4 - peds oncology Flashcards
1
Q
% wilms bilateral?
A
5%
2
Q
MCDK is a risk factor for what? and caveat
A
wilms tumor - would have to do 2,000 prophylactic nx for one prevention
3
Q
what is WT1 gene
A
deletion of 11p13
4
Q
what is WT2 gene
A
loss of heterozygosity of 11p15
5
Q
non WT gene abnormality seen in wilms and sig
A
1p and 16q loss of heterozygosity - inc risk of death/ relapse
6
Q
names of 3 genetic syndromes assd w wilms
A
WAGR, denys drash, beckwith wiedemann
7
Q
what is WAGR
A
wilms, aniridia, genital abnormalities, MR
8
Q
gene for WAGR
A
WT1
9
Q
What is denys drash
A
male pseudohermaphroditism, renal masangial scleosis (renal failure), Wilms
10
Q
gene for denys drash
A
WT1
11
Q
what is beckwith Wiedmann
A
macroglossia, hemihypertrophy
12
Q
gene for beckwith wiedman
A
WT2
13
Q
beckith wiedman % risk of wilms
A
4-10%
14
Q
what is classic triphasic histology in wilms
A
(epithelial, blastemal, stromal)
15
Q
difference btw favorable and unfavorable wilms histology
A
favorable has triphasic histology (epithelial, blastemal, stromal). Unfavorable - anaplasia
16
Q
significance of unfavorable wilms histology
A
chemo resistance and 50% death
17
Q
intralobar vs perilobar nephrogenic rests assd with
A
INTRALOBAR - early in development. Assd w/ WAGR, denys drach. PERILOBAR - late in development, beckwith weidman syndrome and hemihypertrophy
18
Q
what is nephroblastomatosis
A
clusters of persistent nephrogenic blastemal cells - histologically identical to wilms tumor
19
Q
significance of nephroblastomatosis
A
high risk of wilms, esp bilateral wilms
20
Q
important part of wilms presentation
A
kids look healthy
21
Q
2 most common complication of surgery for wilms
A
bowel obstruciton and hemorrhage
22
Q
% caval extension in wilms
A
4%
23
Q
long term complication of doxorubicin
A
CHF at 20 yrs in 20%
24
Q
most imp outcomes based on (2)
A
histopathology, tumor stage
25
wilms tumor staging called
NTWS (nitwit's)
26
NTWS wilms stages
stage 1: confined, total resection. Stage 2: outside capsule, total resection. Stage 3: incomplete resecton or biopsy, any spill, + LN. Stage 4: hematogenous spread. Stage 5: bilateral tumors
27
tumor spillage increases recurrence by x?
6x
28
UNILATERAL wilms surgery caveats - 3
1. transperitoneal nx, 2. don’t need to explore contralateral kidney (ct's are better now), 3. selective LN sampling, not RPLND
29
UNILATERAL wilms recurrence RF's - 4
tumor spillage, unfavorable histology, incomplete resection, absence of LN sampling
30
BILATERAL wilms surgery caveats
no open bx, just do upfront chemo for tumor shrinkage and pnx.
31
BILATERAL wilms Chemo mgmt
if response - do pnx. If NO response - bilateral open biopsy. At 2nd look PNX if 2/3 kidney can be preseerved. do NX if unfavorable histology or chemo failure
32
who gets neoadjuvant chemo in wilms - 4
bilateral, vascular invasion, unresectable tumor, solitary kidney
33
only group not getting radiation in NWTS protocol
stage 1 or 2 favorable or unfavorbale histology
34
late chemo effects in wilms tumor (4)
infertility, hypogonadism, 2nd malignancy, CHF (doxorubicin)
35
most common renal tumor of infancy
mesoblastic nephroma
36
mesoblastic nephroma tx
nx is curative
37
clear cell sarcoma - %, location of mets, and mgmt
3% kids renal tumors, **bone mets common**, multimodal tx needed
38
mesoblastic nephroma spec chemo
doxorubicin
39
rhabdoid renal tumors - %, location of mets, features
2% kids renal tumors, ** brain mets common**, very bad
40
why are rhabdoid renal masses so bad
chemo resistant, advanced stage, high mortality
41
kids RCC - when, subtype, incidence
most commin 2nd decade, papillary most common, 5% kids
42
neuroblastoma - incidence
most comm extracranial solid tumor in kids,
43
are mets common in neuroblastoma
yes - 50% with mets
44
where do mets present in neuroblastoma
presents anywhere along symp chain (75% retroperitoneum)
45
neuroblastoma origin
neural crest, like pheo
46
neuroblastoma genetics
n-myc amplification in 20% and poor prognostic marker
47
neuroblastoma inheritance
autosomal dominant
48
neuroblastoma signs
racoon eyes (periorbital mass), sick appearing, anemia (bm mets), blueberry muffin spots on trunk, Opsoclonus-Myoclonus
49
neuroblastoma workup
urine catecholamine (VMA, HVA) in upto 90%, BM bx
50
positive prognostic markers for neuroblastoma - 3
BETTER prognosis: age < 1 (best prognosis), nonadrenal origin, tumor stage.
