1. 2018 Solid Tumor Rules: What’s New- Working through the rules
Lois Dickie, NCI/SEER
2017 Multi-state Regional Fall Conference
Lakewood, Colorado
September 15, 2017

2. Agenda Introduction/Process Activity & challenges
Revisions: What’s new
Next steps
Implementation

3. Solid Tumor Rules Revisions
Revised Solid Tumor rules implementation delayed until 2018
NAACCR Registry Steering Committee requested the revised rules be delayed until 2018
Due to the expected changes in AJCC 8th Ed and accompanying education issues, it would be unreasonable to implement three new data collection manuals/rules
Revisions and additions of Site Specific Diagnostic Indicators (SSDI formerly SSF)
Why the delay in updating MPH

4. Solid Tumor Rules Revisions
New ICD-O-3 codes, changes in behavior codes must be included in the revised rules
Revised 4th Ed Tumors of CNS
4th Ed Tumors of Breast
4th Ed Tumors of Digestive System
4th Ed Tumors of Female Reproductive Organs
4th Ed Tumors of Head & Neck
4th Ed Tumors of Lung
4th Ed Tumors of Soft Tissue & Bone
4th Ed Tumors of Urinary System & Male Genital Organs
While the 4th Ed Digestive System was released in 2011, the others were released between 2016 and 2017

5. Solid Tumor Rules: Update Process
Review SINQ & Squish: identify issues
1000+ MPH questions in SINQ
8600+ MPH questions sent to AASR
Consult with specialty matter experts
WHO Classifications of Tumors (aka: Blue Books)

6. Solid Tumor Rules: Update Process
1st revision-ST project team reviews
Three members
2nd revision- ST task force reviews
40+ members from central & hospital registries, standard setters, vendors, physicians
Final revision based on task force review

7. Solid Tumor Rules: Update Process
QC of ICD-O-3 codes and histology terms
Beta testing if necessary
Statistical review: impact on rates
Contractor-508 compliant
Edits
Education
Implementation

8. Challenges and delays
Revisions began in 2011with half site groups ready for final review by early 2013
Support resources re-allocated to other projects
Specialty mater experts (SME’s) committed to 8th Ed and 4th Ed WHO blue books
Second revision needed due to WHO 4th Ed blue books released between 2016 and 2017
Standard setters primary focus is TNM, EOD, and summary staging
Experts and standard setter representatives were and continue to be spread thin between the many major projects currently in play:
In the “old days” AJCC did their thing with TNM, SEER controlled MPH, EOD, and SS. Then CS became a joint project.

9. What’s new for 2018
The 2018 Solid Tumor rules will include comprehensive revisions for the following site groups only:
Benign brain/CNS
Malignant brain/CNS
Head & Neck
Colon
Lung
Breast
Kidney
Urinary

10. What’s new for 2018
The 2018 Solid Tumor rules will include minor revisions to the following site groups only:
Cutaneous melanoma
Other

11. Delay in two site groups
Cutaneous melanoma
WHO is expected to release 4th Ed Tumors of Skin sometime in 2018
Other
WHO just released 4th Ed Tumors of Endocrine Organs
Plan to break-out GYN, soft tissue/bone, endocrine, and male genital into individual site groups or sub-sites within Other

12. What’s new Text format only (T&D, M rules and H rules combined)
No change in how you use the rules: STOP at 1st rule that applies
Expanded histology tables
WHO grade tables (Brain site groups)
Determining primary site (H&N)
Non-reportable neoplasms
Added notes and examples

13. Revisions: what to expect

14. Benign & Malignant Brain/CNS
We collaborate with Central Brain Tumor Registry of the United States (CBTRUS) on benign and malignant brain rules
CBTRUS developed the rules for 2007 manual
Recognized the rules resulted in over-reporting of certain tumors (meningiomas for example)
Reporting neurofibromatosis
BB and MB were ready for beta testing when WHO released an update to the 4th Ed CNS Tumors

15. BB Summary of sections I, II, and III
Section I
Coding behavior for CNS tumors
Section II
Reportable primary sites and histologies
Section III
Additional Reportability and coding instructions

16. BB Section I: Behavior coding
 Behavior determines whether the Non-Malignant CNS rules apply
Priority order for using information for assigning behavior
Pathology
Cytology
Pathology done, no report, use physician statement
Imaging
If instructions 1-4 do not apply, use Table 1  WHO Grades for CNS to determine behavior (hyperlink to Table 1)

