Data Collection of Primary Central Nervous System (CNS) Tumors

HEALTH AND HUMAN SERVICES
CENTERS FOR DISEASE CONTROL AND PREVENTION
Atlanta, Georgia,

data collection rules adopted by the North American Association of

cause disruption in normal function similar to that caused by

States (CBTRUS) formed to report population-based data on primary benign,

1 and 2
Deferred recommendations 3 and 4

for collecting precise data for all primary intracranial and CNS

Brain
Spinal cord
Pituitary gland
Craniopharyngeal duct

Meninges
Cauda equina
Pineal gland

Cranial nerves and other parts of the CNS.

tabulating estimates of CNS tumors to allow comparability of information

primary intracranial and CNS tumors, and develop computerized edit- checking

and World Health Organization (WHO) 2000 Brain Tumor Classification are

the BTWG to work on Recommendations 3 and 4.
2002 NAACCR

tumors.
The term “brain-related tumor” means a listed primary tumor (whether

lobe (C71.3)
Occipital lobe (C71.4).

of the brain (C71.8)
Brain NOS (C71.9)

(C70.9)
Spinal cord (C72.0)
Cauda equina (C72.1)

(C72.4)
Cranial nerve NOS (C72.5)

(C75.2)
Pineal gland (C75.3)

For the sites described, benign, borderline, and malignant

also report non-malignant CNS tumors diagnosed on or after January

in number of cases estimated by doubling the number of

case load is estimated to increase by 1%.

In 2002, 21

adding non-malignant CNS tumors to reportable case definition may have

registries must:
Have the same definition for brain-related tumors.
Implement data edits

and departments
Gamma or cyber knife center.

gamma or cyber knife centers
Free-standing oncology centers
Data exchange with other

CNS “neoplasm” or “mass”, an ICD-O-3 histology code must exist

Coding Grade

tumors
Germ cell tumors
Meningiomas
Tumors of cranial nerves

up of astrocytes and oligodendrocytes

Glial tumors assigned ICD-O-3 histology codes

mildly and moderately anaplastic astrocytomas (M9401)
Anaplastic astrocytomas
Glioblastoma multiforme

(M9392)
Ependymoblastomas (M9392)
Oligodendroglial tumors
Well-differentiated oligodendrogliomas (M9450)
Anaplastic oligodendrogliomas (M9451)

gliomas
Ganglioneuromas (M9490)
Optic nerve gliomas

(M9400)
Germ cell tumors
Germinomas (M9064)
Embryonal carcinomas (M9070)
Choriocarcinomas (M9100)
Teratomas (M9080)

plexus tumors
Choroid plexus papilloma (M9390)
Choroid plexus carcinoma
Choroid plexus meningioma

schwannomas/neuromas

Neuroblastomas (M9500)

in meninges
High incidence in patients with AIDS
Vascular tumors
Rare, non-malignant tumors
Arise

(M9084)
Granular cell tumors (M9580)
Rathke pouch tumors (M9350)
Not reportable
Epidermoid cysts
Colloid cysts
Enterogenous

in adult and children.

Tumor location and extent of spread affect

children.
50% are infratentorial.
Common infratentorial tumors:
Cerebellar astrocytomas
Meduloblastomas
Ependymomas
Brain stem gliomas
Atypical

tumors
Diencephalic and hypothalamic gliomas
Low grade astrocytomas
Mixed gliomas
Anaplastic astrocytomas
Oligodendrogliomas
PNETs
Meningiomas
Glioblastoma

tumors is the same as for childhood brain tumors.

Primary spinal

unknown.

American Academy of Pediatrics has outlined guidelines for pediatric cancer

if the primary site is intracranial or the skull (C41.0).
Non-malignant

pilocytic astrocytoma.

When the primary site for intracranial schwannoma (9560/0)

tumor differentiation or grade.
Is not usually specified for CNS tumors.

Site-specific factor 1 for Brain.

Four-category tumor grading system
Grade I
Slow

grade tumors
May be non-malignant or malignant .

