Data Collection of Primary Central Nervous System (CNS) Tumors

Portions of this presentation are based on non-malignant CNS tumor data

Part I

Rationale
History
Definition of Reportable Cases
Casefinding
Anticipated Impact on Registries

Rationale for Non-malignant CNS Tumor Surveillance and Registration

Non-malignant CNS tumors cause

History 1992 -1996

1992 Central Brain Tumor Registry of the United States

History 1998

BTWG forwarded four recommendations to the NCCCS
NCCCS
Accepted recommendations 1

BTWG Recommendations (1)

The following standard definition is to be used for

BTWG Recommendations (2)

Develop a standard site and histology definition for tabulating

BTWG Recommendations (3)

Develop training for reporting and tabulating primary intracranial and

History 2000

International Classification of Diseases for Oncology 3rd Edition (ICD-O-3) and

History 2001-2002

2001 NCCCS
Accepted Recommendations 1 and 2 as completed.
Reconvened the

Reportable Brain-Related Tumors (1)

Public Law 107-260 requires reporting of brain-related tumors.
The

Reportable Brain-Related Tumors (2)

Brain
Cerebrum (C71.0)
Frontal lobe (C71.1)
Temporal lobe (C71.2)
Parietal lobe

Reportable Brain-Related Tumors (3)

Brain (continued)
Ventricle (C71.5)
Cerebellum (C71.6)
Brain stem (C71.7)
Overlapping lesion of

Reportable Brain-Related Tumors (4)

Meninges
Cerebral meninges (C70.0)
Spinal meninges (C70.1)
Meninges NOS (C70.9)
Spinal

Reportable Brain-Related Tumors (5)

Cranial nerves
Olfactory nerve (C72.2)
Optic nerve (C72.3)
Acoustic nerve (C72.4)
Cranial

Reportable Brain-Related Tumors (6)

Other CNS (C72.8, C72.9)
Pituitary gland (C75.1)
Craniopharyngeal duct (C75.2)
Pineal

History 2003

2003 SEER-supported registries and COC-approved hospital cancer registries will also

Impact of Collecting Data on Non-malignant CNS Tumors (1)

Annual increase in

Impact of Collecting Data on Non-malignant CNS Tumors (2)

Central registry case

Impact of Collecting Data on Non-malignant CNS Tumors (3)

Central registries adding

Impact of Collecting Data on Non-malignant CNS Tumors (4)

All cancer registries

Case-finding (1)

Additional or expanded case-finding mechanisms:
Pathology
Radiology
Treatment facilities:
Radiation oncology centers and

Case-finding (2)

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

Case-finding Sources

Free-standing radiation therapy centers
Free-standing Magnetic Resonance Imaging (MRI) centers
Free-standing gamma

ICD-9-CM Codes for Case-finding

Unusual and Ambiguous Terminology

If the final pathologic diagnosis is a CNS

Part II

CNS Anatomy and Function
Histologies and Primary Sites
Grading Systems and Coding

CNS Functional Anatomy

Source: URL: www.solinas.com/solinas/brain.html accessed 7/18/03.

CNS Anatomy

C71

C71.6

C71.7

C72.0

C71.0

C75.3

C75.1

C71.7

Source: URL: www.universalpeace.ca/principles.htm accessed 7/18/03.

Intracranial Sites

C71.0

C71.6

C41.0

C71.7

C72.0

Source: URL: mscenter.ucsf.edu/faq.htm accessed 7/18/03.

Parietal lobe

Frontal lobe

Cerebrum

C71.1

C71.2

C71.7

C71.3

C71.4

C71.6

C71.0

Source: URL: www.sciencebob.com/lab/bodyzone/brainprint.html Accessed 7/18/03.

Cerebellum and Brain Stem

C71.0

C71.1

C71.2

C71.7

C71.3

C71.4

C71.6

URL: www.sciencebob.com/lab/bodyzone/brain.html 7/18/03

The Ventricular System

http://www.abta.org/primer2.htm

Pineal and Pituitary Glands

C75.1

C71.7

C75.3

C71.6

C72.0

Source: URL: training.seer.cancer.gov/module_anatomy/unit6_3_endo_gl… Accessed 7/18/03.

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.

Meninges

C71.0

C70.0

C70.0

Source: URL: www.cardioliving.com/consumer/Stroke/Hemorrhagic_Stroke.sht Accessed 7/18/03.

Tentorium

C70.0

C70.0

Source: URL: neurosurgery.mgh.harvard.edu/abta/primer.htm Accessed 7/18/03.

