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This study evaluates the documentation of Wilms tumor histologic presentation to improve awareness and prognosis. Wilms tumor characteristics, epidemiology, histopathology, and survival rates are discussed. Results show the importance of accurate documentation for treatment decision-making.
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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 NU58DP003901. The views presented are those of the authors and do not necessarily reflect the views or opinions of the CDC.
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.
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
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%
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
Wilms tumor histology small uniform nuclei Favorable histology Unfavorable histology (anaplasia) big bizarre nuclei
Wilms tumor - 4-Year Survival Diffuse anaplasia adversely affects survival more than any other disease characteristic! Source: American Cancer Society, https://www.cancer.org/cancer/wilms-tumor/detection-diagnosis-staging/survival-rates.html
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 2004-15. • 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.
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
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
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