Oncology Flashcards
1
Q
what are the two classes of acute leukemia
A
ALL: 80%
AML: 15%
2
Q
what are the two types of chronic leukemia
A
CML
JMML
3
Q
what percentage of patients with leukemia will not have blasts on peripheral smear
A
20%
4
Q
what are some symptoms of leukemia
A
lack of normal bone marrow output
anemia- pallor, fatigue
thrombocytopenia- bruising, bleeding
infection
infiltration by blasts HSM lymphadenopathy bone pain limp fever
Sanctuary sites
CNS symptoms
testicular
5
Q
list 5 childhood conditions that predispose them to the development of leukemia
A
down syndrome NF1 Fanconi anemia Li- Fraumeni syndrome Noonan Bloom Ataxia telangiectasia WAS SCID DBA SCN
6
Q
what type of leukemia can be seen in babies with trisomy 21? Do they need treatment?
A
transient myeloproliferative disorder
present with increased WBC, peripheral blasts, pancytopenia, hepatosplenomegaly
typically do not need any treatment
7
Q
what is the most common leukemia is children with down syndrome
A
ALL
8
Q
what investigations are done to help diagnose leukemia
A
CBC+ smear
bone marrow aspirate and biopsy
LP
Testicular exam
9
Q
what percentage of blasts in the BM is required for the diagnosis of leukemia?
A
> 25% blasts in the BM
10
Q
what is the most common childhood cancer?
A
ALL
11
Q
what is the peak age for ALL
A
2-5 years
12
Q
is ALL usually B cell or T cell?
A
80% B cell
15% T cell
13
Q
how long is treatment for ALL? what’s the prognosis?
A
2.5-3.5 years
prognosis is good, 90% cure
14
Q
what are the prognostic factors for ALL
A
age <1 WBC >50 testicular or CNS disease cytogenetics( t9:22 is very poor) MRD
15
Q
what is MRD
A
minimal residual disease
disease reassessment after 1 month of therapy
one of the most important prognostic indicators
16
Q
how long is the therapy for AML? what’s the cure rate?
A
6 months of intensive chemotherapy
cure rate is 60%
17
Q
what is hyperleukocytosis
A
WBC >100
18
Q
how would you manage a patient with hyperleukocytosis
A
hydration- 1.5x maintenance allopurinol rasburicase platelets antibiotics call ICU
19
Q
what are the common chemotherapy drugs for ALL
A
Vincristine
steroid
asparaginase
daunorubicin
20
Q
a patient presents with a supraclavicular lymph node, what is the next step?
A
CXR
have to rule out a mediastinal mass
safety first!
21
Q
what is a common cancer seen in age 15-19
A
Hodgkins lymphoma
22
Q
how many blasts are seen on bone marrow aspiration for lymphoblastic lymphoma?
A
<25%
23
Q
what investigations would you do in an 8 year old male with an abdominal mass
A
CBC + smear LFTs electrolytes renal function LDH Urate
24
Q
what is the most common cause of lymphadenopathy
A
infection
25
what size lymph node is normal in cervical area? pelvic?
1cm in cervical area
| 1.5cm in pelvic area
26
what does a reed Sternberg cell mean?
Hodgkin's lymphoma
27
what are the 3 classes of non-hodgkins lymphoma?
Mature B cell: Burkitt's
Mature T cell: Anaplastic
Precursor T or B: Lymphoblastic lymphoma
28
what investigations should be done prior to chemotherapy for Hodgkins lymphphoma
echo
| PFT
29
what is the expected outcome for Hodgkins lymphoma?
90% cure
| but huge consequence of cure
30
what are some of the late toxicities associated with Hodgkin's lymphoma
```
infertility
pulmonary fibrosis
high risk of secondary malignancy- breast ca, thyroid ca
psychosocial
cardiovascular disease, heart failure
```
31
at what age do we typically see non-hodgkins lymphoma
grade 4 or 5 (8 to 10 years old)
32
what group of conditions is non-hodgkins lymphoma associated with?
up to 10% of children with congenital or acquired immunodeficiency develop NHL
33
what are some conditions associated with non-hodgkins lymphoma?
```
ataxia telangiectasia
wiskott aldrich syndrome
congenital hypogammaglobulinemia
Post solid organ transplant
HIV
```
34
how do patients with lymphoblastic lymphoma present?
bone marrow aspirate with <25% blasts
large mediastinal mass
elevated white count
35
how does Burkitts lymphoma present?
abdominal mass 80%
| waldeyer's ring 20%
36
what is waldeyer's ring?
