) A computer algorithm uses the collaborative stage (CS) data fields to calculate site-specific American Joint Committee on Cancer (AJCC) TNM stage, SEER Summary Stage 1977, and SEER Summary Stage 2000. 95 Coding Collaborative Stage

(1) Separate sets of extension codes for: Brain and cerebral meninges Other parts of the CNS Glands: pituitary gland, craniopharyngeal duct, and pineal gland. 96 Coding Collaborative Stage (2) Site-specific codes for lymph nodes Same for the Brain, cerebral meninges

and other CNS. Code 88: Not applicable. For pituitary gland, craniopharyngeal duct, and pineal gland Code 99: Not applicable. Metastasis at Diagnosis Same for the pituitary gland, craniopharyngeal duct, and pineal gland and other CNS. Different for brain and cerebral meninges. 97 CS Extension: Brain and

Meninges C70.0, C71.0 C71.9 (1) 05 Benign or borderline brain tumors 10 Supratentorial tumor confined to CEREBRAL HEMISPHERE (cerebrum) or MENINGES of cerebral hemisphere one side: frontal lobe, occipital lobe, parietal lobe, or temporal lobe 11 Infratentorial tumor confined to CEREBELLUM or MENINGES of CEREBELLUM on one side: Vermis, lateral lobes, median lobe of cerebellum 98 CS Extension: Brain and

Meninges C70.0, C71.0 C71.9 (2) 12 15 20 30 Infratentorial tumor confined to BRAIN STEM or MENINGES of BRAIN STEM on one side: medulla oblongata, midbrain (mesencephalon), pons, hypothalamus, or thalamus Confined to brain, NOS, Confined to meninges, NOS

Infratentorial tumor: Both cerebellum and brain stem involved with tumor on one side Confined to ventricles - Tumor invades or encroaches upon ventricular system 99 CS Extension: Brain and Meninges C70.0, C71.0 C71.9 (3) 40 50 51 60

Tumor crosses the midline: involves the contralateral hemisphere, involves corpus callosum (including splenium) Supratentorial tumor extends infratentorially to involve cerebellum or brain stem Infratentorial tumor extends supratentorially to involve cerebrum (cerebral hemisphere) Tumor invades bone (skull), major blood vessel(s), meninges (dura), nerves, NOS (cranial nerves), or spinal cord/canal 100

CS Extension: Brain and Meninges C70.0, C71.0 C71.9 (4) 70 80 95 99 Circulating cells in cerebral spinal fluid; nasal cavity; nasopharynx; posterior pharynx; or outside CNS Further contiguous extension No evidence of primary tumor Unknown extension; Primary tumor cannot be accessed; Not documented

in patient record 101 CS Extension: Other CNS C70.1-9, C72.0C72.9 (1) Spinal meninges, meninges NOS Spinal cord Caudia equina Olfactory, acoustic, cranial nerve, NOS Overlapping brain and CNS Nervous system, NOS 102

CS Extension: Other CNS C70.1-9, C72.0C72.9 (2) 05 10 30 40 50 60 Benign or borderline tumors Tumor confined to tissue or site of origin Localized, NOS Meningeal tumor infiltrates nerve; nerve tumor infiltrates meninges (dura) Adjacent connective/soft tissue;

adjacent muscle Brain, for cranial nerve tumors; major blood vessel(s); sphenoid and frontal sinuses (skull) 103 CS Extension: Other CNS C70.1-9, C72.0C72.9 (3) 70 80 95 99 Brain except for cranial nerve tumors; bone, other than skull; eye

Further contiguous extension No evidence of primary tumor Unknown extension; primary tumor cannot be assessed; not documented in patient record 104 CS Extension: Other Endocrine C75.1, C75.2, C75.3 00 05 10 30 40

60 80 95 99 In situ; non-invasive; intraepithelial Benign or borderline tumors Invasive carcinoma confined to gland of origin Localized, NOS Adjacent connective tissue Pituitary and craniopharyngeal duct: Cavernous sinus; infundibulum; pons; sphenoid body and siunses