Spinal Cord

C72.0

C70.1

Source: URL: www.merck.com/pubs/mmanual/figures/182fig1.htm Accessed 7/18/03

Cellular Classification

Neuroepithelial tumors
Astrocytomas
Oligodendrogliomas
Ependymomas
Pineal parenchymal tumors
Other CNS tumors
Sellar tumors
Hematopoetic tumors
Germ

Glial Tumors (1)

Glial tissue: supportive tissue of brain made up

Glial Tumors (2)

Astrocytic tumors
Noninfiltrating
Juvenile pilocytic (M9421)
Subependymal (M9383)
Infiltrating
Well-differentiated mildly

Glial Tumors (3)

Ependymal tumors
Myxopapillary and well-differentiated ependymomas (M9394)
Anaplastic ependymomas (M9392)
Ependymoblastomas

Glial Tumors (4)

Mixed tumors
Mixed astrocytoma-ependymomas
Mixed astrocytoma-oligodendrogliomas
Mixed astrocytoma-ependymoma-oligodendrogliomas
Other gliomas
Ganglioneuromas

Non-Glial Tumors (1)

Pineal region tumors
Parenchymal tumors
Pineocytomas (M9361)
Pineoblastomas (M9362)
Pineal astrocytomas (M9400)
Germ

Non-Glial Tumors (2)

Meningiomas
Meningioma: Benign (M953_)
Malignant meningiomas
Anaplastic meningioma
Hemangiopericytoma (M9150)
Papillary meningioma (M9538)
Choroid plexus

Other CNS Tumors (1)

Craniopharyngiomas (M9350)
Rathke pouch tumors
Chordomas (M9370)
Schwannomas (M9560)
Acoustic schwannomas/neuromas

Other CNS Tumors (2)

Embryonal tumors
Retinoblastomas (M9510)
Primitive neuroectodermal tumors (PNETs)
Meduloblastomas (M9470)
Neuroblastomas

Other CNS Tumors (3)

Lymphomas (M9590)
Arise from
Indigenous brain histiocytes (microglia)
Rare lymphocytes in

Other CNS Tumors (4)

Cysts and tumor-like lesions
Reportable
Dermoid cysts (M9084)
Granular

Childhood versus Adult Tumors

CNS tumor histology and location are different in

Childhood Brain Tumors

Meduloblastomas are the most common CNS histology in children.
50%

Cellular Classification Childhood Brain Tumors (1)

Supratentorial tumors in children

Craniopharyngiomas
Germ cell tumors
Diencephalic

Cellular Classification Childhood Brain Tumors (2)

The histopathology of childhood spinal tumors

Cellular Classification Childhood CNS Tumors

Cause of childhood CNS tumors remains unknown.
American

ICD-O-3 Coding Issues (1)

Some histologies may be difficult to determine if

ICD-O-3 Coding Issues (2)

Continue to assign histology code M9421/3 to pilocytic

Grade for CNS Tumors

Sixth digit of ICD-O-3 histology code
Describes tumor

WHO Grade (1)

WHO grade coded in Collaborative Stage data field:
Site-specific

WHO Grade (2)

Grade II
Relatively slow growing
Sometimes recur as higher grade

Kernohan Grade

Defines progressive malignancy for astrocytoma
Grade 1: benign astrocytomas
Grade 2: low-grade

St. Anne-Mayo Grade (1)

Used for astrocytomas.
Uses four morphologic criteria:
Nuclear atypia
Mitosis
Endothelial

St. Anne-Mayo Grade (2)

Grade 1: No criteria
Grade 2: One criterion, usually

Grade for CNS Tumors

Do not record WHO grade, Kernohan grade, or

Part III

Laterality
Multiple Primaries
Malignant Transformation
Sequence Numbers
Date of Diagnosis

Determining Multiple Primaries: Laterality

Brain is not a paired organ.
Laterality collected on

Coding Laterality (1)

CNS sites to be coded with laterality:
Cerebral meninges, NOS

Coding Laterality (2)

CNS sites to be coded with laterality (continued):
Olfactory nerve

Determining Multiple Primaries: Definitions

Non-malignant tumor
Tumor with ICD-O-3 behavior code
0 (benign) or

Determining Multiple Primaries Malignant (1)

NO CHANGES (at this time)
Site
Rule: Each category

Determining Multiple Primaries: Malignant (2)

Histology
Rule: Differences in histologic type refer

Determining Multiple Primaries Non-malignant (1)

NEW RULES
Site
Rule: Each sub-site (fourth-digit level) as

Determining Multiple Primaries Non-malignant (2)

Site (cont)
EXCEPT NOS (C_ _.9) with specific

Determining Multiple Primaries Non-malignant (3)

