Onco Flashcards
1
Q
most common tumors first year of life
A
neuroblastoma nephroblastoma retinoblastoma rhabdomyosarcoma hepatoblastoma medulloblastoma
2
Q
most common tumor in 2-5yrs old
A
embryonal tumors
leukemia
non hodgkin lymphoma
glioma
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
4
Q
AML risk factor
A
Down syndrome and NF1
5
Q
optic glioma risk factor
A
NF1
6
Q
Hodgkin disease risk factor
A
ebv
7
Q
burkitt lymphoma risk factor
A
ebv in africa
8
Q
osteosarcoma risk factor
A
Li-Fraumeni syndrome and hereditary retinoblastoma
9
Q
wilms tumor risk factor
A
Aniridia (WAGR)
Beckwith-Wiedemann syndrome
10
Q
rhabdomyosarcoma
A
Li-Fraumeni syndrome and NF1
11
Q
hepatoblastoma risk factor
A
Beckwith-Wiedemann syndrome, hemihypertrophy, Gardner syndrome, and family
history of adenomatous polyposis
12
Q
leiomyosarcoma risk factor
A
EBV
13
Q
testicular germ cell tumors risk factor
A
Cryptorchidism
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
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
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
17
Q
blueberry muffin rash
A
neuroblastoma
18
Q
Chromosome 11p deletion syndrome
with sporadic aniridia
A
Wilms tumor
19
Q
Chromosome 13q deletion syndrome
A
Retinoblastoma, sarcoma
20
Q
Trisomy 21
A
Lymphocytic or nonlymphocytic leukemia,
especially megakaryocytic leukemia;
transient leukemoid reaction
21
Q
Klinefelter syndrome (47,XXY)
A
Breast cancer, extragonadal germ cell tumors
22
Q
Noonan syndrome
A
jmml
23
Q
Xeroderma pigmentosum
A
Basal cell and squamous cell carcinomas;
melanoma
24
Q
Fanconi anemia
A
Leukemia, myelodysplastic syndrome, liver
neoplasias, rare head and neck tumors, GI
and GU cancers
25
Bloom syndrome
Leukemia, lymphoma, and solid tumors
26
Ataxia-telangiectasia
Lymphoma, leukemia, less commonly central
| nervous system and nonneural solid tumors
27
Dysplastic nevus syndrome
melanoma
28
Wiskott-Aldrich syndrome
Lymphoma, leukemia
29
X-linked immunodeficiency (Duncan
| syndrome
Lymphoproliferative disorder
30
X-linked agammaglobulinemia (Bruton
| disease)
Lymphoma, leukemia
31
Severe combined immunodeficiency
Leukemia, lymphoma
32
Neurofibromatosis 1
Neurofibroma, optic glioma, acoustic
neuroma, astrocytoma, meningioma,
pheochromocytoma, sarcoma
33
Neurofibromatosis 2
Bilateral acoustic neuromas, meningiomas
34
Tuberous sclerosis
Fibroangiomatous nevi, myocardial
| rhabdomyoma
35
Li-Fraumeni syndrome p53 gene
Bone, soft tissue sarcoma, breast
36
Retinoblastoma also prone to
Sarcoma
37
Hemihypertrophy ± Beckwith syndrome
Wilms tumor, hepatoblastoma, adrenal
| carcinoma
38
von Hippel-Landau disease
Hemangioblastoma of the cerebellum and
| retina, pheochromocytoma, renal cancer
39
```
Multiple endocrine neoplasia syndrome,
type 1 (Wermer syndrome)
```
Parathyroid, pancreatic islet, and pituitary
| tumors
40
```
Multiple endocrine neoplasia syndrome,
type 2A (Sipple syndrome)
```
Medullary carcinoma of the thyroid,
| hyperparathyroidism, pheochromocytoma
41
Familial adenomatous polyposis
Colorectal, thyroid carcinoma, duodenal and
periampullar carcinomas; pediatric
hepatoblastoma
42
Familial juvenile polyposis
Colorectal carcinoma
43
Hereditary nonpolyposis colon cancer
| Lynch syndrome, NHPCC
Colon cancer
44
Gardner syndrome
Adenocarcinoma of colon, skull and soft
| tissue tumors
45
Peutz-Jeghers syndrome
Gastrointestinal carcinoma, ovarian neoplasia
46
Hemochromatosis
Hepatocellular carcinoma
47
Glycogen storage disease 1 (von Gierke
| disease)
Hepatocellular carcinoma
48
Tyrosinemia, galactosemia
Hepatocellular carcinoma
49
Diamond-Blackfan anemia
AML, myelodysplastic syndrome, osteogenic
| sarcoma
50
Shwachman-Diamond syndrome
AML, myelodysplasia
51
Congenital or cyclic neutropenia
Myelodysplastic syndrome
| AML
52
cancer with diarrhea
neuroblastoma
ganglioneuroma
release of vasoactive intestinal peptide
53
if ALL develops in first twin during first year of life, risk for second twin for ALL is