Pineal: Infratentorial and central brain Further contiguous extension No evidence of primary tumor 105 Unknown extension CS Lymph Nodes Describes tumor involvement of regional lymph nodes. Code for CS Lymph Nodes is 88 (not applicable) for meninges, brain, spinal cord, cranial nerves, and other parts of the CNS. Code for CS Lymph Nodes is 99 (unknown, not stated) for pituitary

gland, craniopharyngeal duct, and pineal gland. 106 CS Metastasis at Diagnosis Brain and Meninges C70.0, C71.0-9 00 10 85 99 No; None Distant metastases Drop metastases

Unknown; distant metastasis cannot be assessed; not documented in patient record 107 CS Metastasis at Diagnosis Other CNS and Other Endocrine C70.1-9, C72.09, C75.1, C75,2, 00 10 40 50 99

No; None C75.3 Distant lymph node(s) Distant metastasis except lymph nodes (code 10) Distant metastasis, NOS Carcinomatosis (40) + (10) Unknown; distant metastasis cannot be assessed; not documented in patient record 108 CS Site-specific Factor 1 (1) C70.0-C70.9, C71.0-C71.9, C72.0-C72.9

010 020 030 040 999 WHO Grade I WHO Grade II WHO Grade III WHO Grade IV Clinically diagnosed; grade unknown; Not documented in the medical record; Grade unknown, NOS 109

CS Site-specific Factor 1 (2) C70.0-C70.9, C71.0-C71.9, C72.0-C72.9 C75.1- C75.3 Code the WHO grade for CNS tumors in CS Site-specific factor 1. Do not code WHO grade in the sixth digit histology data field. 110 Possible Risk Factors Genetic predispositions for the development of brain tumors have been identified.

Population-based studies suggest that no more than 4% are attributed to heredity. Several environmental factors that may be associated with CNS tumors. 111 Possible Risk Factors Epstein-Barr virus in the DNA of primary lymphoma suggests a viral etiology for CNS tumors. Reference: Surveillance of Primary Intracranial and Central Nervous System Tumors:

Recommendations from the Brain Tumor Working Group. 112 Genetic Syndromes Genetic syndromes associated with multiple CNS tumors are: Neurofibromatosis I (von Recklinghausens disease) Neurofibromatosis II (bilateral acoustic neurofibromatosis) Von Hippel-Lindau disease Tuberous sclerosis (Bourneville-Pringle syndrome) Gorlin syndrome (Nevoid Basal Cell Carcinoma syndrome

Hermans-Grosfeld-Spaas-Valk disease Li-Fraumeni syndrome Familial retinoblastoma Turcot syndrome (Adenomatous Polyposis syndrome) Cowden disease 113 Diagnostic Tools Physical Exam Neurological examination Eye movements Vision Hearing Reflexes Balance and coordination

Sense of smell and touch Abstract thinking Memory 114 Diagnostic Tools: Radiology Computerized tomography (CT) scan Magnetic resonance imaging (MRI) Positron emission tomography (PET) Single photon emission computed tomography (SPECT) Magnetoencephalography (MEG) Angiography

115 Diagnostic Tools: Laboratory tests Audiometry Electroencephalogram (EEG)

Endocrine evaluation Evoked potentials Lumbar puncture Myelogram Perimetry 116 Diagnostic Tools Needle biopsy Needle inserted through a burr hole and tissue extracted for tissue diagnosis. Stereotactic biopsy

Computer used to guided needle biopsy to extract tissue. 117 College of American Pathologist (CAP) Protocols Site-specific checklists Required to be completed in the health record in hospitals with COC-approved cancer programs for cases diagnosed January 1, 2004 and later.

www.cap.org/cancerprotocols/ protocols_index.html. 118 Brain and Spinal Cord CAP Protocols (1) Macroscopic Specimen type Specimen size Tumor site Tumor size 119

Brain and Spinal Cord CAP Protocols Microscopic Histologic type Histologic grade Margins Additional studies* Additional pathologic findings* Comments* *Not required for COC approval. 120 Treatment (1)

