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29 Cards in this Set

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  • Back
what's another name for Wilms' tumor?
nephroblastoma
how often is wilms' tumor bilateral?
5%; multifocal in 12%
what is the most common GU tumor of children?
wilms' tumor; incidence 8: 1,000,000
Which genetic loci are important in WT?
11p13
11p15
16q
T/F: most sporadic cases of WT are genetic.
False; only 10%
what are the 3 elements in the favorable histology variant of WT?
epithelial
blastomal
stromal
describe the anaplastic variant of WT
only 5% of specimens, >50% of deaths; syn. unfavorable histology
what are nephrogenic rests?
persistent nephrogenic blastemal cells; found in <1% of normal infant autopsies, 45% in unilateral WT and 100% of bilateral cases
why is local LN sampling important with nephrectomy in WT?
absence of LN in the specimen = automatic upstaging to Stage III
what is the NWTS staging for WT?
I - confined tumor, total resection
II - outside capsule (bx, spill), total resection
III - incomplete resection, massive spill, +LN
IV - distant mets (hematogenous)
V - bilateral tumors
what are the 2 most common post-op complications from WT nephx?
11% complication rate; hemorrhage and bowel obs
what are criteria for presurgical downstaging with chemoTx in WT?
bilateral, unresectable, solitary kidney or major vascular involvement
what is the survival rate for FH WT stages I-III?
>90%
what is congenital mesoblastic nephroma?
most common renal neoplasm in infants <3mos and almost universally assoc with a benign course; nephx w/o chemoTx
child with abdominal mass that crosses midline
think neuroblastoma; check urine VMA; >70% present with mets
what is the international neuroblastoma staging system?
1 - localized with complete resection
2a - localized w/o complete gross rx; (-) LN
2b - (+) ipsilateral and (-) contralateral LN
3 - unresectable local tumor and/or (+) contralateral LN
4 - distant hematogenous or LN mets
4S - stage 1, 2a or b tumor with spread to skin, liver, and/or bone marrow (age < 1yo)
what is unique about stage IVS in neuroblastoma?
spontaneous regression is the norm but can have a high morbidity and even mortality from pulmonary issues
what are bad prognostic factors for neuroblastoma?
N-myc amplication
serum ferritin elevation
diploid DNA
tyrosine kinase A & B
what proportion of rhabdomyosarcomas arise from the GU tract?
1/3
what genetic abnormality is important in RMS?
2q37
how do you treat RMS?
surgery with bx of lesion + VAC (vincristine, actinomycin D, cyclophosphamide +/- ifosfamide) chemoTx; survival >90% for low stage, favorable histology
what is the most common testicular tumor in children?
teratoma (used to be yolk sac tumor)
what tumor marker in elevated in yolk sac tumor (YST)?
AFP is elevated in > 90%; bHCG is not useful marker to follow prepubertal testicular neoplasms; AFP is elevated for up to 6-9mos postpartum in males
boy with heterogeneous testis lesion on US with calcification and normal AFP
teratoma
what is the most common stromal testis tumor in kids?
leydig cell tumor
boy with precocious puberty, testis mass, elevated seum testosterone and urinary 17-ketosteroids
leydig cell tumor
phenotypic female 46XY with testes
needs prepubertal gonadectomy d/t risk for gonadoblastoma (80%)
what is the risk of cancer in cryptorchidism?
the higher the testis prior to orchiopexy, the higher the risk; 20% incidence in contralateral testis; risk overall 10-35X
T/F: orchiopexy reduces the risk of malignancy
True: orchiopexy prior to puberty significantly reduces the risk of malignancy