Exam2 Kids tumors-lecture slides Flashcards
which demographics are most affected by nephroblastomas (Wilms Tumor)
asians>whites>black
What are the 4 most common childhood malignant
Acute Leukemia, Neuroblastoma, Retinoblastoma and Wilms
What ages does the peak Dx occur with wilms tumor
2-5 yrs
what is clinical presentation of wilms tumor
large abdominal mass, may exhibit pain, microscopic hematuria, HTN
What other congenital syndromes are associated with Wilms tumors
WAGR: wilms, aniridia, genital, retardation
Denys Drash
Beckwith Wiedemann
What syndromes are assoc with a chromosome 11 WT1 mutation leading to wilms
WAGR and Denys Drash
which syndrome is associated with gonadal and renal tumors, 90% wilms
denys drash
What gene is affected in Beckwith wiedemann syndrome
WT2
Do Majority of wilms tumor patients have WT1 mutation or other genetic syndromes
no 90% previously healthy
what is the precursor lesion in Wilms tumor
nephrogenic rests, 100% bilateral
What are the 3 cell types in wilms tumors
blastemal, epithelial and stromal
describe the unfavorable histo of a wilms tumor
anaplastic
focal or diffuse
diffuse anaplasia is worse
anaplasia associated with p53 mutations
what is the morphology of a wilms tumor
well circumscribed tan to gray
what does the stromal component of wilms tumor look like?
spindle shaped cells
what does the epithelial component of a wilms tumor look like
immature tubule
