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PPS - From Nelson's > Onco > Flashcards

Onco Flashcards

1
Q

most common tumors first year of life

A 
neuroblastoma
nephroblastoma
retinoblastoma
rhabdomyosarcoma
hepatoblastoma
medulloblastoma
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2
Q

most common tumor in 2-5yrs old

A

embryonal tumors
leukemia
non hodgkin lymphoma
glioma

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3
Q

ALL risk factor

A

ionizing radiation
Down syndrome is associated with an estimated 10-20–fold increased risk
NF1, Bloom syndrome, ataxia-telangiectasia, and Langerhans cell histiocytosis

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4
Q

AML risk factor

A

Down syndrome and NF1

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5
Q

optic glioma risk factor

A

NF1

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6
Q

Hodgkin disease risk factor

A

ebv

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7
Q

burkitt lymphoma risk factor

A

ebv in africa

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8
Q

osteosarcoma risk factor

A

Li-Fraumeni syndrome and hereditary retinoblastoma

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9
Q

wilms tumor risk factor

A

Aniridia (WAGR)

Beckwith-Wiedemann syndrome

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10
Q

rhabdomyosarcoma

A

Li-Fraumeni syndrome and NF1

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11
Q

hepatoblastoma risk factor

A

Beckwith-Wiedemann syndrome, hemihypertrophy, Gardner syndrome, and family
history of adenomatous polyposis

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12
Q

leiomyosarcoma risk factor

A

EBV

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13
Q

testicular germ cell tumors risk factor

A

Cryptorchidism

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14
Q

WAGR

A

Wilms tumour (a tumour of the kidneys)
Aniridia (absence of the coloured part of the eye, the iris), Genitourinary anomalies
mental Retardation

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15
Q

mutation in the adenomatous polyposis coli (APC gene)
autosomal dominant
multiple polyps in the colon together with tumors outside the colon

The extracolonic tumors may include osteomas of the skull, thyroid cancer, epidermoid cysts, fibromas

A

Gardner’s syndrome or familial colorectal polyposis

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16
Q

autosomal dominant
numerous adenomatous polyps form mainly in the epithelium of the large intestine
start out benign, malignant transformation into colon cancer occurs when they are left untreated

A

Familial adenomatous polyposis FAP

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17
Q

blueberry muffin rash

A

neuroblastoma

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18
Q

Chromosome 11p deletion syndrome

with sporadic aniridia

A

Wilms tumor

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19
Q

Chromosome 13q deletion syndrome

A

Retinoblastoma, sarcoma

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20
Q

Trisomy 21

A

Lymphocytic or nonlymphocytic leukemia,
especially megakaryocytic leukemia;
transient leukemoid reaction

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21
Q

Klinefelter syndrome (47,XXY)

A

Breast cancer, extragonadal germ cell tumors

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22
Q

Noonan syndrome

A

jmml

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23
Q

Xeroderma pigmentosum

A

Basal cell and squamous cell carcinomas;

melanoma

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24
Q

Fanconi anemia

A

Leukemia, myelodysplastic syndrome, liver
neoplasias, rare head and neck tumors, GI
and GU cancers

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25
Q

Bloom syndrome

A

Leukemia, lymphoma, and solid tumors

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26
Q

Ataxia-telangiectasia

A

Lymphoma, leukemia, less commonly central

nervous system and nonneural solid tumors

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27
Q

Dysplastic nevus syndrome

A

melanoma

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28
Q

Wiskott-Aldrich syndrome

A

Lymphoma, leukemia

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29
Q

X-linked immunodeficiency (Duncan

syndrome

A

Lymphoproliferative disorder

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30
Q

X-linked agammaglobulinemia (Bruton

disease)

A

Lymphoma, leukemia

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31
Q

Severe combined immunodeficiency

A

Leukemia, lymphoma

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32
Q

Neurofibromatosis 1

A

Neurofibroma, optic glioma, acoustic
neuroma, astrocytoma, meningioma,
pheochromocytoma, sarcoma

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33
Q

Neurofibromatosis 2

A

Bilateral acoustic neuromas, meningiomas

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34
Q

Tuberous sclerosis

A

Fibroangiomatous nevi, myocardial

rhabdomyoma

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35
Q

Li-Fraumeni syndrome p53 gene

A

Bone, soft tissue sarcoma, breast

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36
Q

Retinoblastoma also prone to

A

Sarcoma

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37
Q

Hemihypertrophy ± Beckwith syndrome

A

Wilms tumor, hepatoblastoma, adrenal

carcinoma

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38
Q

von Hippel-Landau disease

A

Hemangioblastoma of the cerebellum and

retina, pheochromocytoma, renal cancer

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39
Q 
Multiple endocrine neoplasia syndrome,
type 1 (Wermer syndrome)
A

