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44 Cards in this Set

  • Front
  • Back

most common renal tumor of infants?

congenital mesoblastic nephroma

age during which congential mesoblastic nephroma usually presents?

< 3-6 months

is congenital mesoblastic nephroma benign or malignant?

benign

Is HTN a/w congential mesoblastic nephroma?

NO

tx for congential mesoblastic nephroma?

nephrectomy

is congential mesoblastic nephroma usually u/l or b/l? is it a/w polyhydramnios or oligohydramnios?

u/l

polyhydramnios

mean age of diagnosis of wilms' tumor?

3.5 years

genes/chromosomes a/w wilms' tumor

WT1 - 11p13
WT2- 11p15

what is the classis triphasic pattern a/w wilm's tumor?

Blastema, Epithelium, Stroma- Triphasic
("BEST")

What must you be concerned for if you see nephrogenic rests, esp if < 12 mo?

b/l wilms' tumor

what is always present in b/l wilms tumor?

nephrogenic rests

what is the most important prognostic factor in wilms tumor?

histology (favorable)

how does wilms' present?

large, smooth, firm flank mass

aniridia is a/w what childhood renal tumor?

wilm's tumor

does wilms cross midline?

No

does neuroblastoma cross midline?

yes

are patients with wilms well or ill appearing?

well

are patients with neuroblastoma will or ill appearing?

ill

what is the treatment for wilms tumor?

nephrectomy unless stage 5- partial after chemo

when do you use neoadjuvant chemo for wilms' tumor?

what agents?

B/L, unresectable, solitary kidney, extensive intracaval involvement

vincristine, dactinomycin and/or doxorubicin

when and where do most wilms' recurrences occur?

< 2 years after diagnosis

lung

what syndromes is wilm's a/w?

Denys- Drash
WAGR
Beckwith-Wiedeman

what do you find in a patient with Denys-Drash?

wilms' tumor
DSD- cryptorchidism, hypospadias (proximal)
nephropathy

what do you find in a patient with WAGR?

Wilms'
aniridia
GU abnormalities (hypospadias, UDT)
MR

what do you find in a patient with Beckwith-Wiedeman?

wilm's
macroglossia
hemi-hypertrophy
visceromegaly

how often do you image (RBUS) patients with syndromes a/w wilms'?

RBUS q 3 months

when does neuroblastoma present?

< 4yo
50% < 2yo

homer-wright rosettes are a/w what childhood tumor?

neuroblastoma

how does neuroblastoma present?

hard, fixed, abdominal mass crossing midline

what lab value abnormalities in neuroblastoma?

levated norepi
urinary VMA

most common treatment for neuroblastoma? why?

observation

spontaneously regress

if a patient presents with raccoon eyes (periorbital ecchymosis) what childhood tumor do you think of? why?

neuroblastoma

periorbital mets --> edema, proptosis, raccoon eyes

if patient has localized, metastatic (skin, liver, bone marrow) neuroblastoma and age is < 1yo, what is the treatment?

nothing. spontaneous regression

location of rhabdomyosarcoma with the worst prognosis?

prostate > bladder

location of rhabdomyosarcoma with the best prognosis?

vagina

childhood renal tumor with the worst prognosis overall?

rhabdoid tumor of the kidney

rhabdoid tumor often metastasizes to where? what tests must be ordered?

brain

brain CT/MRI

treatment for rhabdoid tumor?

nephrectomy then chemo (carboplatin, etoposide, cyclophosphamide)

average age of diagnosis of rhabdoid tumor?

13 months

what is the treatment for children with large complex cystic lesions?

nephrectomy

2 yo male patient presents with u/l, painless abdominal mass. cystic on imaging. what is most likely diagnosis? tx?

multilocular cystic nephroma

nephrectomy

common presentation of renal vein thrombosis

abdominal mass, thrombocytopenia (decreased platelets), gross hematuria

mesoblastic nephroma

most common solid tumor in NEWBORN


benign but may be local invasive


present ave 3.5 months, uncommon > 6mos


a/w polyhydramnios


14% a/w congenital abnormality


firm, rubbery


no capsule or calcification


prone to rupture


microspindle cells


Tx= rad nephrectomy, avoid tumor spill

what is the most important prognostic factor regarding wilms tumor?

unfavorable histology