Grade III
Malignant tumors
Often

low-grade astrocytomas
Grade 3: anaplastic astrocytomas
Grade 4: glioblastoma multiforme

No NAACCR data

atypia
Mitosis
Endothelial proliferation
Necrosis

No NAACCR data field for the St. Anne-Mayo grade.

usually nuclear atypia
Grade 3: Two criteria, usually nuclear atypia and

or St. Anne/Mayo grade in the sixth digit histology code

on both non-malignant and malignant tumors.
Used to determine if multiple

NOS (C70.0)
Cerebrum (C71.0)
Frontal lobe (C71.1)
Temporal lobe (C71.2)
Parietal lobe (C71.3)
Occipital lobe

nerve (C72.2)
Optic nerve (C72.3)
Acoustic nerve (C72.4)
Cranial nerve, NOS (C72.5)

or 1 (borderline).

CNS
Includes intracranial and central nervous system topographic sites.

category (first three characters) as delineated in ICD-O-3 is considered

refer to differences in the FIRST THREE digits of the

as delineated in ICD-O-3 is considered a separate site.
Same site

specific four-digit site code in same rubric

Example: meninges, NOS

multiple tumors of the same site and same histologic type

same site, refer to Table 2, and use the following

listed in the same histologic group in Table 2, then

digits are the same as the first three digits for

digits are the same and the histologies are from two

of the same site and same histology, diagnosed within 2

new non-malignant tumor of the same histology as an earlier

lesions: all non-malignant

If different sites, then DIFFERENT primaries.
If different histologies,

lesions: all non-malignant (cont.)
If same site and same histology:
Laterality is

tumors: One non-malignant and one malignant
Non-malignant tumor followed by

by pathological review.
Final diagnosis made by review of previous

a higher grade tumor,
SAME tumor.
Do not change the histology or

tumor is rare.
Malignant transformations include:
Changes from WHO grade I to

reportable-by-agreement series.
Malignant tumors: assigned sequence numbers from the malignant series.
Example:

medical practitioner diagnosed the non-malignant tumor either clinically or histologically.

Malignant

over the lifetime of the person.

Codes 00 – 35: Malignant

and/or central cancer registry:

Non-malignant CNS tumors are assigned reportable-by-agreement sequence

assigned over the lifetime of the person.
Example: Patient diagnosed with

for malignant and non-malignant tumors.

Review source records carefully to

data fields to calculate site-specific American Joint Committee on Cancer

cerebral meninges

Other parts of the CNS

Glands: pituitary gland, craniopharyngeal duct,

the Brain, cerebral meninges and other CNS.
Code 88: Not applicable.
For

borderline brain tumors
10 Supratentorial tumor confined to CEREBRAL HEMISPHERE (cerebrum) or

tumor confined to BRAIN STEM or MENINGES of BRAIN STEM

the midline: involves the contralateral hemisphere, involves corpus callosum (including

in cerebral spinal fluid; nasal cavity; nasopharynx; posterior pharynx; or

cord
Caudia equina
Olfactory, acoustic, cranial nerve, NOS
Overlapping brain and CNS
Nervous system,

to tissue or site of origin
30 Localized, NOS
40 Meningeal tumor infiltrates nerve;

nerve tumors; bone, other than skull; eye
80 Further contiguous extension
95 No evidence

or borderline tumors
10 Invasive carcinoma confined to gland of origin
30 Localized,

CS Lymph Nodes is 88 (not applicable) for meninges, brain,

metastases
99 Unknown; distant metastasis cannot be assessed; not documented in patient

C72.0—9, C75.1, C75,2, C75.3
00 No; None
10 Distant lymph node(s)
40 Distant metastasis except lymph

Grade II
030 WHO Grade III
040 WHO Grade IV
999 Clinically diagnosed; grade unknown;
Not

grade for CNS tumors in CS Site-specific factor 1.

Do not

have been identified.
Population-based studies suggest that no more than 4%

suggests a viral etiology for CNS tumors.