Watchful waiting Surgery Radiation Chemotherapy Hormonal therapy Immunotherapy Hematologic Transplant and Endocrine procedures

121 Treatment (2) Inoperable or inaccessible tumors may be treated with primary radiation and other systemic therapy: Chemotherapy, immunotherapy, and hormone therapy. Shunt insertion to reduce intracranial swelling is not coded as surgical treatment. 122 Surgical Procedure of Primary

Site Brain: Site-specific surgery codes Meninges Brain Spinal cord, cranial nerves, other CNS. All Other Sites: Site-specific surgery codes Pituitary gland Craniopharyngeal duct Pineal gland. 123

Surgical Procedure of Primary Site C70-0-C70.9, C71.0-C71.9, C72.0-C72.9 (1) Code 10: Tumor destruction, NOS Laser surgery Laser surgery with photodynamic therapy Ultrasonic aspirator. No specimen sent to pathology from surgical procedure. 124 Surgical Procedure of Primary Site

C70-0-C70.9, C71.0-C71.9, C72.0-C72.9 (2) 20:Local Excision (biopsy) of tumor, lesion, or mass Specimen sent to pathology from surgical event. 40: Partial resection 55: Gross total resection 90: Surgery, NOS 125 Surgical Procedure of Primary Site C75.1, C75.2, C75.3 (1)

Code NOS Code Code Code Code 10: Local tumor destruction, 11: 12: 13: 14: Photodynamic therapy Electrocautery; fulguration

Cryosurgery Laser No specimen is sent to pathology from surgical events 10-14. 126 Surgical Procedure of Primary Site C75.1, C75.2, C75.3 (2) Code 20: Local tumor excision, NOS Code 26: Polypectomy Code 27: Excisional biopsy Any combination of 20 or 26-27 WITH 21: Photodynamic therapy (PDT)

22: Electrocautery 23: Cyrosurgery 24: Laser ablation 127 Surgical Procedure of Primary Site C75.1, C75.2, C75.3 (3) Code 25: Laser excision Specimen sent to pathology from surgical event 20-27. Code 30: Simple or partial surgical removal of primary site.

128 Surgical Procedure of Primary Site C75.1, C75.2, C75.3 (4) Code 40: Total surgical removal of primary site; enucleation Code 50: Surgery stated to be debulking Code 60: Radical surgery Partial or total removal of the primary site WITH resection in continuity (partial or total removal) with other organs

Code 90: Surgery, NOS 129 Surgical Margins of the Primary Site Code final status of surgical margins COC-required data item. Serves as quality control measure for pathology reports. May be prognostic factor in recurrence. 130

Scope of Regional Lymph Node Surgery Identifies removal, biopsy, or aspiration of regional lymph node(s): NPCR-, COC-, and SEER-required data item. Code 9: Meninges, brain, and spinal cord; cranial nerves; and other parts of the CNS. Code as appropriate: Pituitary gland, craniopharyngeal duct, and pineal gland. 131

Radiation Therapy (1) Radiation codes indicate type of radiation therapy performed as part of the first course of treatment. Records modality of radiation therapy used to deliver significant regional dose to the primary volume of interest. COC-required data item. SEER collects these data from COCapproved facilities NPCR: Supplementary or recommended. 132 Radiation Therapy (2) Beam radiation Codes 20 29:

Conventional radiation therapy: from an external beam directed at the tumor. Energy is orthovoltage, cobalt, photon, and/or electron. Code 30: Boron neutron capture therapy (BNCT) Code 31: Intensity-modulated radiation therapy (IMRT) 133 Radiation Therapy (3) Beam radiation Code 32: Conformal radiation Code

Code Code Code 40: 41: 42: 43: Particle or proton beam Stereotactic radiosurgery, NOS Linac radiosurgery Gamma knife 134

