1. Pam Gaillard Pamela is a certified Family Nurse Practitioner with over 30 years of nursing experience. She has worked at St. Jude for over 7 years but has over 24 years as a nursing practitioner in primary care. She received her BSN and Masters from University of Tennessee Health Science Center Her area of expertise and research interests are in Desmoid tumors and renal tumors. She has been published in Pediatric Blood and Cancer on Hematometrocolpos in an adolescent female treated for pelvic Ewing sarcoma. She has been a presenter in the Oncology Nursing Society’s course on cancer and genetics. Pamela’s has a personal interest in complimentary/Alternative medicine for cancer treatment related side effects.

2. Pediatric Renal Tumors

3. Renal Cell Carcinoma 2-6% of all pediatric renal tumors Less than 2% of all cases of RCC occur in children Assoc. with von Hippel-Landau syndrome Multiple tumors, younger age at diagnosis R/o VHL in pediatric pts. Dx with RCC, esp if bilateral disease Clinical presentation Gross, painless hematuria-more common in RCC than WT Palpable mass Flank pain 20% present with metastasis at time of diagnosis

4. Several pediatric renal tumors Wilms tumor, RCC, RTK, Clear cell sarcoma of the kidney RCC, RTK and CCSK more rare in children Wilms tumor is most common- accounts for 87% of pediatric renal masses. Occurs 1:10,000 persons Peak incidence 3-4 yrs. 80% present< 5 yrs. Bilateral tumors in 4-13% of children

5. Prognosis influenced by stage at diagnosis Overall survival 64% for all stages Treatment Radical nephrectomy and regional lymphadenectomy Resistant to chemotherapy Interferon/Interleukin post-op for higher grade tumors ? Benefit Can have late recurrences Lowe, L., Isuani, B. et al. Pediatric Renal Masses: Wilms Tumor and Beyond. Radiographis. 2000;20: 1585-1603.

6. Rhabdoid tumor of the kidneys Rare, highly aggressive malignancy of early childhood 80% occur in pts. <2 yrs. Of age Median age of dx- 11 months Clinical manifestations Hematuria Fever Anemia Symptoms of metastatic disease-most pt. present with advanced disease Associated with synchronus/ metachronus primary intracranial masses Hypercalcemia Amar, Aneesa M.; Thomlinson, Gail, et al. Clinical Presentation of Rhabdoid Tumors of the Kidney. Journal of Pediatric Hematology. Vol. 23(2), February 2001, pp 105-108.

7. Treatment Worst prognosis of all renal tumors 18 month survival-20% Resection, chemotherapy, radiation therapy(? Benefit) Improved survival with VDCy alternating with ICE- Yamamoto, Suzuki, et al High dose chemo/autologous stem cell rescue under study May need to discuss palliative therapy/hospice care early

8. Clear Cell Sarcoma Uncommon renal neoplasm of childhood-20 new cases/yr. U.S. Aggressive behavior-higher rate of relapse/mortality than WT Long term survival-60-70% Survival assoc. with stage at diagnosis Usually presents with abd. Mass Treatment-nephrectomy/chemotherapy Addition of Doxorubicin to VCR/Dactinomycin improved survival Bone metastasis most common Can recur long after therapy Argani, Perlman et al. Clear Cell Sarcoma of the Kidney: A Review of 351 cases from the NWTSG Pathology Center. The American Journal of Surgical Pathology. Vol. 24(1), January 2000.

9. Wilms Tumor Wilms Tumor (nephroblastoma) predominant renal tumor in children 3 months -6 yrs. 90% overall survival Chromosomal abnormalities assoc. with some WT Two locations on chromosome 11 11p13-WT 1 gene, 11p15-WT 2 gene Abnormalities on 1, 12 and 8

10. Familial WT 1-2% FWT1 at 17Q12-q21 FTW2 at 19q13.4 Biallelic BRAC 2 mutations assoc. with multiple pediatric malignancies include. WT Ruteshouser,EC, Huff,V. Am J Med Genet C Semin Med Genet. 2004 Aug 15;129 Reid,S et al. Journal of Medical Genetics 2005; 42: 147-151.

11. Genetic Syndromes WAGR-WT, aniridia, genitourinary malformation, mental retardation; somatic germline deletions of 11p13 Denys-Drash-pseudohermaphroditism, severe glomerulopathy, WT; inactivating point mutations in the WT1 gene, 11p13 Beckwith-Wiedemann-hemihypertrophy, macroglossia, omphalocele, visceromegaly; loss of heterozygosity in 2 nd WT locus on 11p15

14. Clinical Presentation Most common asymptomatic abd. mass in children Abdomen pain,distention Fever Hematuria Hypertension Anorexia Vomiting

15. Diagnostic evaluation History and physical Urinalysis Renal function tests Chemistries Abdominal USD Chest x-ray Abd/Chest CT

17. Classification Stages I-V based on NWTSG; SIOP Stage r/t degree tumor has spread outside the kidney and ability to resect Stage I- 97% survival rate Stage IV-about 80% (NWTS-3) 5-10% have bilateral Wilm’s at dx

18. Prognostic Factors Age- younger age better prognosis Anaplasia- worse prognosis, 13% of pts.>5 yrs. Old Focal Diffuse LOH at chormosomes 1p and 16q- greater risk of relapse and mortality regardless of stage, histology

19. Standard of Care Stage 1,2- VA x 18 weeks Stage 3- VDA x 24 weeks; 10.8 Gy flank Stage 4- VDA x 24 weeks; 10.8 Gy flank, 12 Gy lung ( if metastasis) Anaplastic WT(diffuse)- cyclophosphamide improved survival Stage II,III,IV diffuse anaplasia Stage II, III, IV focal anaplasia Stage I diffuse or focal anaplasia Bilateral WT-preoperative chemotherapy and kidney- preserving resection. Use of Topotecan with relapsed pts.-WILTOP

20. Nursing considerations Preoperative care Avoid palpation of abdomen Preop teaching for parents and age- appropriate teaching for child Monitor VS for possible hypertension, hemorrhage Teaching on central line, subcutaneous port

22. Postoperative care Monitor for complications, good pulmonary hygiene Chemotherapy Monitor for side effects of drugs (general, specific) VCR Actinomycin D Doxorubicin Cyclophosphamide Teach parents fever/neutropenia precautions

23. Radiation Monitor for skin changes If lung xrt- monitor for pneumonitis Monitor ovarian function

24. Long term effects 4-5% develop kidney failure Higher incidence of renal dysfunction Reproductive dysfunction Pulmonary fibrosis if lung radiation used Congestive heart failure-Green et al (2001) cumulative incidence of CHF 4.4% 20 yrs after initial tx with Doxorubicin Scoliosis, asymmetry Muscle atrophy 2 nd primary tumors Long-term follow-up guidelines: www.childrensoncologygroup.orgwww.childrensoncologygroup.org Nelson, MB and Meeske, K. Recognizing Health Risks in Childhood Cancer Survivors. Journal of the American Academy of Nurse Practitioners. 2005.