Site (cont)
Laterality: For non-malignant cases only
If multiple

Determining Multiple Primaries: Non-malignant (4)

Histology

Determining Multiple Primaries: Non-malignant (5)

Histology
If multiple tumors are in the same

Determining Multiple Primaries: Non-malignant (6)

Histology (cont.)
B. If all histologies are listed

Determining Multiple Primaries: Non-malignant (7)

Histology (cont)
C: If the first three digits

Determining Multiple Primaries: Non-malignant (8)

Histology (cont)
D: If the first three digits

Determining Multiple Primaries: Timing (1)

Primary malignant CNS tumors
NO CHANGE
Malignant tumors of

Determining Multiple Primaries: Timing (2)

Primary non-malignant CNS tumors
NEW
No timing rule
If a new

General Rules for Determining Multiple Primaries of CNS Sites (1)

Multiple lesions:

General Rules for Determining Multiple Primaries of CNS Sites (2)

Multiple lesions:

General Rules for Determining Multiple Primaries of CNS Sites (3)

Multiple tumors:

Histologic Transformation (1)

Histologic transformation or progression to a higher grade:
Determined by

Histologic Transformation (2)

If a malignant CNS tumor recurs (transforms) as a

Histologic Transformation (3)

Transformation of a non-malignant tumor to a malignant tumor

Histologic Transformation (4) Sequence Numbers

Non-malignant tumors: assigned sequence numbers from the reportable-by-agreement

Histologic Transformation (5) Date of Diagnosis

Non-malignant tumors: First date that a medical

Coding Sequence Numbers (1)

Indicates the sequence of all reportable neoplasms over

Coding Sequence Numbers (2)

Reportable-by-agreement neoplasms are defined by each facility and/or

Coding Sequence Numbers (3)

Sequence numbers for non-malignant CNS tumors are assigned

Assigning Diagnosis Date

Rules for assigning diagnosis date are the same for

Part IV

Staging
Risk Factors
Genetic Syndromes
Diagnostic Tools
Treatment
Edits
Data Analysis

Collaborative Stage (CS)

A computer algorithm uses the collaborative stage (CS) data

Coding Collaborative Stage (1)

Separate sets of extension codes for:
Brain and cerebral

Coding Collaborative Stage (2)

Site-specific codes for lymph nodes
Same for the

CS Extension: Brain and Meninges C70.0, C71.0 – C71.9 (1)

05 Benign or borderline

CS Extension: Brain and Meninges C70.0, C71.0 – C71.9 (2)

12 Infratentorial tumor

CS Extension: Brain and Meninges C70.0, C71.0 – C71.9 (3)

40 Tumor crosses the

CS Extension: Brain and Meninges C70.0, C71.0 – C71.9 (4)

70 Circulating cells in

CS Extension: Other CNS C70.1-9, C72.0–C72.9 (1)

Spinal meninges, meninges NOS
Spinal cord
Caudia

CS Extension: Other CNS C70.1-9, C72.0–C72.9 (2)

05 Benign or borderline tumors
10 Tumor confined to

CS Extension: Other CNS C70.1-9, C72.0–C72.9 (3)

70 Brain except for cranial nerve

CS Extension: Other Endocrine C75.1, C75.2, C75.3

00 In situ; non-invasive; intraepithelial
05 Benign or

CS Lymph Nodes

Describes tumor involvement of regional lymph nodes.
Code for CS

CS Metastasis at Diagnosis Brain and Meninges C70.0, C71.0-9

00 No; None
10 Distant metastases
85 “Drop” metastases
99 Unknown;

CS Metastasis at Diagnosis Other CNS and Other Endocrine C70.1-9, C72.0—9, C75.1,

CS Site-specific Factor 1 (1) C70.0-C70.9, C71.0-C71.9, C72.0-C72.9

010 WHO Grade I
020 WHO Grade

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

Possible Risk Factors

Genetic predispositions for the development of brain tumors have

Possible Risk Factors

Epstein-Barr virus in the DNA of primary lymphoma suggests

Genetic Syndromes

Genetic syndromes associated with multiple CNS tumors are:
Neurofibromatosis I (von

Diagnostic Tools – Physical Exam

Neurological examination
Eye movements
Vision
Hearing
Reflexes
Balance and coordination
Sense of

Diagnostic Tools: Radiology

Computerized tomography (CT) scan
Magnetic resonance imaging (MRI)
Positron emission

Diagnostic Tools: Laboratory tests

Audiometry
Electroencephalogram (EEG)
Endocrine evaluation
Evoked potentials
Lumbar puncture
Myelogram
Perimetry