51
worse prognostic markers for neuroblastoma - 2
WORSE: N-MYC amplification, elevated serum ferritin
52
neuroblastoma staging - 5
1 - localized tumor, 2 - unilteral tumor w gross total resection and neg LN. 2b - stage 2 w/ ipsilateral +LN only. 3 - tumor crosses midline OR + contralat LN. 4 - distant LN + or mets to BM, bone, or liver
53
2 good prognostic signs in neuroblastoma
adrenal tumor origin and those < 1 yo
54
neuroblastoma stage 4s location of mets
mets to liver, skin, or BM, but NOT cortical bone
55
neuroblastoma stage 4s survival and caveat
90% survival, ** may spontaneously regress**
56
low risk neuroblastoma staging
stage 1, 2, or 4s
57
low risk genetics/ histology - neuroblastoma - 3
N-MYC neg, no diploidy, favorable histology
58
low risk tx and survival - neuroblastoma
only surg, 95% survival
59
intermediate risk - staging - neuroblastoma (4)
stage 3 and 4, or 4s if symptomatic AND unfavorable histology
60
intermediate risk - genetics and age - neuroblastoma
N-MYC neg, < 18 mo old
61
intermediate risk - tx - neuroblastoma
surg + chemo
62
intermediate risk - survival- neuroblastoma
90%
63
high risk - staging/ genetics - neuroblastoma
all stages if N-MYC +
64
high risk - tx and survival - neuroblastoma
chemo + surg +/- rad. Survival - 20-40%
65
rhabdomyosarcoma - how common
most comm soft tissue sarcoma < 15 yo, 25% GU tract
66
rhabomyosarcoma origin
mesenchyma
67
RMS gene
2q37 locus
68
RMS - presentation age
bimodal - < 10yo and late adolescence
69
RMS assd w/ what other syndrome?
neurofibromatosis
70
RMS path subtypes - 3 - and significance
embryonal and botryoid (grapes) - younger pts and better prognosis. alveolar
71
RMS - tx optons
surgery is diagnostic. Chemo is mainstay. Radiation is controversial b/c long term effects
72
RMS - tx goal
organ preservation
73
RMS chemo agents - 3
VAC - vincristine, actinomycin D, cyclophosphamide
74
RMS - how to do bx
cold cup
75
RMS presenting in retention
do not place SPT (seeding). Foley until tumor shrinkage w chemo
76
RMS - how to evaluate residual disease
PET scan differentiates fibrosis from tumor. Residual disease is RF for local recurrence.
77
paratesticular RMS - mgmt - 3
all get orchiectomy (as opposed to just bx) then chemo. +/- RPLND
78
paratesticular RMS - who gets RPLND
kids > 10 yo w neg LN on CT should get ipsilateral RPLND prior to chemo. If + LN - chemo +/- rplnd
79
peds testis tumors - distribution
bimodal - < 2 yo and young adulthood
80
most comm testicular tumor in kids
teratoma
81
yolk sac tumor workup
ct CHEST, abdomen and pelvis
82
tumor markers in kids
AFP only, no HCG as no pediatric tumors make this
83
what testicular tumors do kids NOT get - 3
embryonal, choriocarcinoma, seminoma
84
AFP and infants
elevated - normal by 6 months
85
peds tumor with elev AFP
90% yolk sack
86
who gets orchiectomy automatically when testicular mass present in infant
> 6 mo and elev AFP
87
who gets testis sparing
most pre-pubertal.
88
how to do testis sparing
Clamp vessels, frozen section of bx. Remove tumor alone if benign
89
risk of leaving testis - pre vs post pubertal
CIS present in most POST-pubertal, rare in PRE-pubertal (1 case).
90
yolk sac adjuvant tx - stage 1, mets, postchemo mass
stage 1 - observation. Mets/rec - chemo. RPLND if postchemo mass
91
AFP t1/2
5 days
92
AFP caveat in babies
remains elevated for 6-9 months postpartum
93
leydig cell tumor triad
precocious puberty, testis mass, elevated serum testosterone and urinary 17-ketosteroids
94
leydig cell tumor labs
high testosterone, low-nl gonadotropins
95
leydig tumor mgmt
testis sparing sx if possible
96
sertoli cell - symptoms
usu hormonally inactive, but can see gynecomastia
97
sertoli cell - tx
orchiectomy. r/o mets if aggressive histology. Benign tumor
98
large cell calcifying sertoli cell tumors - who gets this? - 2
1/3 syndromic - putz-jeghers, carney's synd
99
large cell calcifying sertoli cell tumors - mgmt
benign - orchiectomy
100
juvenile granulosa cell tumors - age
1st yr of life
101
juvenile granulosa cell tumors - genetics
y chr abnormalities
102
juvenile granulosa cell tumors - mgmt
benign, rare, testis sparing
103
gonadoblastoma - genetics
dysgenetic gonad WITH y-chromosome
104
what is testicle like in pre-gonadoblastoma patients
streak, dysgenetic, indeterminate
105
gonadoblastoma - contents
have germ cell + stromal elements
106
gonadoblastoma - mgmt
10% malig after puberty. Remove early while benign
107
why does dysgerminoma (gonadoblastoma) need to be treated before puberty
germ cell elements outgrow stromal components after puberty --> dysgerminoma develops
108
what happens to gonadoblastoma after puberty
dysgerminoma (seminoma)
109
undescended testis and testis tumors - incidence
4-6x increased risk
110
undescended testis and its position
higher up, the higher the likelyhood of malignancy
111
undescended testis and tumor type
seminoma before ox, NSGCT after ox
112
undescended testis and orchiopexy
orchidopexy BEFORE puberty decreases ca risk
