1. Evaluating Adequate Documentation of Wilms Tumor Histologic Presentation
Martin Whiteside, DC, PhD, MSPH
Director, Tennessee Cancer Registry
NAACCR Annual Conference
June 14th-16th, 2016
Harold N. Lovvorn, III, MD, co-Author
This study was supported in part through a cooperative agreement obtained from the Centers for Disease Control & Prevention (CDC), 5 NU58DP
The views presented are those of the authors and do not necessarily reflect the views or opinions of the CDC.

2. Goals of Study
To determine the quality of Wilms tumor abstract-level data in one statewide central cancer registry.
To determine if Wilms tumor cases had histologic presentation documented in abstract-level text submitted by reporting facilities.
To determine whether this information was available in pathology reports for those Wilms tumor patients without documentation of histologic presentation.
To increase awareness of this important prognostic factor, i.e. histologic presentation, for Wilms tumor patients.

3. Wilms tumor
Intraoperative photo of a Wilms tumor in a 2 year old girl that occupies her entire abdomen; Lovvorn patient. For orientation, child’s chest is to right of picture, pelvis to the left, right side is top and left side is bottom of picture.

4. Wilms tumor - Introduction
Named after the German surgeon, Dr. Max Wilms
Most common renal tumor of children < 15 years of age
Constitutes 85% of all pediatric renal tumors
Fourth most common pediatric cancer overall
Only 1% of cases have a previously affected family member, suggesting it is not a classic, heritable cancer, though many genetic alterations have been observed
Affects both kidneys simultaneously in only 5% of all cases: these bilateral cases are classified as stage V

5. Wilms tumor - Epidemiology
Wilms tumor affects about 650 individuals of all ages every year in the United States
Girls are affected slightly more than boys
Most cases are diagnosed between the ages of 2-5 years
about 90% diagnosed before the age of 6 years
Africans and African-Americans most frequently affected
Overall 5-year survival in the U.S. is approximately 92%

6. Wilms tumor - Histopathology
Classic, triphasic cellular pattern with epithelial, blastemal and stromal components
Mimics kidney development
Two separate histologic types:
Favorable: 90% of tumors
Unfavorable
Unfavorable more difficult to treat
Diffuse anaplasia = unfavorable histology
Focal anaplasia = favorable histology
Part of the CAP Protocol

7. Wilms tumor histology Favorable histology Unfavorable (anaplasia)
small
uniform
nuclei
big
bizarre
Histology of Wilms tumor. Top two panels show small and uniform nuclei and represent favorable histology. Bottom two panels show big, bizarre nuclei denoting anaplasia or unfavorable histology

8. Wilms tumor - 4-Year Survival
Diffuse anaplasia adversely affects survival
more than any other disease characteristic!
Tumor Stage
Favorable Histology
Focal
Anaplasia
Diffuse Anaplasia I
95% - 100%
85% - 90%
75% - 80% II
80% - 85%
III
75% - 90%
50% - 70% IV
70% - 75%
30% - 45% V
65% - 70%
Source: American Cancer Society,

9. Summary of Methods
Primary site code C64.9 and histology code M-8960/3 were obtained from the Tennessee Cancer Registry (TCR) for the time period
Children less than 20 years of age were selected.
Since histologic presentation has no coded variable, abstract-level text was evaluated for histologic presentation
Pathology reports were obtained from facilities for cases not documenting histologic presentation
All cases extracted were histologically confirmed at diagnosis.

10. Results
130 Wilms tumor cases initially extracted from the TCR database; one case was incorrectly coded
Black children accounted for 33 cases, or 25.6% of cases
50 cases were males (38.8%) and 79 females (61.2%)
Most cases (n=57, 44.2%) were diagnosed at localized stage

11. Results Cont.
A total of 62 cases (48.1%) had text documentation of histologic presentation; 67 cases (51.9%) did not
Facility-specific documentation of histologic presentation reporting >10 Wilms cases (4 facilities) yielded the following percentages: 11.8%, 15.8%, 45.5% and 90.2%.
For cases evaluated to date lacking documentation of histologic presentation in the text, 100% of path reports actually provided this information

12. Path Report Examples – favorable histology
Demonstrate anaplasia usage, no mention of favorable

13. Path Report Examples Cont. – favorable histology
Combined favorable designation in Diagnosis Summary, but note in the Comment section it states no anaplasia present

14. Path Report Examples Cont. – unfavorable histology
Here is diffuse anaplasia and note to audience that the CAP Cancer Checklist used this designation as well; never mentioned unfavorable

15. Path Report Examples Cont. – favorable histology**

16. Path Report Examples Cont. – favorable histology
This comment is from the same path report in slide 13.

17. Path Report Examples Cont. – unfavorable histology
Example of unfavorable histology with microscopic description. Note that this is the only one amongst the many path reports I have evaluated where diffuse anaplasia was actually noted as unfavorable histology.

18. Conclusions
Histologic presentation of Wilms tumor is an important prognostic factor that guides treatment, yet cancer abstracts lack coded information for this important tumor characteristic
Unfavorable histologic presentation results in about a 10-15% reduction in overall 4-year survival at earlier stages with a significantly greater disparity in survival observed at stage IV (50% reduction in overall 4-year survival)
Overall, preliminary results signify that there needs to be better text documentation of Wilms tumor histologic presentation, since over half of patients lacked such documentation in the text
Pathology reports are the key medical record resource for obtaining this important information and 100% of path reports evaluated to date provided the information necessary to document histologic presentation in the abstract text

19. Questions?

20. Kidney Anatomy
This slide is FYI