Pam Gaillard n n n Pamela is a

Pam Gaillard n n n 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.

Pediatric Renal Tumors

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

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

n n n Prognosis influenced by stage at diagnosis Overall survival 64% for all stages Treatment n n 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.

Rhabdoid tumor of the kidneys n Rare, highly aggressive malignancy of early childhood n n n 80% occur in pts. <2 yrs. Of age Median age of dx- 11 months Clinical manifestations n n n 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.

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

Clear Cell Sarcoma n n Uncommon renal neoplasm of childhood-20 new cases/yr. U. S. Aggressive behavior-higher rate of relapse/mortality than WT n n Usually presents with abd. Mass Treatment-nephrectomy/chemotherapy n n Long term survival-60 -70% Survival assoc. with stage at diagnosis Addition of Doxorubicin to VCR/Dactinomycin improved survival Bone metastasis most common Can recur long after therapy n 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.

Wilms Tumor n Wilms Tumor (nephroblastoma) predominant renal tumor in children 3 months -6 yrs. n n 90% overall survival Chromosomal abnormalities assoc. with some WT n Two locations on chromosome 11 n n 11 p 13 -WT 1 gene, 11 p 15 -WT 2 gene Abnormalities on 1, 12 and 8

n n Familial WT 1 -2% n FWT 1 at 17 Q 12 -q 21 n FTW 2 at 19 q 13. 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.

Genetic Syndromes n n n WAGR-WT, aniridia, genitourinary malformation, mental retardation; somatic germline deletions of 11 p 13 Denys-Drash-pseudohermaphroditism, severe glomerulopathy, WT; inactivating point mutations in the WT 1 gene, 11 p 13 Beckwith-Wiedemann-hemihypertrophy, macroglossia, omphalocele, visceromegaly; loss of heterozygosity in 2 nd WT locus on 11 p 15

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

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

Classification n n 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

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

Standard of Care n n 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 n n n Stage II, IV diffuse anaplasia Stage II, IV focal anaplasia Stage I diffuse or focal anaplasia Bilateral WT-preoperative chemotherapy and kidneypreserving resection. Use of Topotecan with relapsed pts. -WILTOP

Nursing considerations n Preoperative care n n Avoid palpation of abdomen Preop teaching for parents and ageappropriate teaching for child Monitor VS for possible hypertension, hemorrhage Teaching on central line, subcutaneous port

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

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

Long term effects n n n n n 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. org Nelson, MB and Meeske, K. Recognizing Health Risks in Childhood Cancer Survivors. Journal of the American Academy of Nurse Practitioners. 2005.