>70%
54
if ALL develops in first twin during 5-7yrs old, risk for second twin for ALL is
2x general population
55
ALL diagnosis BMA
>25% lymphoblasts
56
traumatic initial lumbar tap for ALL assoc with
increased risk of CNS relapse
57
important predictive factors in ALL tx
1. age of presentation (>1yr, <10yr)
2. initial leukocyte count (50, 000)
3. speed of response to therapy
58
chromosome abnormality with high risk of relapse
t (9,22)
| t (4,11)
59
remission definition
<5% blasts in marrow
| return of neutrophil and plt to normal after 4-5weeks
60
effect rapid metabolizer mercaptopurine
toxicity
61
effect slow metabolizer mercaptopurine
treatment failure
62
diagnosis aml on bma
>20% blast
63
FAB M3
acute promyeloblastic leukemia
t 15;17
all trans retinoic acid
64
imatinib is a
tyrosine kinase
65
```
elevated leukocytes with increased monocytes
thrombocytopenia
anemia with erythroblasts
BMA myelodysplastic with <30% blasts
<2yrs old
noonan or neurofibromatosis 1
```
jmml
| juvenile myelomonocytic leukemia
66
translocation in infantile leukemia
t 4;11
67
reed sternberg
hodgkin lymphoma
68
b symptoms
fever 39C
weight loss >10% in 3mo
night sweats
69
poor prognostic factors hodgkins lymphoma
tumor bulk
b symptoms
stage
70
oncologic emergency assoc with NHL
1. superior mediastinal syndrome
2. spinal cord compression
3. tumor lysis
71
tx nhl
cyclophosphamide
vincristine
prednisone
doxorubicin
72
tx HL
cyclophosphamide
vincristine
procarbazine
prednisone
73
second most common malignancy in childhood
brain tumors
74
third most common malignancy in childhood
lymphoma
75
malignancy with highest morbidity
brain tumors
76
most common brain tumor 0-14yr old
pilocytic astrocytoma
and
medulloblastoma/primitive neuroectodermal tumor
77
brain tumor prevalence over all
| supratentorial __ infratentorial
<
infrantentorial
43.2%
78
common brain tumor
| <1yr
supratentorial
choroid plexus
teratomas
79
common brain tumor
| 1-10yr
infratentorial
pilocytic astrocytoma
medulloblastoma
80
common brain tumor
| >10yr
supratentorial
diffuse astrocytoma
81
headache
nausea
vomiting
papilledema
midline brain tumor
| infratentorial
82
equilibrium problem
gait
coordination
infratentorial brain tumor
83
blurred vision
diplopia
nystagmus
infratentorial brain tumor
84
gaze palsy
multiple cranial nerve palsies
upper motor neuron deficit
brainstem tumor
85
```
motor weakness
sensory change
speech change
seizure
reflex change
```
supratentorial tumor
86
neuroendocrine defect
suprasellar tumor
87
paresis of upward gaze
pupillary dilatation reactive to accomodation but not to light
nystagmus to convergence/retraction
eyelid retraction
parinaud syndrome
| pineal gland tumor
88
preferential site for germ cell tumor
suprasellar and pineal gland
89
tumor with propensity to spread to csf
medulloblastoma
PNET
ependymomma
germ cell tumor
90
pilocytic astrocytoma usually found at
cerebellum
91
ct scan finding pilocytic astrocytoma
contrast enhancing nodule within the wall of a cystic mass
92
rosenthal fibers
pilocytic astrocytoma
93
microscopic finding of biphasic appearance of bundles of compact fibrillari tissue interspersed with loose spongy area
pilocytic astrocytoma
94
rosenthal fiber
condensed mass of glial elements in compact areas
95
mri of fibrillary low grade astrocytoma
lack of enhancement after contrast
96
Histologic characteristics
include perivascular pseudorosettes, ependymal rosettes, monomorphic
nuclear morphology, and occasional nonpalisading foci of necrosis
ependymomma
| WHO grade II
97
poor prognostic factor for ependymomma
age (younger -> worse)
| location (posterior fossa -> worse)
98
choroid plexus carcinoma associ with
li fraumeni
99
most common location of medulloblastoma
cerebellar vermis
100
ct or mri of medulloblastoma
solid, homogeneous,
contrast medium–enhancing mass in the posterior fossa causing
fourth ventricular obstruction and hydrocephalus
