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4 - peds oncology Flashcards

1
Q

% wilms bilateral?

A

5%

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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

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3
Q

what is WT1 gene

A

deletion of 11p13

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4
Q

what is WT2 gene

A

loss of heterozygosity of 11p15

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5
Q

non WT gene abnormality seen in wilms and sig

A

1p and 16q loss of heterozygosity - inc risk of death/ relapse

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6
Q

names of 3 genetic syndromes assd w wilms

A

WAGR, denys drash, beckwith wiedemann

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7
Q

what is WAGR

A

wilms, aniridia, genital abnormalities, MR

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8
Q

gene for WAGR

A

WT1

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9
Q

What is denys drash

A

male pseudohermaphroditism, renal masangial scleosis (renal failure), Wilms

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10
Q

gene for denys drash

A

WT1

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11
Q

what is beckwith Wiedmann

A

macroglossia, hemihypertrophy

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12
Q

gene for beckwith wiedman

A

WT2

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13
Q

beckith wiedman % risk of wilms

A

4-10%

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14
Q

what is classic triphasic histology in wilms

A

(epithelial, blastemal, stromal)

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15
Q

difference btw favorable and unfavorable wilms histology

A

favorable has triphasic histology (epithelial, blastemal, stromal). Unfavorable - anaplasia

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16
Q

significance of unfavorable wilms histology

A

chemo resistance and 50% death

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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

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18
Q

what is nephroblastomatosis

A

clusters of persistent nephrogenic blastemal cells - histologically identical to wilms tumor

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19
Q

significance of nephroblastomatosis

A

high risk of wilms, esp bilateral wilms

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20
Q

important part of wilms presentation

A

kids look healthy

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21
Q

2 most common complication of surgery for wilms

A

bowel obstruciton and hemorrhage

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22
Q

% caval extension in wilms

A

4%

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23
Q

long term complication of doxorubicin

A

CHF at 20 yrs in 20%

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24
Q

most imp outcomes based on (2)

A

histopathology, tumor stage

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25
Q

wilms tumor staging called

A

NTWS (nitwit’s)

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26
Q

NTWS wilms stages

A

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

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27
Q

tumor spillage increases recurrence by x?

A

6x

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28
Q

UNILATERAL wilms surgery caveats - 3

A
  1. transperitoneal nx, 2. don’t need to explore contralateral kidney (ct’s are better now), 3. selective LN sampling, not RPLND
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29
Q

UNILATERAL wilms recurrence RF’s - 4

A

tumor spillage, unfavorable histology, incomplete resection, absence of LN sampling

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30
Q

BILATERAL wilms surgery caveats

A

no open bx, just do upfront chemo for tumor shrinkage and pnx.

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31
Q

BILATERAL wilms Chemo mgmt

A

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

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32
Q

who gets neoadjuvant chemo in wilms - 4

A

bilateral, vascular invasion, unresectable tumor, solitary kidney

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33
Q

only group not getting radiation in NWTS protocol

A

stage 1 or 2 favorable or unfavorbale histology

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34
Q

late chemo effects in wilms tumor (4)

A

infertility, hypogonadism, 2nd malignancy, CHF (doxorubicin)

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35
Q

most common renal tumor of infancy

A

mesoblastic nephroma

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36
Q

mesoblastic nephroma tx

A

nx is curative

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37
Q

clear cell sarcoma - %, location of mets, and mgmt

A

3% kids renal tumors, bone mets common, multimodal tx needed

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38
Q

mesoblastic nephroma spec chemo

A

doxorubicin

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39
Q

rhabdoid renal tumors - %, location of mets, features

A

2% kids renal tumors, ** brain mets common**, very bad

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40
Q

why are rhabdoid renal masses so bad

A

chemo resistant, advanced stage, high mortality

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41
Q

kids RCC - when, subtype, incidence

A

most commin 2nd decade, papillary most common, 5% kids

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42
Q

neuroblastoma - incidence

A

most comm extracranial solid tumor in kids,

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43
Q

are mets common in neuroblastoma

A

yes - 50% with mets

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44
Q

where do mets present in neuroblastoma

A

presents anywhere along symp chain (75% retroperitoneum)

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45
Q

neuroblastoma origin

A

neural crest, like pheo

46
Q

neuroblastoma genetics

A

n-myc amplification in 20% and poor prognostic marker

47
Q

neuroblastoma inheritance

A

autosomal dominant

48
Q

neuroblastoma signs

A

racoon eyes (periorbital mass), sick appearing, anemia (bm mets), blueberry muffin spots on trunk, Opsoclonus-Myoclonus

49
Q

neuroblastoma workup

A

urine catecholamine (VMA, HVA) in upto 90%, BM bx

50
Q

positive prognostic markers for neuroblastoma - 3

A

BETTER prognosis: age < 1 (best prognosis), nonadrenal origin, tumor stage.

