Accessioning Primary Intracranial and Central Nervous System Tumors

**General Reporting Rules**

**Prepared by the NAACCR Registry Operations Committee Benign Brain
Tumor Subcommittee**

**6/27/2003**

###### Introduction

In the early 1900's, the neurosurgeon Harvey Cushing made the
observation that some brain tumors are malignant because of their
histology, and some are malignant because of their location. By this he
meant that in the early 1900's some tumors were not resectable and would
result in the death of the patient because of mass effects on vital
areas of the brain. In the past 100 years, with advances in
microsurgery, radiation therapy, and earlier diagnosis, the maxim of Dr.
Cushing still stands, although at a greatly diminished number. The
tumors, whether benign or malignant, produce clinical effects by similar
mechanisms of mass effect, hemorrhage, seizure activity, and edema.
Population-based data on benign brain tumors has not been routinely
collected and has limited our present knowledge of these tumors to
hospital case series of 10 to 20 cases. Long term follow-up in these
studies are generally absent. Although these tumors are individually
rare, patients with benign brain tumors represent an under-appreciated
financial and health burden in the United States. These cases include
those tumors arising in families with an inherited tendency to develop
benign and malignant brain tumors, tumors arising from developmental
abnormalities, morbidity from ruptured benign brain tumors, and eventual
malignant transformation in a subgroup of patients with optic nerve
gliomas.

Existing coding rules for brain and CNS tumors have been guided by the
behavior of these tumors. With the change to a site definition to guide
their collection, the ROC Benign Brain Tumor Subcommittee reviewed
coding rules applicable to both nonmalignant and malignant brain and CNS
tumors. Recommendations applicable to the current rules guiding multiple
primaries for malignant brain and CNS tumors are contained in a separate
document and have been forwarded to the SEER Histology Coding Committee
for review in 2003.

**Proposed Rules for Benign Brain Tumors**

Effective with cases diagnosed January 2004 and after

(Note: the rules for malignant brain tumors follow the same rules for
multiple primaries that have been in effect, but are presented with
those for non-malignant brain tumors for ease of use.)

Beginning with tumors diagnosed on or after January 1, 2004, reportable
tumors required to be abstracted include the following non-malignant
primary intracranial and central nervous system tumors with a behavior
code of /0 or /1 (benign and borderline, or "non-malignant") regardless
of histologic type, for these ICD-O-3 topography codes.

+-----------+----------------------------------------------------------+
| ### Tabl  |                                                          |
| e 1. Topo |                                                          |
| graphy Co |                                                          |
| des for B |                                                          |
| enign Bra |                                                          |
| in Tumors |                                                          |
+-----------+----------------------------------------------------------+
| **Codes** | ## Description                                           |
+-----------+----------------------------------------------------------+
| > C70.0   | Meninges                                                 |
| >         |                                                          |
| > C70.1   | Cerebral meninges                                        |
| >         |                                                          |
| > C70.9   | Spinal meninges                                          |
|           |                                                          |
|           | Meninges, NOS                                            |
+-----------+----------------------------------------------------------+
| C71.0     | Brain                                                    |
|           |                                                          |
| C71.1     | Cerebrum                                                 |
|           |                                                          |
| C71.2     | Frontal lobe                                             |
|           |                                                          |
| C71.3     | Temporal lobe                                            |
|           |                                                          |
| C71.4     | Parietal lobe                                            |
|           |                                                          |
| C71.5     | Occipital lobe                                           |
|           |                                                          |
| C71.6     | Ventricle, NOS                                           |
|           |                                                          |
| C71.7     | Cerebellum, NOS                                          |
|           |                                                          |
| C71.8     | Brain stem                                               |
|           |                                                          |
| C71.9     | Overlapping lesion of brain                              |
|           |                                                          |
|           | ##### Brain, NOS                                         |
+-----------+----------------------------------------------------------+
| #####     | Spinal Cord, Cranial Nerves and Other Parts of the       |
|           | Central Nervous System                                   |
| ###       |                                                          |
| ## 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                                |
+-----------+----------------------------------------------------------+
| C75.1     | Other Endocrine Glands and Related Structures            |
|           |                                                          |
| C75.2     | Pituitary gland                                          |
|           |                                                          |
| C75.3     | Craniopharyngeal duct                                    |
|           |                                                          |
|           | Pineal gland                                             |
+-----------+----------------------------------------------------------+

-   For non-malignant primary intracranial and central nervous system
    tumors (C70.0 -- C72.9, C75.1 -- C75.3), the terms "tumor" and
    "neoplasm" are considered diagnostic for the purpose of case
    reporting, in addition to the terms generally applicable to
    malignant tumors.