101
homer wright rosettes
| small blue tumor
medulloblastoma
102
radiation medulloblastoma
24gy on craniospinal
| 50 to 55gy on tumor bed
103
solid and cystic tumor in suprasellar
craniopharyngioma
104
small round blue cell tumors
neuroblastoma
rhabdomyosarcoma
ewing sarcoma
non hodgkin lymphoma
105
neuroblastoma can develop anywhere in the ___
sympathetic NS
106
most common site of mets neuroblastoma
```
lymph node
bone marrow
long bones
liver
skin
```
107
ct scan of neuroblastoma
multiple masses with calcification and hemorrhage
108
test for neuroblastoma
HVA homovanillic acid
| VMA vanillmandelic acid
109
second most common malignant ab tumor in kids
wilms
110
kids with horshoe kids have __ the risk of wilms
twice
111
prognostic factors Wilms
age
stage
tumor weight
loss of heterozygosity of chromosome 1p, 16q
112
wilms
<2yr
<550grams tumor
low risk
| nephrectomy only
113
renal cell carcinoma assoc with
con hippel lindau
114
most common soft tissue sarcoma
rhabdomyosarcoma
115
most common extracranial solid tumor
neuroblastoma
116
vaginal rhabdomysarcoma
sarcoma botyroides
117
```
short stature
skin telangiectasia
small hand and feet
absent thumbs
osteosarcoma
```
rothmund thomsun syndrome
118
xray osteosarcoma
sunburst
119
differentials lytic bone lesion
```
osteosarcoma
histiocytosis
ewings
lymphoma
bone cyst
```
120
xray ewings
periosteal elevation
| onion skinning
121
ewing tumor chest wall
askin tumor
122
most common benign bone tumor
osteochondroma
123
bonde dysplasi
mlutifocal benign lesion of hyaline cartilage
short stature
limb length discrepancy
ollier disease
124
xray osteochondroma
stalk or broad based projection
125
xray chondromyxoid fibroma
eccentric, lobular, metaphyseal radiolucency with sharp sclerotic scalloped margins
126
xray osteoid osteoma
round lucency surrounded with sclerotic bone
127
xray unicameral bone cyst
centrally located lesion in medullary of bone
128
xray aneurysmal bone cyst
lytic lesion with metaphyseal expansion surrounded by thin sclerotic rim
129
flexner wintersteiner rosettes
retinoblastoma
130
elevated B HCG
choriocarcinoma
| germinoma
131
elevated AFP
endodermal sinus tumor or yolk sac tumor
132
sertoli
estrogen
133
leydig
androgen
134
mets of hepatoblastoma
lymph node
| lungs
135
tumor marker hepatoblastoma
afp
136
tennis shaped bilamellar granula on electron microscope
| dx?
birbeck granule
| langerhans cell histiocytosis
137
Neurofibromatosis type 1 (autosomal dominant)
Optic pathway gliomas, astrocytoma, malignant peripheral
| nerve sheath tumors, neurofibromas
138
Neurofibromatosis type 2 (autosomal dominant)
Vestibular schwannomas, meningiomas, spinal cord
| ependymoma, spinal cord astrocytoma, hamartomas
139
von Hippel–Lindau (autosomal dominant)
Hemangioblastoma
140
Tuberous sclerosis (autosomal dominant)
Subependymal giant cell astrocytoma, cortical tubers
141
Li-Fraumeni (autosomal dominant)
Astrocytoma, primitive neuroectodermal tumor
142
Massive involvement of the liver with metastatic disease with or without respiratory distress.
Pepper syndrome
143
Unilateral ptosis, myosis, and anhidrosis associated with a thoracic or cervical primary tumor. Symptoms do not
resolve with tumor resection.
Horner syndrome
144
Limping and irritability in young child associated with bone and bone marrow metastases
Hutchinson syndrome
145
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.
Opsoclonus-myoclonus-ataxia
| syndrome
146
Intractable secretory diarrhea due to tumor secretion of vasointestinal peptides. Tumors are generally
biologically favorable.
Kerner-Morrison syndrome
147
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.
Neurocristopathy syndrome
148
diagnosis for HLH
| Hemophagocytic Lymphohistiocytosis
one of the following two criteria:
1. A molecular diagnosis consistent with HLH (e.g., PRF mutations,
SAP mutations)
or
2. 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)