The palatine tonsils, nasopharyngeal tonsil (adenoid) and lingual tonsil constitute the major part of Waldeyer's ring
37
what is the most important thing to worry about for burkitts lymphoma
Tumor lysis syndrome
| fastest proliferating malignancy that exists
38
what are the concerns with a mediastinal mass?
airway compromise- CXR, CT, PFTs
SVC syndrome- echo
RVOT obstruction- echo
cardiac tamponade- pulses paradoxus, ekg, echo
39
what is the ddx for anterior mediastinal mass?
thyroid
terrible lymphoma
teratoma
thymus
40
how does superior vena cava syndrome present?
dilated neck veins
facial swelling
altered mental status
41
what are some management options for a patient with a mediastinal mass
```
DO NOT LAY FLAT
biopsy under local
CT neck/chest/abdo/pelvis
PET scan
bilateral bone marrow aspirate/biopsy
Echo
consult anesthesia, ICU, Oncology
```
42
for what tumors do we worry about tumor lysis syndrome
Burkitts, other NHL
lymphoma
leukemia
has been reported in solid tumors
43
what are the laboratory features associated with tumor lysis syndrome
elevated k
elevated phosphate
elevated urate
low calcium
can have a metabolic acidosis and therefore low bicarb
44
what are 2 treatment options for high urate?
rasburicase
| allopurinol
45
what are the most common solid tumors in children?
brain tumors
46
what is the most common type of glioma
astrocytoma
| sometimes these terms are used interchangeably
47
what are common low grade astrocytomas in supratentorial area? infratentorial area?
supratentorial- optic pathway glioma
infratentorial- pilocytic astrocytoma
48
what is the most common malignant brain tumor
medulloblastoma
49
what is the most common type of brain tumor
glial tumors
50
where are most brain tumors found in children?
infratentorial
51
what are the common infratentorial brain tumors in children
1) astrocytomas
- cerebellar
- brainstem (diffuse intrinsic pontine glioma)
2) medulloblastoma
3) ependymoma
52
what are signs of supratentorial brain tumor
```
seizures**
visual changes
hemiparesisis
hemisensory loss
hyperreflexia
```
53
DI can indicate what type of brain tumor?
germinoma
54
what are signs of infratentorial brain tumor
raised ICP- h/a, N/V, 6th nerve palsy, papilledema, lethargy, cushings triad
ataxia
55
what syndromes are associated with gliomas/astrocytomas
NF1
| TS
56
what syndromes are associated with medulloblastoma
```
FAP
Gorlin
Rubinstein- Taybi
Li fraumeni
Fanconi anemia
```
57
gorlin syndrome is associated with which cancers?
PTCH mutation SHH pathway mutation *GROUP
Gorlin syndrome- associated with basal cell carcinoma primarily
but increased risk of medulloblastoma
also associated with Odontogenic cysts, skeletal and cutaneous abnormalities
58
Li- Fraumeni syndrome
```
mutation in tumor suppressor gene, p53.
breast cancer
osteosarcoma
sarcomas
brain tumors
leukemias
```
59
what could a 6th nerve palsy indicate?
brain tumor!!
60
what is considered the gold standard treatment for pediatric oncology patients
participation in clinical trials
61
which tumor is associated with claw sign on CT
wilm's tumor
62
```
Which tumour diagnosis would be supported by a
raised AFP level?
a) Hepatoblastoma
b) Wilms tumour
c) Neuroblastoma
d) Burkitt’s lymphoma
```
hepatoblastoma
63
```
What is the commonest site for metastases in
Osteosarcoma?
a) Lymph nodes
b) Adjacent bone (“skip lesions”)
c) Brain
d) Lung
```
lung
64
```
Which of these IS a long long-term side effect of
Cisplatin?
a) Hepatic fibrosis
b) Hearing loss
c) Infertility
d) Cardiomyopathy
```
hearing loss
65
```
Which of these is NOT a short short-term side effect of
Doxorubicin
a) Myelosuppression
b) Hair loss
c) Nausea/vomiting
d) Haemorrhagic cystitis
```
hemorrhagic cystitis
66
what type of cell do these suggest:
CD19
CD3
MPO
CD19- B cell
CD3- T cell
MPO- myeloid marker
67
```
Acute Lymphoblastic Leukaemia comprises
what proportion of childhood cancer cases?
a) 10%
b) 15%
c) 25%
d) 40%
```
25%
68
Which of these drugs MUST NOT be given intrathecally?
a) Hydrocortisone
b) Vincristine
c) Methtrexate
d) Cytarabine
Vincristine- huge neurotoxin
Intrathecal administration:
- Severe demyelination
- Severe encephalopathy
- Severe pain
- Death, usually
69
Which of these is NOT a B symptom?