17. BB Section II: Reportable Primary Sites & Histologies
Primary site definitions:
Cranial
Extramedullary
Extracranial
Meninges, intraosseous meninges
Cranial and spinal nerves
Cavernous sinus
Sphenoid wing
Priorities for coding primary site

18. BB Section III: Additional coding instructions
Section III includes instructions and notes for the following tables:
Table 6: Histology NOS term, and associated synonyms, subtypes, and variant
Table 7: Paired sites
Table 8: Non-malignant CNS tumors with potential of transforming to malignant behavior

19. Table I: Who Grades General Information
Instructions for coding grade in CNS tumors
Table lists histology term, additional information is appropriate, and corresponding WHO grade
Do not code WHO grade in the field “Grade of Tumor”
WHO Grade for CNS does not parallel the ICD-O grade code (6th digit of the morphology code)
Code WHO grade in the Site-Specific Factor (SSF) for CNS neoplasms. Do not enter ICD-O behavior code in SSF field

20. Table II: Reportable primary sites & codes
Table lists site term and ICD-O topography code
Hyperlink back to primary site definitions

21. Table III: Reportability of Non-Malignant Cranial Nerve (CN) Tumors
General instructions
Table includes:
nerve name and cranial nerve number
Related reportable sites
Non-reportable sites

22. Table 4: Non-reportable neoplasms
Table includes:
Histology term
ICD-O morphology code
Definitions and sites

23. Table 5: Histologic Types of Non-Malignant Intracranial (Brain and Glands) Tumors
Because non-Malignant brain and gland tumors are less common, this table identifies histologies which occur in the brain (C710-C719) and the glands within the cranium (C750-C753).
Hyperlinks through out

24. BB M rules
Added rules for cases with initial clinical diagnosis of benign tumor with subsequent resection with pathology noting malignant (/3) tumor
Active surveillance
Changing information or correcting original abstract
Multiple meningiomas

25. BB H rules No major changes Additional notes and examples
Incorporate new histology codes
It is important to refer to ICD-O-3.1 AND the 2018 ICD-O-3 update documents as they include many new codes and alternate names for CNS tumors
The malignant brain rules will be very similar to the benign. CBTRUS is looking at more new codes for malignant histologies and we are waiting to hear from WHO CNS authors

26. Head & Neck Terms & Defs Expanded equivalent/equal terms
Added terms that are not equal
Instructions for coding primary site
Priority rules for identifying primary site
Table 1: Contiguous sites
Site group, codes within site group, and contiguous site(s)

27. Head & Neck Terms & Defs Tables 2 – 8: Common histology by site
NOS term, synonym, NOS code and variant/subtype ICD-O code
A histology may be common to one site but not another
Table will assist in determining primary site
Table 9: Sites for which laterality must be coded

28. Head & Neck M rules
Added the following to unknown, single tumor & multiple tumors:
Note 1: These rules are NOT used for any tumor(s) described as metastases.
Note 2: The rules list ONLY the four-digit histology code; they apply to in situ /2 and invasive /3 malignancies. Code behavior as stated on pathology report.
Note 3: NOT REPORTABLE: Basal cell carcinoma of skin of lip, vermillion border or vermillion surface of lip. Basal cell originates in skin; it is not reportable.

29. Head & Neck M rules
Instructions for coding single versus multiple primaries based on overlapping tumors, contiguous tumors and separate tumors
Multiple primary rule for tumors diagnosed more than 5 years apart now includes instruction rule applies only when the patient has been disease free or NED for 5 years

30. Head & Neck H rules Repeat priorities for coding histology
Clarified terms focal, foci, and focus are not used to indicate a more specific histology
Clarified terms used to identify subtypes or variants of in situ tumors
Clarified terms used to identify subtypes or variants of invasive tumors

31. Breast Terms & Definitions
IMPORTANT CHANGE
NST (no specific type) and carcinoma NST are the new terms for duct or ductal carcinoma. Previously, it was thought that carcinoma originated in the ducts or lobules of the breast, hence the names duct carcinoma and lobular carcinoma. Current thinking is that carcinoma originates in the “terminal duct lobular unit” therefor the preferred term is NST or carcinoma NST.
DCIS/Carcinoma NST in situ has a major classification change
It is very important to code the grade of all DCIS/carcinoma NST in situ
The current breast WHO emphasizes coding the grade of tumor rather than the subtype/variant
Internationally, pathologists use the WHO editions to keep their nomenclature and histology identification current
Over time, subtypes/variants will be diagnosed less frequently