Radiation Therapy (3) Tumors typically treated with stereotactic radiosurgery include: Acoustic neuroma Chordoma Pineal tumor Astrocytoma Craniopharyngioma Hemangioblastoma Pituitary adenomal tumor

135 Radiation Therapy (4) Radioactive implants Code 50: Brachytherapy, radiation implants, radiation seeding, radioactive implants, interstitial implants, intracavitary radiation NOS Code 51: Intracavitary radiation with low dose rate applicators (Cesium- 137, Fletcher applicator) 136

Radiation Therapy (5) Radioactive implants (continued) Code 52: Intracavitary radiation with high dose rate applicator Code 53: Interstitial radiation with low dose rate sources Code 54: Interstitial radiation with high dose rate sources Code 55: Low dose rate interstitial or intracavitary radium 137 Chemotherapy (1)

Record type of chemotherapy administered as first course of treatment: Code 01: Chemotherapy, NOS Code 02: Single-agent chemotherapy Code 03: Multi-agent chemotherapy 138 Chemotherapy (2) Blood-brain barrier Protects the brain from foreign substances, including chemotherapy. May be disrupted by receptormediated permeabilizers.

Intrathecal chemotherapy Drugs directly injected into the cerebrospinal fluid by spinal injection or Ommaya reservoir. 139 Chemotherapy (3) Interstitial chemotherapy Administered directly to involved tissues. Polymer wafers soaked in a chemotherapeutic agent are inserted in the tumor bed after tumor resection.

140 Hormone Therapy Record systemic hormonal agents administered as first course of treatment. Tamoxifen and RU-486 (Mifepristone) may be used to treat meningioma. Steroids given to treat swelling caused by CNS tumors are not coded as hormone therapy. 141 Immunotherapy (1) Record whether immunotherapeutic

agents were administered as first course of treatment: Angiogenesis inhibitors block the development of new blood vessels and starve the tumor. Interleukins are growth factors that manipulate the tumors ability to grow. 142 Immunotherapy (2) Gene therapy replaces or repairs the gene responsible for tumor growth. Vaccine therapy allows the immune system to detect the tumor antigens and attack the tumor cells.

143 Hematologic Transplant and Endocrine Procedures Identify systemic therapeutic procedures administered as first course of treatment: Code Code

Code Code Code 10: 11: 12: 20: 30: Bone marrow transplant, NOS Autologous bone marrow transplant Allogeneic bone marrow transplant Stem cell harvest Endocrine surgery and/or endocrine

radiation therapy Code 40: Combination of endocrine surgery and/or radiation with transplant procedure 144 Technical Issues Edit Checks NAACCR Edits Committee is developing and modifying data edits to accommodate data collection of non-malignant CNS tumors. 145

Technical Issues Data Analysis Recommendations Report and analyze data for nonmalignant CNS tumors separately from malignant tumors. Footnote that pilocytic astrocytomas are included in the analysis for malignant brain tumors for continuity of trends. Review the standard site and histology groupings for tabulating estimates of these tumors to allow comparability of information across registries. 146 References

Manuals, Articles, Reports A Primer of Brain Tumors, 1998; American Brain Tumor Association, Des Plaines, IL; 800-886-2282 (can link to the manual through their website: www.abta.org) Gershman S, Surawicz T, McLaughlin V, Rousseau V. Completeness of Reporting of Brain and Other Central Nervous System Neoplasms. Journal of Registry Management, Winter 2001, Volume 28, Number 4. 147 References Manuals, Articles, Reports (continued)

Fritz A, Percy C, Jack V, Shanmugaratnam K, Sobin V, Parkin D M , Whelan S. International Classification of Diseases for Oncology, 3rd ed. Geneva: World Health Organization, 2000 Report: Surveillance of Primary Intracranial and Central Nervous System Tumors: Recommendations from the Brain Tumor Working Group, National Coordinating Council for Cancer Surveillance, September 1998 148 References Websites American Brain Tumor Association

www.abta.org American College of Surgeons, Commission on Cancer Information, Facility Oncology Data Standards (FORDS) www.facs.org/dept/cancer/index.html American Joint Committee on Cancer, Collaborative Stage Documentation www.edits.cx/cs/ 149 References Websites (continued) Brain and Neurosurgery Information