Parathyroid, pancreatic islet, and pituitary

tumors

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40
Q 
Multiple endocrine neoplasia syndrome,
type 2A (Sipple syndrome)
A

Medullary carcinoma of the thyroid,

hyperparathyroidism, pheochromocytoma

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41
Q

Familial adenomatous polyposis

A

Colorectal, thyroid carcinoma, duodenal and
periampullar carcinomas; pediatric
hepatoblastoma

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42
Q

Familial juvenile polyposis

A

Colorectal carcinoma

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43
Q

Hereditary nonpolyposis colon cancer

Lynch syndrome, NHPCC

A

Colon cancer

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44
Q

Gardner syndrome

A

Adenocarcinoma of colon, skull and soft

tissue tumors

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45
Q

Peutz-Jeghers syndrome

A

Gastrointestinal carcinoma, ovarian neoplasia

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46
Q

Hemochromatosis

A

Hepatocellular carcinoma

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47
Q

Glycogen storage disease 1 (von Gierke

disease)

A

Hepatocellular carcinoma

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48
Q

Tyrosinemia, galactosemia

A

Hepatocellular carcinoma

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49
Q

Diamond-Blackfan anemia

A

AML, myelodysplastic syndrome, osteogenic

sarcoma

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50
Q

Shwachman-Diamond syndrome

A

AML, myelodysplasia

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51
Q

Congenital or cyclic neutropenia

A

Myelodysplastic syndrome

AML

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52
Q

cancer with diarrhea

A

neuroblastoma
ganglioneuroma

release of vasoactive intestinal peptide

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53
Q

if ALL develops in first twin during first year of life, risk for second twin for ALL is

A

> 70%

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54
Q

if ALL develops in first twin during 5-7yrs old, risk for second twin for ALL is

A

2x general population

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55
Q

ALL diagnosis BMA

A

> 25% lymphoblasts

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56
Q

traumatic initial lumbar tap for ALL assoc with

A

increased risk of CNS relapse

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57
Q

important predictive factors in ALL tx

A
  1. age of presentation (>1yr, <10yr)
  2. initial leukocyte count (50, 000)
  3. speed of response to therapy
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58
Q

chromosome abnormality with high risk of relapse

A

t (9,22)

t (4,11)

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59
Q

remission definition

A

<5% blasts in marrow

return of neutrophil and plt to normal after 4-5weeks

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60
Q

effect rapid metabolizer mercaptopurine

A

toxicity

61
Q

effect slow metabolizer mercaptopurine

A

treatment failure

62
Q

diagnosis aml on bma

A

> 20% blast

63
Q

FAB M3

A

acute promyeloblastic leukemia
t 15;17
all trans retinoic acid

64
Q

imatinib is a

A

tyrosine kinase

65
Q 
elevated leukocytes with increased monocytes
thrombocytopenia
anemia with erythroblasts
BMA myelodysplastic with <30% blasts
<2yrs old
noonan or neurofibromatosis 1
A

jmml

juvenile myelomonocytic leukemia

66
Q

translocation in infantile leukemia

A

t 4;11

67
Q

reed sternberg

A

hodgkin lymphoma

68
Q

b symptoms

A

fever 39C
weight loss >10% in 3mo
night sweats

69
Q

poor prognostic factors hodgkins lymphoma

A

tumor bulk
b symptoms
stage

70
Q

oncologic emergency assoc with NHL

A
  1. superior mediastinal syndrome
  2. spinal cord compression
  3. tumor lysis
71
Q