Diagnostic Tools

Needle biopsy
Needle inserted through a burr hole and tissue extracted

College of American Pathologist (CAP) Protocols

Site-specific checklists
Required to be completed in

Brain and Spinal Cord CAP Protocols (1)

Macroscopic
Specimen type
Specimen size
Tumor site
Tumor size

Brain and Spinal Cord CAP Protocols

Microscopic
Histologic type
Histologic grade
Margins
Additional studies*
Additional pathologic findings*
Comments*
*Not required

Treatment (1)

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

Treatment (2)

Inoperable or inaccessible tumors may be treated with primary radiation

Surgical Procedure of Primary Site

Brain: Site-specific surgery codes
Meninges
Brain
Spinal cord, cranial nerves,

Surgical Procedure of Primary Site C70-0-C70.9, C71.0-C71.9, C72.0-C72.9 (1)

Code 10: Tumor destruction,

Surgical Procedure of Primary Site C70-0-C70.9, C71.0-C71.9, C72.0-C72.9 (2)

20:Local Excision (biopsy) of

Surgical Procedure of Primary Site C75.1, C75.2, C75.3 (1)

Code 10: Local tumor

Surgical Procedure of Primary Site C75.1, C75.2, C75.3 (2)

Code 20: Local tumor

Surgical Procedure of Primary Site C75.1, C75.2, C75.3 (3)

Code 25: Laser excision
Specimen

Surgical Procedure of Primary Site C75.1, C75.2, C75.3 (4)

Code 40: Total surgical

Surgical Margins of the Primary Site

Code final status of surgical margins
COC-required

Scope of Regional Lymph Node Surgery

Identifies removal, biopsy, or aspiration of

Radiation Therapy (1)

Radiation codes indicate type of radiation therapy performed

Radiation Therapy (2)

Beam radiation
Codes 20 – 29:
Conventional radiation therapy: from

Radiation Therapy (3)

Beam radiation
Code 32: Conformal radiation
Code 40: Particle or

Radiation Therapy (3)
Tumors typically treated with stereotactic radiosurgery include:

Acoustic neuroma
Chordoma
Pineal

Radiation Therapy (4)

Radioactive implants
Code 50: Brachytherapy, radiation implants, radiation seeding, radioactive

Radiation Therapy (5)

Radioactive implants (continued)
Code 52: Intracavitary radiation with high dose

Chemotherapy (1)

Record type of chemotherapy administered as first course of treatment:
Code

Chemotherapy (2)

Blood-brain barrier
Protects the brain from foreign substances, including chemotherapy.
May

Chemotherapy (3)

Interstitial chemotherapy
Administered directly to involved tissues.
Polymer wafers soaked in a

Hormone Therapy

Record systemic hormonal agents administered as first course of treatment.
Tamoxifen

Immunotherapy (1)

Record whether immunotherapeutic agents were administered as first course of

Immunotherapy (2)

Gene therapy replaces or repairs the gene responsible for tumor

Hematologic Transplant and Endocrine Procedures

Identify systemic therapeutic procedures administered as first

Technical Issues Edit Checks

NAACCR Edits Committee is developing and modifying data edits

Technical Issues Data Analysis Recommendations

Report and analyze data for non-malignant CNS tumors

References

Manuals, Articles, Reports
A Primer of Brain Tumors, 1998; American Brain Tumor

References

Manuals, Articles, Reports (continued)
Fritz A, Percy C, Jack V, Shanmugaratnam K,

References

Websites
American Brain Tumor Association www.abta.org
American College of Surgeons, Commission on Cancer

References

Websites (continued)
Brain and Neurosurgery Information Center www.brain-surgery.com/index.html
Brain and Spinal Cord Tumors:

References

Websites (continued)
College of American Pathologists (CAP), Protocol – Brain ftp://ftp.cap.org/cancerprotocols/Brain03_p.doc
Illustrated Glossary

References

Websites (continued)
International RadioSurgery Association www.isra.org/index.html
National Brain Tumor Radiosurgery Association www.braintumors.com/radiosurgery/radiosrugery.info#TWO
NCI Brain

References

Websites (continued)
PDQ Cancer Information Summaries: Adult Treatment www.cancer.gov/cancerinfo/pdq/adulttreatment
PDQ Cancer Information Summaries:

Acknowledgments (1)

Prepared by
Shannon Vann, CTR
for the
North American Association of Central Cancer

Acknowledgments (2)

Sponsors
Centers for Disease Control and Prevention
National Program for Cancer Registries
National

Acknowledgments (3)

CDC National Program of Cancer Registries Planning Committee
Kimberly Cantrell
Gayle G.