## Definitions:

A.  Non-malignant: behavior code of /0 or /1.

B.  Malignant: behavior code of /2 or /3.

## Same Site:

> 1\. Non-malignant: same 4 digit site (except for 4 digit site pairs
> where one is C70.9,

C71.9, or C72.9)

2\. Malignant: same 3 digit site

C.  Different site

    1.  Non-malignant: different 4 digit site code except NOS (C\_ \_.9)
        with specific 4 digit site code in same rubric

*Example:* meninges, NOS (C70.9) with spinal meninges (C70.1) or

cerebral meninges (C70.0)

> 2\. Malignant: different 3 digit site

D.  Same histology

    1.  Non-malignant (in priority order):

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a)  Use Table 2 listed under II.D. in this document -- if both
    histologies are in the same histologic group, then same histology

b)  If same first 3 digits as any histology in Table 2, then same
    histology

c)  If same first 3 digits but neither in Table 2, then same histology

> 2\. Malignant (current rule): same at 3 digit level

E.  Different histology

    1.  Non-malignant:

        a.  If 2 different histologic groups in Table 2

        b.  If different at 3 digit level and not in same group in Table
            2

        c.  If different at 3 digit level and neither in Table 2, then
            different histology

> 2\. Malignant (current rule): different at 3 digit level

F.  Timing

1\. Non-malignant: current 2-month timing rule does not apply.

> 2\. Malignant:

a.  Within 2 months

b.  2+ months

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G.  Laterality:

    1.  Single side (SS): involves only one side of sites listed in
        Table 3

    2.  Both sides (BS): involves both sides of sites listed in Table 3

    3.  Laterality unknown (LX): Site does not have laterality coded or
        laterality is not coded for site

*Rationale:* a*.* Researchers, including epidemiologists, have requested
the collection of

laterality *(*Inskip PD*, Neuroepidemiology 2003; 22;130-138*). The

location of certain tumors might help in determining causation. Certain

investigations such as those involving cell phone usage would benefit

from having this variable routinely available.

> b\. Non-treatment-related factors such as location of tumor by
> hemisphere
>
> can be predictive factors for cognitive outcome. Brown PD, Buckner,
>
> JC, Uhm JH, and Shaw EG (2003) The neurocognitive effects of
>
> radiation in adult low-grade glioma patients. *Neuro-Oncology* 5, 161-
>
> 167, 2003.

I.  **General Rules for Determining Multiple Primaries: The following
    rules apply for defining multiple primaries for non-malignant and
    malignant primary intracranial and central nervous system tumors
    (C70.0 -- C72.9, C75.1 -- C75.3).**

> *Rationales for multiple primaries rules*:
>
> 1\. The natural biology of non-malignant tumors is that of expansive,
> localized growth, with
>
> local recurrences common, and metastasis uncommon or unusual.
>
> 2\. Non-malignant tumors of the same histology, same site, and same
> side will recur in the

same location. If they recur, even after 20 years, they are still the
same tumor.

> 3\. The corollary to statement 2 is that multiple non-malignant tumors
> of the same histology
>
> identified in different locations or sides of the CNS should be
> considered separate
>
> primaries.

A.  Multiple lesions in which all are non-malignant tumors

    1.  If different sites, then separate primaries

    2.  If different histologies, then separate primaries

    3.  If same site and same histology\*:

        a.  and laterality is same side, one side unknown or not
            applicable, then single primary

        b.  and laterality is both sides, then separate primaries

> \* Note: if two histologies are in the same group in Table 2, code the
> more
>
> specific histology

B.  Multiple tumors in which one was non-malignant and the other was a
    malignant lesion

> 1\. Non-malignant tumor followed by malignant tumor: separate
> primaries
>
> regardless of timing
>
> 2\. Malignant tumor followed by a non-malignant tumor : separate
> primaries
>
> regardless of timing

C.  Multiple malignant tumors

    1.  If same histology:

        a.  \< 2 months:

> i\. 1 if same site
>
> ii\. 2 if different site and not stated to be a recurrence or
> metastases

b\. 2+ months (site does not matter):

i.  2 unless stated to be a recurrence or metastases

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2.  If different histologies:

    a.  \<2 months:

