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Terms in this set (13)
What is Wilms Tumor?
Embryonal tumor of renal origin
What is the incidence?
Very rare: 500 new cases in the United States per year
What is the average age at diagnosis?
Usually between 1 and 5 years of age
What are the symptoms?
Usually asymptomatic except for abdominal mass; 20% of patients present with minimal blunt trauma to mass
What is the classic history?
Found during dressing or bathing
What are the signs?
Abdominal mass (most do not cross the midline); hematuria (10%-15%); HTN in 20% of cases, related to compression of juxtaglomerular apparatus; signs of Beckwith-Wiedemann syndrome
What are the diagnostic radiologic tests?
Abdominal and chest CT
Define the stages:
Stage I Limited to kidney and completely resected Stage II Extends beyond kidney, but completely resected; capsule invasion and perirenal tissues may be involved
Stage III Residual nonhematogenous tumor after resection
Stage IV Hematogenous metastases (lung, distal lymph nodes, and brain)
Stage V Bilateral renal involvement
What are the best indicators of survival?
Stage and histologic subtype of tumor; 85% of patients have favorable histology (FH); 15% have unfavorable histology (UH); overall survival for FH is 85% for all stages
What is the treatment?
Radical resection of affected kidney with evaluation for staging, followed by chemotherapy (low stages) and radiation (higher stages)
What is the neoadjuvant treatment?
Large tumors may be shrunk with chemotherapy/XRT to allow for surgical resection
What are the associated abnormalities?
Aniridia, hemihypertrophy, Beckwith-Wiedemann syndrome, neurofibromatosis, horseshoe kidney
What is the Beckwith-Wiedemann syndrome?
1. Umbilical defect
2. Macroglossia (big tongue)
4. Visceromegaly (big organs) (Think: Wilms' Beckwith-Wiedemann)
THIS SET IS OFTEN IN FOLDERS WITH...
Lipid disorders (PEARLS)
Pleural Diseases (PEARLS)
OTHER SETS BY THIS CREATOR
Renal cell carcinoma
Neoplastic Diseases of the Renal System (PEARLS)
Chronic Kidney Disease
Acid Base Disorders (PEARLS)