32. Breast Terms & Definitions
Code histology that is majority of tumor
Previously reportable terms are not used including differentiation, components of, features of, etc.
See Histology  coding rules for definition of/criteria for majority of tumor
 Hyperlink to Histology coding rules

33. Breast Terms & Definitions
Equivalent or equal terms
Duct, ductal, NST (no specific type); carcinoma NST, mammary carcinoma

34. Breast Tables 1 & 2 Table 1: Primary site codes
ICD-O site/topography code/term/terms & descriptive language
Table 2: Combination histology codes

35. Breast Table 3: AJCC Table 3: NOS or NST with subtypes/variants
AJCC STATEMENTS
There are non-specific codes which cannot be mapped to more specific histology
Diagnosis is too vague to allow mapping
Cancer registry may collect as codes are included in the AJCC category of “allow for clinical diagnosis only ”
Allow for registry collection only (histology not listed in AJCC breast chapter)
Cancer registries are allowed to use this code for case reporting
Code is not included in CAP Protocols or AJCC 8th edition staging manual because it is not an appropriate diagnosis for patient care AND
Code(s) should only be used in a physician’s clinical diagnosis (no pathology report)
AJCC had requested we include this information in the site rules however, they are now re-considering

36. Breast Table 3: AJCC AJCC Statement about Histology Not Staged in AJCC
Notes will inform
Appropriate chapter which should be used for staging
Which histology-site combinations cannot be staged in the AJCC 8th edition
Data in the AJCC chapter are based on common histologies. Rare, but valid, histologies do not have AJCC staging criteria (T, N, M, and stage grouping)

37. Breast M rules M4: Multiple tumor timing rule
Clinically disease free >5 years is new primary
When a recurrence occurs <5 years from diagnosis, the “clock” resets using the date of last recurrence
Example: Pt DX’d 1/2010 with R breast ductal ca. “New” tumor found 1/2014 in right breast with ductal ca. This is a single primary. A third tumor is found 1/2016 in the right breast, again with ductal ca. This is not a new primary as it occurred <5 years following the 2014 tumor. Do not use the original 2010 diagnosis date
Most important rule and clarification

38. Breast H rules Major change:
Do not code differentiation or features unless there is a specific code for the NOS with differentiation
invasive breast carcinoma with neuroendocrine differentiation 8574
Invasive breast carcinoma with neuroendocrine features 8500
Effective 1/1/2018, mammary carcinoma will be coded as carcinoma NST 8500 rather than carcinoma NOS 8010

39. Breast H rules
Per WHO 4th Ed Tumors of Breast, several histologies must meet specific criteria
Percentage of type required
Clarified and expanded how to code mixed histologies such as metaplastic, NOS with a single subtype/variant and how to code metaplastic, NOS with more than one subtype/variant

40. Colon/Rectum Terms & Defs
Important changes:
Malignancies arising in the appendix are reportable
There are dysplasias that have the suffix /2 in WHO and in the ICD-O-3 Addendum. They are not reportable in the US. They are reportable in Canada.
Dysplasia was not collected in the past. If dysplasia is added to the database with the same code as in situ tumors, there will be a huge upsurge in the incidence of in situ neoplasms
There would be no way to separate the dysplasias from the in-situ neoplasms in the database, which would cause problems with surveillance (long-term studies) since the prognosis and probabilities of disease progression are different between an in-situ tumor and a dysplasia, however, pathologists frequently use the term “severe dysplasia” in place of carcinoma in situ. We code CIS only if it is expressly stated.

41. Colon/Rectum Terms & Definitions
Added definitions for:
Local recurrence
Local metastasis
Regional recurrence
Regional metastasis
Anastomosis

42. Colon/Rectum Terms & Definitions
Table 1: Colon, Rectum, & Appendix Histology NOS and Variant s or Subtypes
Include synonyms and ICD-O codes
Table 2: ICD-O Histologies NOT reportable for colon, rectum, and appendix
Benign adenomas and syndromes

43. Colon/Rectum M rules Currently revising multiple polyps rule
2 polyps with different histologies in same site
New rule for recurrent or new tumor in anastomotic site