> i\. 2 if same site unless one is more specific histology
>
> ii\. 2 if different site

b.  2+ months:

    i.  always 2 primaries

> D. **Table 2. Histologic groupings to determine same histology for
> non-malignant**
>
> **[brain tumors]{.underline}**

+----------------------------------+-----------------------------------+
| > Gliomas\*                      | > 9380, 9381, 9382, 9400, 9401,   |
|                                  | > 9410, 9411, 9420, 9421, 9423,   |
|                                  | > 9424, 9430, 9440, 9441, 9442    |
+----------------------------------+-----------------------------------+
| > Subependymomas                 | > 9383, 9384                      |
+----------------------------------+-----------------------------------+
| > Choroid plexus neoplasms       | > 9390                            |
+----------------------------------+-----------------------------------+
| > Ependymomas                    | > 9391, 9392, 9393, 9394, 9444    |
+----------------------------------+-----------------------------------+
| > Neuronal and neuronal-glial    | > 9412, 9413, 9505, 9506          |
| > neoplasms                      |                                   |
+----------------------------------+-----------------------------------+
| > Oligodendrogliomas             | > 9450, 9451, 9460                |
+----------------------------------+-----------------------------------+
| > []{.mark}                      |                                   |
+----------------------------------+-----------------------------------+
| > \* includes gliomas,           |                                   |
| > astrocytomas, astroblastomas,  |                                   |
| > and glioblastomas              |                                   |
+----------------------------------+-----------------------------------+

*Rationale:* Brain tumor histologies grouped in Table 2 do not follow
the standard 3-digit histology difference rule because they represent a
progression, differentiation or subtype of a single histologic category.

In a review of the ICD-O histology codes, applying the current 3 digit
histology rule to non-malignant tumors would combine tumors that are no
longer considered to be biologically related.

II. **Collection of additional data**

A. Laterality

> For each of the following malignant and non-malignant primary brain
> and CNS tumors with a diagnosis date on or after January 1, 2004, code
> Laterality using codes 1- 4 or 9 (for paired sites). Midline tumors
> are coded Laterality = 9.

# Table 3. Sites to be coded with laterality

> C70.0 Cerebral meninges, NOS
>
> C71.0 Cerebrum
>
> C71.1 Frontal lobe
>
> C71.2 Temporal lobe
>
> C71.3 Parietal lobe
>
> C71.4 Occipital lobe
>
> C72.2 Olfactory nerve
>
> C72.3 Optic nerve
>
> C72.4 Acoustic nerve
>
> C72.5 Cranial nerve, NOS

All other subsites of C70.\_ , C71.\_ and C72.\_ are coded Laterality =
0 (not a paired organ) regardless of the date of diagnosis. All
pituitary and pineal gland and craniopharyngeal duct tumors (C75.1-3)
are coded Laterality = 0 (not a paired site).

All primary brain and CNS tumors diagnosed prior to January 1, 2004, are
coded Laterality = 0 (not a paired site).

*Rationale:* a*.* Researchers, including epidemiologists, have requested
the

> collection of laterality *(Inskip PD, Neuroepidemiology 2003;*
>
> *22;130-138*). The location of certain tumors might help in
>
> determining causation. Certain investigations such as those
>
> involving cell phone usage would benefit from having this variable
>
> routinely available.

b\. Laterality is needed to determine multiple primaries for benign
brain

> tumors.
>
> B. WHO Grade Code
>
> This item is to be coded in Site Specific Factor 1 of the
> Collaborative Staging System for
>
> Brain and other Central Nervous System sites.
>
> WHO Grade I - Code 010 in Collaborative Staging System
>
> WHO Grade II - Code 020
>
> WHO Grade III - Code 030
>
> WHO Grade IV - Code 040
>
> WHO Grade unknown - Code 999
>
> WHO grade I generally describes non-malignant or benign tumors;
> however, non-malignant tumors should not be coded as Grade I unless
> specifically stated as such in the source document.
>
> WHO grade II generally describes a malignant tumor but it can describe
> a non-malignant tumor depending on histologic type.
>
> WHO grade III and IV describe malignant tumors.
>
> For certain types of CNS tumors, no WHO grade is assigned.
>
> C. Reportability/Sequence number

1\. Non-malignant: a primary non-malignant tumor of any of the sites
specified diagnosed *on or after* January 1, 2004, is reportable. The
sequence number for the tumor is in the range 60 -- 87.