a) Drenching night sweats (within the last month)
b) Persistent itch
c) Weight loss >10% (over six months)
d) Fevers >38 o C
persistent itch
70
what are the 2 treatment options for SVC syndrome
1. steroids
| 2. radiotherapy
71
staging for lymphoma (stage 1, 2, 3, 4)
Stage 1- Single nodal group
Stage 2- 2 or more nodal groups on SAME side of diaphragm
Stage 3- 2 or more nodal groups on BOTH sides of diaphragm
Stage 4- Involvement of extra-lymphoid tissues
72
what is the definition of febrile neutropenia
Fever
> 38.3 oC x 1, or > 38oC over at least 1 hour
Absolute neutrophil count
< 0.5 x 10^9/L
+/ -evidence of sepsis = hypotension needing fluid resuscitation
73
what are some long term side effects from radiation therapy
```
Fibrosis/scarring
Growth impairment (children)
Endocrinopathies
Second malignancies
Xerophthalmia
Xerostomia
Cataract
Intellectual impairment
```
74
what are some short term side effects from radiation therapy
Nausea/vomiting
Fatigue
Radiation “burns”
75
what is an emergent treatment for hydrocephalus secondary to brain tumor
dexamethasone
76
A 6 yo girl presents with 2 month hx of head tilt,
diplopia, papilledema and ataxia
Where is the lesion?
posterior fossa
77
what signs would suggest brainstem involvement
head tilt
| cranial nerve palsies
78
A 3 year old girl presents with abdominal
distention and pallor. A mass is palpable on exam.
Hgb 80, normal WBC.
What is your ddx?
List 3 investigations that may help you
narrow your ddx pre biopsy
1. hepatoblastoma
2. neuroblastoma
3. wilm's tumor
4. lymphoma
1. AFP
2. urine VNA, HVA
3. ultrasound
79
what are some physical exam
| features that may be associated with neuroblastoma?
```
hypertension
abdominal mass
bruising below eyes
bone pain
nystagmus
```
80
```
Opsoclonus-myoclonus-ataxia (OMA) is seen in
which of the following malignancies?
a) Leukemia
b) Rhabdomyosarcoma
c) Neuroblastoma
d) Medulloblastoma
```
neuroblastoma
81
what are two unique paraneoplastic syndromes associated with neuroblastoma?
• Opsoclonus-Myoclonus-Ataxia
Rapid eye movements, ataxia, myoclonus
• VIP-induced diarrhea
get hypokalemia and dehydration
82
```
Better prognosis with neuroblastoma is
associated with?
a) Female
b) High excretion of VMA/HVA
c) Age < 12-18 months
d) Normal blood pressure
e) Unilateral disease
```
age <12- 18 months
83
what is the most common renal tumor in children
wilm's
84
what are 3 predisposing syndromes for wilm's tumor
Beckwith-Wiedemann
Hemi-hyperplasia
WAGR
85
what are 3 predisposing conditions for hepatoblastoma
BWS
Hemihyperplasia
FAP – family history of polyps
Prematurity/LBW
90% have ↑↑↑ alphafetoprotein=marker of embryonic liver tissue
86
A child presents with a distended abdomen. An US
shows an adrenal mass and hepatomegaly. Which
of the following tests will make the diagnosis?
a) MIBG
b) Serum AFP
c) Urine VMA/HVA
d) CT chest/abdo
Urine VMA/ HVA
87
what is the most common intraabdominal tumor in children
Neuroblastoma is the most common intraabdominal
tumor in children
wilm's= second most common
88
14 year old female presents with pain above right
knee, worse at night and with activity. On exam there
is no fever, but there is tenderness to palpation above
the knee.
X-ray shows periosteal elevation and mottling of the
distal femur. The most likely diagnosis is:
a) Osteomyelitis
b) Osteosarcoma
c) Osteoid osteoma
d) Ewing’s sarcoma
e) Sub-periosteal hematoma
osteosarcoma
89
how does an osteoid osteoma present
dull pain at night
improves with NSAIDS
on x ray: well defined lucency surrounded by sclerotic bone
90
where does osteosarcoma occur
metaphysis of long bones
91
what is the xray appearance for osteosarcoma
sunburst appearance
92
what is the xray appearance for Ewings sarcoma
onion skinning
93
what are two predisposing conditions for osteosarcoma
LFS, RB
94
where does Ewings sarcoma occur?
diaphysis of long bones
| axial skeleton
95
what are the treatment options for osteosarcoma
chemotherapy and surgery
96
what are the treatment options for Ewings sarcoma
chemo, surgery and radiation
97
```
A 12 year old boy presents with an X-ray
(described, no picture given) of mottled
bone on right rib with new bone forming
on top. What is the most likely diagnosis?