Center www.brain-surgery.com/index.html Brain and Spinal Cord Tumors: Hope through Research www.ninds.nih.gov/health_and_medical /pubs/brain_tumor_hope_through_rese arch.htm Brain Tumor Guide http://virtualtrials.com/faq/toc.cfm Central Brain Tumor Registry of the United States 150 www.cbtrus.org/page2t.htm References

Websites (continued) College of American Pathologists (CAP), Protocol Brain ftp://ftp.cap.org/cancerprotocols/Brain0 3_p.doc Illustrated Glossary of Radiology: Anatomy, Examinations and Procedures; Department of Radiology and Radiological Services, The Uniformed Services University of the Health Sciences http://rad.usuhs.mil/glossary.html 151 References

Websites (continued) International RadioSurgery Association www.isra.org/index.html National Brain Tumor Radiosurgery Association www.braintumors.com/radiosurgery/r adiosrugery.info#TWO NCI Brain Tumor Home Page www.nci.nih.gov/cancer_information/c ancer_type/brain_tumor / 152 References Websites (continued)

PDQ Cancer Information Summaries: Adult Treatment www.cancer.gov/cancerinfo/pdq/adulttr eatment PDQ Cancer Information Summaries: Pediatric Treatment www.cancer.gov/cancerinfo/pdq/pediat rictreatment The Brain Tumor Foundation www.braintumorfoundation.org/neuros urgery/ss3_3.htm 153 Acknowledgments (1) Prepared by

Shannon Vann, CTR for the North American Association of Central Cancer Registries (NAACCR) This training presentation was supported by contract #2002001-00044 from CDC. The content of this training presentation does not necessarily reflect the views or policies of the Department of Health and Human Services, nor does mention of trade names, commercial products, or organizations imply endorsement by the U.S. Government. 154 Acknowledgments (2) Sponsors Centers for Disease Control and Prevention

National Program for Cancer Registries National Cancer Institute Surveillance, Epidemiology and End Results Program North American Association of Central Cancer Registries American Joint Committee on Cancer Collaborative Stage Task Force 155 Acknowledgments (3) CDC National Program of Cancer Registries Planning Committee

Kimberly Cantrell Gayle G. Clutter Faye Floyd Michael Lanzilotta Frances Michaud 156 Acknowledgments (4) Materials Review Committee Trista Aarnes-Leong St. Vincent Medical Center, NAACCR Registry Operations Subcommittee, Susan Bolick-Aldrich South Carolina Central Cancer Registry, NAACCR Registry Operations Subcommittee, Chair, Co-chair, Registry Operations Committee Gayle Clutter CDC National Program of Cancer Registries, Registry Operations Subcommittee, National Coordination Council on Cancer Surveillance Brain Tumor

Working Group, Chair Faye Floyd CDC National Program of Cancer Registries April Fritz NCI Surveillance, Epidemiology and End Results Program, Registry Operations Subcommittee Elaine Hamlyn Canadian Cancer Registry, Registry Operations Subcommittee, Holly Howe North American Association of Central Cancer Registries, Executive Director Betsy Kohler New Jersey State Cancer Registry, NAACCR Education Committee

Carol Kruchko Central Brain Tumor Registry of the United States, Registry Operations Subcommittee, National Coordination Council on Cancer Surveillance Brain Tumor Working Group Donna Morrel Cancer Surveillance Program of Los Angeles. Registry Operations Subcommittee Linda Mulvihill North Carolina Central Cancer Registry, Registry Operations Subcommittee Wendy Scharber Minnesota Cancer Surveillance Program James Smirniotopoulos Professor of Radiology, Uniformed Services University,

Registry Operations Subcommittee Katheryne Vance California Cancer Registry, Registry Operations Subcommittee Valerie Vesich American College of Surgeons, Commission on Cancer, Registry Operations Subcommittee 157

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