> Non-malignant tumors diagnosed before January 1, 2004 should be
> included in the lifetime sequence of non-malignant and borderline
> tumors in the range 60-87.
>
> A primary non-malignant tumor of any of the sites specified diagnosed
> *before* January 1, 2004, is not reportable unless there are specific
> preexisting regional or state reporting requirements.
>
> *Rationale:* To clarify reporting implementation date and sequence
> rules for non-malignant tumors*.*

2\. Malignant: the sequence number for the malignancy is in the range
00-35.

3\. The sequencing of non-malignant tumors does not affect the
sequencing of malignant

tumors, and vice versa. For example, a first malignancy (sequence 00)
will remain

sequence 00 if followed by a non-malignant tumor (sequence 60-87)

III. **Analysis/Reporting of Brain and CNS Tumors:**

The ROC Benign Brain Tumor Subcommittee **recommends** that
non-malignant and malignant brain tumors be reported separately with a
footnote that pilocytic astrocytomas are included in analysis for
malignant brain tumors for continuity of trends.

> **We recommend** reviewing the standard site and histology groupings
> for tabulating estimates
>
> of these tumors to allow comparability of information across
> registries.
>
> **We recommend** that training for reporting and tabulating primary
> intracranial and
>
> CNS tumors be offered on a regular basis.

**Registry Operations Committee Benign Brain Tumor Subcommittee**

**Members:**

Susan Bolick-Aldrich (Chair) SC Central Cancer Registry, Registry
Operations Committee Co-Chair

Trista Aarnes-Leong St. Vincent Medical Center, Los Angeles

Gayle Clutter National Program of Cancer Registries, CDC

April Fritz SEER Program, NCI

Susan Gershman, PhD Massachusetts Cancer Registry, Registry Operations
Committee

> Co-Chair

Bette Smith Ohio Cancer Incidence Surveillance System

Carol Kruchko Central Brain Tumor Registry of the US

Bridget McCarthy, PhD Central Brain Tumor Registry of the US

Roger McLendon, MD Neuropathologist, Duke University Medical Center,
Durham, NC

Fran Michaud National Program of Cancer Registries, CDC

Eileen Morgan Duke University Medical Center Cancer Registry, Durham, NC

Donna Morrell USC School of Medicine Cancer Surveillance Program, Los
Angeles

Jerri Linn Phillips American College of Surgeons, Commission on Cancer

Katheryne Vance California Cancer Registry

Shannon Vann Independent contractor, NAACCR

Claudia Feight Oregon State Cancer Registry

James Gurney, PhD University of Minnesota Division of Pediatric
Epidemiology

Elaine Hamlyn Canadian Cancer Registry, Statistics Canada

Linda Mulvihill North Carolina Central Cancer Registry

James Smirniotopoulos, MD Dept of Radiology & Radiological Sciences,
Uniform Services

> University of the Health Sciences

Valerie Vesich American College of Surgeons, Commission on Cancer

#### SEPARATE ISSUE FOR UDS DISCUSSION

#### 

#### Genetic Syndromes

> The ROC Benign Brain Tumor Subcommittee recommends that these
> syndromes be collected for brain and central nervous system tumors.
> The genetic syndromes listed below are mutually exclusive; one person
> would not be diagnosed with more than one of these syndromes.
>
> These syndromes include:
>
> Number range CHAPTERNeurofibromatosis I (von Recklinghausen's 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 (p-53 Syndrome)
>
> Familial Retinoblastoma
>
> Turcot Syndrome (Adenomatous Polyposis Syndromes)
>
> Cowden Disease
>
> Multiple Endocrine Neoplasia Type I (Wermer Syndrome)
>
> Multiple Endocrine Neoplasia Type II (Sipple Syndrome)
>
> *Rationale:* Several genetic conditions/syndromes are associated with
> increased
>
> predisposition to the development of certain brain neoplasms.
>
> Therefore, knowledge of the presence of such conditions is
>
> important in reaching a proper diagnosis. *Reference: ACoS*
>
> *Brain/Spinal Cord Patient Care Evaluation Protocol. Pg. 9*
>
> Many of these syndromes are associated with the occurrence of numerous
> primary brain tumors in each affected patient; others are associated
> with the early onset of malignant brain tumors. Knowledge of the
> incidence and the financial impact of these inherited brain tumor
> syndromes are markedly incomplete because they are based on individual
> hospital case records not captured in statewide data.

**The subcommittee recommends** that the Uniform Data Standards
Committee consider adding a separate field to the data set so that
genetic syndrome information can be collected for other sites as well.