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