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
what does the blastemal component of wilms tumor look like
densely packed small blue cells
what is the prognosis for wilms tumor patients
majority survive
what does the prognosis of wilmt tumor depend on
histo and stage of disease
the possible mutation involved
what mutation is the worse for wilms tumor
1q gain is worse
are olde ror younger patients have better prognosis for wilms tumor
older
what is the most critical prognositc element in wilms tumor
the absence or presence of diffuse anaplasia