51
Q

worse prognostic markers for neuroblastoma - 2

A

WORSE: N-MYC amplification, elevated serum ferritin

52
Q

neuroblastoma staging - 5

A

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
Q

2 good prognostic signs in neuroblastoma

A

adrenal tumor origin and those < 1 yo

54
Q

neuroblastoma stage 4s location of mets

A

mets to liver, skin, or BM, but NOT cortical bone

55
Q

neuroblastoma stage 4s survival and caveat

A

90% survival, ** may spontaneously regress**

56
Q

low risk neuroblastoma staging

A

stage 1, 2, or 4s

57
Q

low risk genetics/ histology - neuroblastoma - 3

A

N-MYC neg, no diploidy, favorable histology

58
Q

low risk tx and survival - neuroblastoma

A

only surg, 95% survival

59
Q

intermediate risk - staging - neuroblastoma (4)

A

stage 3 and 4, or 4s if symptomatic AND unfavorable histology

60
Q

intermediate risk - genetics and age - neuroblastoma

A

N-MYC neg, < 18 mo old

61
Q

intermediate risk - tx - neuroblastoma

A

surg + chemo

62
Q

intermediate risk - survival- neuroblastoma

63
Q

high risk - staging/ genetics - neuroblastoma

A

all stages if N-MYC +

64
Q

high risk - tx and survival - neuroblastoma

A

chemo + surg +/- rad. Survival - 20-40%

65
Q

rhabdomyosarcoma - how common

A

most comm soft tissue sarcoma < 15 yo, 25% GU tract

66
Q

rhabomyosarcoma origin

A

mesenchyma

67
Q

RMS gene

A

2q37 locus

68
Q

RMS - presentation age

A

bimodal - < 10yo and late adolescence

69
Q

RMS assd w/ what other syndrome?

A

neurofibromatosis

70
Q

RMS path subtypes - 3 - and significance

A

embryonal and botryoid (grapes) - younger pts and better prognosis. alveolar

71
Q

RMS - tx optons

A

surgery is diagnostic. Chemo is mainstay. Radiation is controversial b/c long term effects

72
Q

RMS - tx goal

A

organ preservation

73
Q

RMS chemo agents - 3

A

VAC - vincristine, actinomycin D, cyclophosphamide

74
Q

RMS - how to do bx

75
Q

RMS presenting in retention

A

do not place SPT (seeding). Foley until tumor shrinkage w chemo

76
Q

RMS - how to evaluate residual disease

A

PET scan differentiates fibrosis from tumor. Residual disease is RF for local recurrence.

77
Q

paratesticular RMS - mgmt - 3

A

all get orchiectomy (as opposed to just bx) then chemo. +/- RPLND

78
Q

paratesticular RMS - who gets RPLND

A

kids > 10 yo w neg LN on CT should get ipsilateral RPLND prior to chemo. If + LN - chemo +/- rplnd

79
Q

peds testis tumors - distribution

A

bimodal - < 2 yo and young adulthood

80
Q

most comm testicular tumor in kids

81
Q

yolk sac tumor workup

A

ct CHEST, abdomen and pelvis

82
Q

tumor markers in kids

A

AFP only, no HCG as no pediatric tumors make this

83
Q

what testicular tumors do kids NOT get - 3

A

embryonal, choriocarcinoma, seminoma

84
Q

AFP and infants

A

elevated - normal by 6 months

85
Q

peds tumor with elev AFP

A

90% yolk sack

86
Q

who gets orchiectomy automatically when testicular mass present in infant

A

> 6 mo and elev AFP

87
Q

who gets testis sparing

A

most pre-pubertal.

88
Q

how to do testis sparing

A

Clamp vessels, frozen section of bx. Remove tumor alone if benign

89
Q

risk of leaving testis - pre vs post pubertal

A

CIS present in most POST-pubertal, rare in PRE-pubertal (1 case).

90
Q

yolk sac adjuvant tx - stage 1, mets, postchemo mass

A

stage 1 - observation. Mets/rec - chemo. RPLND if postchemo mass

91
Q

AFP t1/2

92
Q

AFP caveat in babies

A

remains elevated for 6-9 months postpartum

93
Q

leydig cell tumor triad

A

precocious puberty, testis mass, elevated serum testosterone and urinary 17-ketosteroids

94
Q

leydig cell tumor labs

A

high testosterone, low-nl gonadotropins

95
Q

leydig tumor mgmt

A

testis sparing sx if possible

96
Q

sertoli cell - symptoms

A

usu hormonally inactive, but can see gynecomastia

97
Q

sertoli cell - tx

A

orchiectomy. r/o mets if aggressive histology. Benign tumor

98
Q

large cell calcifying sertoli cell tumors - who gets this? - 2

A

1/3 syndromic - putz-jeghers, carney’s synd

99
Q

large cell calcifying sertoli cell tumors - mgmt

A

benign - orchiectomy

100
Q

juvenile granulosa cell tumors - age

A

1st yr of life

101
Q

juvenile granulosa cell tumors - genetics

A

y chr abnormalities

102
Q

juvenile granulosa cell tumors - mgmt

A

benign, rare, testis sparing

103
Q

gonadoblastoma - genetics

A

dysgenetic gonad WITH y-chromosome

104
Q

what is testicle like in pre-gonadoblastoma patients

A

streak, dysgenetic, indeterminate

105
Q

gonadoblastoma - contents

A

have germ cell + stromal elements

106
Q

gonadoblastoma - mgmt

A

10% malig after puberty. Remove early while benign

107
Q

why does dysgerminoma (gonadoblastoma) need to be treated before puberty

A

germ cell elements outgrow stromal components after puberty –> dysgerminoma develops

108
Q

what happens to gonadoblastoma after puberty

A

dysgerminoma (seminoma)

109
Q

undescended testis and testis tumors - incidence

A

4-6x increased risk

110
Q

undescended testis and its position

A

higher up, the higher the likelyhood of malignancy

111
Q

undescended testis and tumor type

A

seminoma before ox, NSGCT after ox

112
Q

undescended testis and orchiopexy

A

orchidopexy BEFORE puberty decreases ca risk

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