a) Osteosarcoma
b) Ewing sarcoma
c) Osteoid osteoma
d) Enchondroma
```
Ewing sarcoma
98
```
List 3 side effects of each of the
following drugs:
• Vincristine
• Asparaginase
• Prednisone
```
Vincristine- SIADH, peripheral neuropathy, constipation
Asparaginase- Allergic reaction, hepatopathy, coagulopathy/thrombosis, pancreatitis
prednisone- Hypertension, hyperglycemia, AVN, cataracts
99
```
The most common organism isolated in episodes
of febrile neutropenia is?
a) PCP
b) E.Coli
c) CONS
d) Pseudomonas
e) Candida
```
CONS
gram positives> gram negatives
100
```
Wilms tumor is associated with all of the
following except?
a) 11p deletion
b) BWS
c) Wiskott Aldrich syndrome
d) NF-1
e) Anirida
```
WAS
101
```
Which condition is most associated with
childhood leukemia?
a) Electromagnetic field exposure during
pregnancy
b) NF-1
c) Family history of adult leukemia
```
NF1
102
Child with hemi-hypertrophy. What
condition would you suspect that would
require serial follow-up?
Beckwith Wiedemann syndrome
Hepatoblastoma: AFP q 3 months until age 4
Wilms’ Tumor: Abdo U/S q 3 months until age 8
103
An 11 mo. old presents with generalized scaly rash
worse in diaper area. He also has exopthalmos
and HSM. Xrays show bony lucencies of the skull.
What is the likely dx?
a) Langerhans cell histiocytosis
b) Neuroblastoma
c) ALL
d) Multifocal osteomyelitis
Langerhands cell histocytosis
* can have CNS involvement and present with DI
104
This baby has had a rash for 4 months, refractory
to barrier creams and clotrimazole. She soaks
through 12 diapers a day
What is your diagnosis?
What test would you do to
confirm your diagnosis?
LCH
| skin biopsy
105
```
Poor prognosis (mortality risk) in LCH
is associated with
a) Mastoiditis
b) Pancytopenia
c) Lymphadenopathy
d) Chronic lung disease
e) Vertebral body involvement
```
pancytopenia
106
A newborn presents with an abdominal mass,
thrombocytopenia, systolic heart murmur, and a
murmur over the liver. An ultrasound shows a
3cm mass in the liver.
What would be your next investigation?
a) DIC work up
b) Bone marrow evaluation
c) Echocardiogram
d) Liver function tests
DIC workup
Kasabach-Merritt
• Disseminated intravascular coagulation with capillary hemangiomas
• Associated with kaposiform hemangioendotheliomas or tufted angioma
• Thrombocytopenia from sequestration/ destruction of platelets in vascular malformation
• Treatment can include steroids and chemotherapy
107
```
what are the common side effects for:
bleomycin
cisplatin/carboplatin
MTX
Cyclophosphamide
Vincristine
```
bleomycin- pulmonary fibrosis
cisplatin/carboplatin- ototoxicity, renal toxicity
MTX- myelosuppression
Cyclophosphamide- hemorrhagic cystitis
vincristine- peripheral neuropathy, SIADH, constipation
108
6 month old baby with pallor, hepatosplenomegaly, irritable. Xray shown with very white bones. Diagnosis:
osteoporosis
109
Complication of doxorubicin?
1. Restrictive cardiomyopathy
2. Dilated cardiomyopathy
3. Hypertrophyic cardiomyopathy
4. Pericarditis
dilated cardiomyopathy
110
Newborn has a flank mass. What is the most likely cause
a. Wilms tumour
b. Hydronephrosis
c. Polycystic kidney
hydronephrosis
111
```
You are a rural doctor seeing a child who had just completed chemotherapy. What side effects of anthracyclines do you need to monitor for?
Hearing loss
Cardiac dysfunction
Thyroid dysfunction
Renal dysfunction
```
main adverse effect is cardiotoxicity
112
Which is the most permanent toxicity of vincristine?
Peripheral neuropathy
Seizures
Constipation
peripheral neuropathy
113
Child with pancytopenia, short stature, abnormal thumbs. What is the diagnosis?
Fanconi anemia
Clinical manifestations:
Hyperpigmentation of the trunk, neck, intertriginous areas, as well as cafe-au-lait spots and vitilgo
Short stature (50%)
Absence of radii and thumbs that are hypoplastic, supernumerary, bifid, or absent
The “r” radial pulse may be weak or absent
Anomalies of the feet, congenital hip dislocation, and leg abnormalities are seen
In males: underdeveloped penis, undescended, atrophic or absent testes, hypospadias or phimosis
Females: malformations of the vagina, uterus and ovaries
FA “facies”: microcephaly, small eyes, epicanthal folds, and abnormal shape, size or positioning of the ears
