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Pediatric Renal Tumors PowerPoint Presentation

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Description : This medical presentation on cancer titled "Pediatric Renal Tumors" talks about pediatric renal tumors like Wilms tumor, RCC, RTK, Clear cell sarcoma of the kidney.

Tags : Cancer, Pediatric Renal Tumors, Wilms tumor, RCC, RTK, Clear cell sarcoma of the kidney, Rhabdoid tumor, Genetic Syndromes

Published on : Apr 28, 2021 | Views : 3688 | Downloads : 3

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Pediatric Renal Tumors
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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
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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
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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.
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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.
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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
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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.
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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
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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.
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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 2nd WT locus on 11p15
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Clinical Presentation Most common asymptomatic abd. mass in children Abdomen pain,distention Fever Hematuria Hypertension Anorexia Vomiting
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Diagnostic evaluation History and physical Urinalysis Renal function tests Chemistries Abdominal USD Chest x-ray Abd/Chest CT
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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
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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
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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
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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
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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
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Radiation Monitor for skin changes If lung xrt- monitor for pneumonitis Monitor ovarian function
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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 2nd 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.