tx nhl

A

cyclophosphamide
vincristine
prednisone
doxorubicin

72
Q

tx HL

A

cyclophosphamide
vincristine
procarbazine
prednisone

73
Q

second most common malignancy in childhood

A

brain tumors

74
Q

third most common malignancy in childhood

A

lymphoma

75
Q

malignancy with highest morbidity

A

brain tumors

76
Q

most common brain tumor 0-14yr old

A

pilocytic astrocytoma
and
medulloblastoma/primitive neuroectodermal tumor

77
Q

brain tumor prevalence over all

supratentorial __ infratentorial

A

<

infrantentorial
43.2%

78
Q

common brain tumor

<1yr

A

supratentorial

choroid plexus
teratomas

79
Q

common brain tumor

1-10yr

A

infratentorial

pilocytic astrocytoma
medulloblastoma

80
Q

common brain tumor

>10yr

A

supratentorial

diffuse astrocytoma

81
Q

headache
nausea
vomiting
papilledema

A

midline brain tumor

infratentorial

82
Q

equilibrium problem
gait
coordination

A

infratentorial brain tumor

83
Q

blurred vision
diplopia
nystagmus

A

infratentorial brain tumor

84
Q

gaze palsy
multiple cranial nerve palsies
upper motor neuron deficit

A

brainstem tumor

85
Q 
motor weakness
sensory change
speech change
seizure
reflex change
A

supratentorial tumor

86
Q

neuroendocrine defect

A

suprasellar tumor

87
Q

paresis of upward gaze
pupillary dilatation reactive to accomodation but not to light
nystagmus to convergence/retraction
eyelid retraction

A

parinaud syndrome

pineal gland tumor

88
Q

preferential site for germ cell tumor

A

suprasellar and pineal gland

89
Q

tumor with propensity to spread to csf

A

medulloblastoma
PNET
ependymomma
germ cell tumor

90
Q

pilocytic astrocytoma usually found at

A

cerebellum

91
Q

ct scan finding pilocytic astrocytoma

A

contrast enhancing nodule within the wall of a cystic mass

92
Q

rosenthal fibers

A

pilocytic astrocytoma

93
Q

microscopic finding of biphasic appearance of bundles of compact fibrillari tissue interspersed with loose spongy area

A

pilocytic astrocytoma

94
Q

rosenthal fiber

A

condensed mass of glial elements in compact areas

95
Q

mri of fibrillary low grade astrocytoma

A

lack of enhancement after contrast

96
Q

Histologic characteristics
include perivascular pseudorosettes, ependymal rosettes, monomorphic
nuclear morphology, and occasional nonpalisading foci of necrosis

A

ependymomma

WHO grade II

97
Q

poor prognostic factor for ependymomma

A

age (younger -> worse)

location (posterior fossa -> worse)

98
Q

choroid plexus carcinoma associ with

A

li fraumeni

99
Q

most common location of medulloblastoma

A

cerebellar vermis

100
Q

ct or mri of medulloblastoma

A

solid, homogeneous,
contrast medium–enhancing mass in the posterior fossa causing
fourth ventricular obstruction and hydrocephalus

101
Q

homer wright rosettes

small blue tumor

A

medulloblastoma

102
Q

radiation medulloblastoma

A

24gy on craniospinal

50 to 55gy on tumor bed

103
Q

solid and cystic tumor in suprasellar

A

craniopharyngioma

104
Q

small round blue cell tumors

A

neuroblastoma
rhabdomyosarcoma
ewing sarcoma
non hodgkin lymphoma

105
Q

neuroblastoma can develop anywhere in the ___

A

sympathetic NS

106
Q

most common site of mets neuroblastoma

A 
lymph node
bone marrow
long bones
liver
skin
107
Q

ct scan of neuroblastoma

A

multiple masses with calcification and hemorrhage

108
Q

test for neuroblastoma

A

HVA homovanillic acid

VMA vanillmandelic acid

109
Q

second most common malignant ab tumor in kids

A

wilms

110
Q

kids with horshoe kids have __ the risk of wilms

A

twice

111
Q

prognostic factors Wilms

A

age
stage
tumor weight
loss of heterozygosity of chromosome 1p, 16q

112
Q

wilms
<2yr
<550grams tumor

A

low risk

nephrectomy only

113
Q

renal cell carcinoma assoc with

A

con hippel lindau

114
Q

most common soft tissue sarcoma

A

rhabdomyosarcoma

115
Q

most common extracranial solid tumor

A

neuroblastoma

116
Q

vaginal rhabdomysarcoma

A

sarcoma botyroides

117
Q 
short stature
skin telangiectasia
small hand and feet
absent thumbs
osteosarcoma
A

rothmund thomsun syndrome

118
Q

xray osteosarcoma

A

sunburst

119
Q

differentials lytic bone lesion

A 
osteosarcoma
histiocytosis
ewings
lymphoma
bone cyst
120
Q

xray ewings

A

periosteal elevation

onion skinning

121
Q

ewing tumor chest wall

A

askin tumor

122
Q

most common benign bone tumor

A

osteochondroma

123
Q

bonde dysplasi
mlutifocal benign lesion of hyaline cartilage
short stature
limb length discrepancy