Reference: “Surveillance of Primary

(von Recklinghausen’s disease)
Neurofibromatosis II (bilateral acoustic neurofibromatosis)
Von Hippel-Lindau disease
Tuberous sclerosis

of smell and touch
Abstract thinking
Memory

emission tomography (PET)
Single photon emission computed tomography (SPECT)
Magnetoencephalography (MEG)
Angiography

extracted for tissue diagnosis.

Stereotactic biopsy
Computer used to guided needle biopsy

completed in the health record in hospitals with COC-approved cancer

www.cap.org/cancerprotocols/protocols_index.html.

required for COC approval.

radiation and other systemic therapy:
Chemotherapy, immunotherapy, and hormone therapy.

Shunt

nerves, other CNS.
All Other Sites: Site-specific surgery codes
Pituitary

destruction, NOS
Laser surgery
Laser surgery with photodynamic therapy
Ultrasonic aspirator.

No

of tumor, lesion, or mass
Specimen sent to pathology from surgical

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

tumor excision, NOS
Code 26: Polypectomy
Code 27: Excisional biopsy
Any combination

excision
Specimen sent to pathology from surgical event 20-27.

Code 30: Simple

surgical removal of primary site; enucleation
Code 50: Surgery stated to

margins

COC-required data item.
Serves as quality control measure for pathology reports.

of regional lymph node(s):
NPCR-, COC-, and SEER-required data item.
Code 9:

performed as part of the first course of treatment.
Records modality

from an external beam directed at the tumor.
Energy is

or proton beam
Code 41: Stereotactic radiosurgery, NOS
Code 42: Linac radiosurgery
Code

neuroma
Chordoma
Pineal tumor
Astrocytoma
Craniopharyngioma
Hemangioblastoma
Pituitary adenomal tumor

radioactive implants, interstitial implants, intracavitary radiation NOS
Code 51: Intracavitary radiation

dose rate applicator
Code 53: Interstitial radiation with low dose rate

treatment:

Code 01: Chemotherapy, NOS

Code 02: Single-agent chemotherapy

Code 03: Multi-agent chemotherapy

chemotherapy.
May be disrupted by receptor-mediated permeabilizers.

Intrathecal chemotherapy
Drugs directly injected into

a chemotherapeutic agent are inserted in the tumor bed after

treatment.

Tamoxifen and RU-486 (Mifepristone) may be used to treat meningioma.
Steroids

of treatment:

Angiogenesis inhibitors block the development of new blood vessels

tumor growth.

Vaccine therapy allows the immune system to detect the

first course of treatment:
Code 10: Bone marrow transplant, NOS
Code 11:

edits to accommodate data collection of non-malignant CNS tumors.

tumors separately from malignant tumors.
Footnote that pilocytic astrocytomas are included

Tumor Association, Des Plaines, IL; 800-886-2282 (can link to the

K, Sobin V, Parkin D M , Whelan S. International

Cancer Information, Facility Oncology Data Standards (FORDS) www.facs.org/dept/cancer/index.html
American Joint Committee

Tumors: Hope through Research www.ninds.nih.gov/health_and_medical/pubs/brain_tumor_hope_through_research.htm
Brain Tumor Guide http://virtualtrials.com/faq/toc.cfm
Central Brain Tumor

Glossary of Radiology: Anatomy, Examinations and Procedures; Department of Radiology

Brain Tumor Home Page www.nci.nih.gov/cancer_information/cancer_type/brain_tumor/

Summaries: Pediatric Treatment www.cancer.gov/cancerinfo/pdq/pediatrictreatment
The Brain Tumor Foundation www.braintumorfoundation.org/neurosurgery/ss3_3.htm

Cancer Registries (NAACCR)

This training presentation was supported by contract #200-2001-00044

Registries
National Cancer Institute
Surveillance, Epidemiology and End Results Program
North American Association

G. Clutter
Faye Floyd
Michael Lanzilotta
Frances Michaud

Operations Subcommittee,
Susan Bolick-Aldrich South Carolina Central Cancer Registry, NAACCR Registry Operations