44. Colon/Rectum M rules
Abstract a single primary 1 when a subsequent tumor arises at the anastomotic site AND
The tumor arises in colon/rectal wall and/or surrounding tissue; there is no involvement of the mucosa AND/OR
The pathologist’s diagnosis is an anastomotic recurrence
Note 1: The physician will commonly stage the subsequent tumor because the depth of invasion determines the second course of treatment. Staging and determining multiple primaries are done for different reasons. Staging determines which course of treatment would be most effective. Determining multiple primaries is done to stabilize the data for the study of epidemiology (long-term studies done on incidence, mortality, and causation of a disease with the goal of reducing or eliminating that disease)
Note 2: These tumors are recurrences. Registrars that collect recurrence information should record the information in the recurrence fields

45. Colon/Rectum M rules
Abstract a multiple primaries ii when a subsequent tumor
Arises in the mucosa at the anastomotic site AND
The pathologist’s diagnosis does not mention an anastomotic recurrence
Note 1: The tumor may or may not invade into the colon wall or adjacent tissue.
Note 2: These rules are hierarchical. Use this rule only when rules M1-M9 do not apply.

46. Colon/Rectum H rules
Included priority order for using documents to code histology
Change in priority coding of carcinoma in a polyp over a subsequent more specific histology
Example: adenocarcinoma in a polyp (8210) followed by segmental resection showing mucinous adenocarcinoma (8480/3). Per 2007 rules, the polyp has priority. Per the 2018 rules, the mucinous histology is coded as this histology will determine treatment and survival/prognosis

47. Lung Terms and Definitions
Major changes
In situ adenocarcinoma (AIIS) terms and codes have been added
New term and code for non-mucinous adenocarcinoma minimally invasive
Code for mucinous adenocarcinomas have changed; change of codes was implemented so mucinous and colloid adenocarcinomas could be analyzed separately
New terms and codes have been added for mucinous carcinoma minimally invasive and mucinous adenocarcinoma pre-invasive and mucinous carcinoma in situ

48. Lung Terms and Definitions
The term and code 8255 for adenocarcinoma with mixed subtypes has become obsolete. It is used ONLY when there are two subtypes/variants of the same NOS and the % of each subtype/variant is not documented
Note 1: As described in the Multiple Primary and Histology coding rules, lung tumors are classified on the basis of the best differentiated (most specific, subtype/variant) portion of the tumor, even if it is a minority of the cell type.  For example, a predominantly undifferentiated carcinoma, or large cell (undifferentiated) carcinoma, with focal squamous differentiation is a squamous carcinoma, even if this only a 10% component. 
Note 2: I Do not use 8255 as a “go to” code. This category has many histology combinations which makes it impossible to analyze. See the priorities for coding histology combinations in the Histology Coding Rules

49. Lung Terms and Definitions
Definitions for local, regional, and distant metastasis
Updated equivalent and not equivalent terms
Table 1: Primary site
Terminology, site code, ICD-O code, laterality
Table 2: Combination/mixed histology codes
Table 3: Nos and subtypes/variants
Includes synonyms and ICD-O codes

50. Lung M rules
Additional notes and examples have been added to timing rules for multiple tumor. Similar to breast timing rules

51. Lung H rules
Added rules for new histologies

52. Kidney rules Minor additions to Terms and Definitions
Minor updates to both M and H rules to reflect new histologies

53. Urinary rules Minor updates to terms and definitions
Clarification of multiple tumor rules specifically M6, M7, and M8
Minor updates to H rules
Consulting with our SME’s how best to code mixed tumors

54. Cutaneous Melanoma rules
Minor updates to terms and definitions
No changes in M or H rules for 2018
The cutaneous melanoma rules will be revised after the WHO 4th Ed Skin Tumors is released in The updated rules will be effective 1/1/2019

55. Other sites rules Minor updates to terms and definitions
No expected updates to M or H rules for 2018
Other Sites rules will undergo a comprehensive revision in GYN, soft tissue/bone, endocrine and male genital will require major updating to reflect changes in WHO 4th Ed blue books. The updated Other sites rules will be effective 1/1/2019

56. Standard updates for ALL sites
Priority documents used to code histology will include CAP protocols
Hyperlinks
Rules for clinically free of disease/NED
Recurrence vs. new primary for lung, breast, urinary, and female genital
???AJCC statement

57. Solid Tumor Committee

58. Questions, comments………

59. Lois Dickie, Public Health Analyst, NCI/SEER dickielo@mail.nih.gov
Thank you!
Lois Dickie, Public Health Analyst, NCI/SEER