A

ollier disease

124
Q

xray osteochondroma

A

stalk or broad based projection

125
Q

xray chondromyxoid fibroma

A

eccentric, lobular, metaphyseal radiolucency with sharp sclerotic scalloped margins

126
Q

xray osteoid osteoma

A

round lucency surrounded with sclerotic bone

127
Q

xray unicameral bone cyst

A

centrally located lesion in medullary of bone

128
Q

xray aneurysmal bone cyst

A

lytic lesion with metaphyseal expansion surrounded by thin sclerotic rim

129
Q

flexner wintersteiner rosettes

A

retinoblastoma

130
Q

elevated B HCG

A

choriocarcinoma

germinoma

131
Q

elevated AFP

A

endodermal sinus tumor or yolk sac tumor

132
Q

sertoli

A

estrogen

133
Q

leydig

A

androgen

134
Q

mets of hepatoblastoma

A

lymph node

lungs

135
Q

tumor marker hepatoblastoma

A

afp

136
Q

tennis shaped bilamellar granula on electron microscope

dx?

A

birbeck granule

langerhans cell histiocytosis

137
Q

Neurofibromatosis type 1 (autosomal dominant)

A

Optic pathway gliomas, astrocytoma, malignant peripheral

nerve sheath tumors, neurofibromas

138
Q

Neurofibromatosis type 2 (autosomal dominant)

A

Vestibular schwannomas, meningiomas, spinal cord

ependymoma, spinal cord astrocytoma, hamartomas

139
Q

von Hippel–Lindau (autosomal dominant)

A

Hemangioblastoma

140
Q

Tuberous sclerosis (autosomal dominant)

A

Subependymal giant cell astrocytoma, cortical tubers

141
Q

Li-Fraumeni (autosomal dominant)

A

Astrocytoma, primitive neuroectodermal tumor

142
Q

Massive involvement of the liver with metastatic disease with or without respiratory distress.

A

Pepper syndrome

143
Q

Unilateral ptosis, myosis, and anhidrosis associated with a thoracic or cervical primary tumor. Symptoms do not
resolve with tumor resection.

A

Horner syndrome

144
Q

Limping and irritability in young child associated with bone and bone marrow metastases

A

Hutchinson syndrome

145
Q

Myoclonic jerking and random conjugate eye movements with or without cerebellar ataxia. Often associated
with a biologically favorable and differentiated tumor. The condition is likely immune mediated, may not
resolve with tumor removal, and often exhibits progressive neuropsychologic sequelae.

A

Opsoclonus-myoclonus-ataxia

syndrome

146
Q

Intractable secretory diarrhea due to tumor secretion of vasointestinal peptides. Tumors are generally
biologically favorable.

A

Kerner-Morrison syndrome

147
Q

Neuroblastoma associated with other neural crest disorders, including congenital hypoventilation syndrome or
Hirschsprung disease. Germline mutations in the paired homeobox gene PHOX2B have been identified in a
subset of patients with this disease.

A

Neurocristopathy syndrome

148
Q

diagnosis for HLH

Hemophagocytic Lymphohistiocytosis

A

one of the following two criteria:

  1. A molecular diagnosis consistent with HLH (e.g., PRF mutations,
    SAP mutations)

or

  1. Having 5 of the following 8 signs or symptoms:
    a. Fever
    b. Splenomegaly
    c. Cytopenia (affecting ≥2 cell lineages; hemoglobin ≤9 g/dL [or
    ≤10 g/dL for infants <4 wk of age], platelets <100,000/μL,
    neutrophils <1,000/μL)
    d. Hypertriglyceridemia (≥265 mg/dL) and/or
    hypofibrinogenemia (≤150 mg/dL)
    e. Hemophagocytosis in the bone marrow, spleen, or lymph
    nodes without evidence of malignancy
    f. Low or absent natural killer cell cytotoxicity
    g. Hyperferritinemia (≥500 ng/mL)
    h. Elevated soluble CD25 (interleukin-2Rα chain; ≥2,400 U/mL)

PPS - From Nelson's (24 decks)

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