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Pediatrics- Noelle > Oncology > Flashcards

Oncology Flashcards

1
Q

what are the two classes of acute leukemia

A

ALL: 80%
AML: 15%

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2
Q

what are the two types of chronic leukemia

A

CML

JMML

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3
Q

what percentage of patients with leukemia will not have blasts on peripheral smear

A

20%

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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

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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
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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

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7
Q

what is the most common leukemia is children with down syndrome

A

ALL

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8
Q

what investigations are done to help diagnose leukemia

A

CBC+ smear
bone marrow aspirate and biopsy
LP
Testicular exam

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9
Q

what percentage of blasts in the BM is required for the diagnosis of leukemia?

A

> 25% blasts in the BM

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10
Q

what is the most common childhood cancer?

A

ALL

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11
Q

what is the peak age for ALL

A

2-5 years

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12
Q

is ALL usually B cell or T cell?

A

80% B cell

15% T cell

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13
Q

how long is treatment for ALL? what’s the prognosis?

A

2.5-3.5 years

prognosis is good, 90% cure

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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
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15
Q

what is MRD

A

minimal residual disease
disease reassessment after 1 month of therapy
one of the most important prognostic indicators

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16
Q

how long is the therapy for AML? what’s the cure rate?

A

6 months of intensive chemotherapy

cure rate is 60%

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17
Q

what is hyperleukocytosis

A

WBC >100

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18
Q

how would you manage a patient with hyperleukocytosis

A 
hydration- 1.5x maintenance
allopurinol
rasburicase
platelets
antibiotics
call ICU
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19
Q

what are the common chemotherapy drugs for ALL

A

Vincristine
steroid
asparaginase
daunorubicin

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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!

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21
Q

what is a common cancer seen in age 15-19

A

Hodgkins lymphoma

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22
Q

how many blasts are seen on bone marrow aspiration for lymphoblastic lymphoma?

A

<25%

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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
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24
Q

what is the most common cause of lymphadenopathy

A

infection

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25
Q

what size lymph node is normal in cervical area? pelvic?

A

1cm in cervical area

1.5cm in pelvic area

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26
Q

what does a reed Sternberg cell mean?

A

Hodgkin’s lymphoma

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27
Q

what are the 3 classes of non-hodgkins lymphoma?

A

Mature B cell: Burkitt’s
Mature T cell: Anaplastic
Precursor T or B: Lymphoblastic lymphoma

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28
Q

what investigations should be done prior to chemotherapy for Hodgkins lymphphoma

A

echo

PFT

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29
Q

what is the expected outcome for Hodgkins lymphoma?

A

90% cure

but huge consequence of cure

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30
Q

what are some of the late toxicities associated with Hodgkin’s lymphoma

A 
infertility
pulmonary fibrosis
high risk of secondary malignancy- breast ca, thyroid ca
psychosocial
cardiovascular disease, heart failure
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31
Q

at what age do we typically see non-hodgkins lymphoma

A

grade 4 or 5 (8 to 10 years old)

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32
Q

what group of conditions is non-hodgkins lymphoma associated with?

A

up to 10% of children with congenital or acquired immunodeficiency develop NHL

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33
Q

what are some conditions associated with non-hodgkins lymphoma?

A 
ataxia telangiectasia
wiskott aldrich syndrome
congenital hypogammaglobulinemia
Post solid organ transplant
HIV
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34
Q

how do patients with lymphoblastic lymphoma present?

A

bone marrow aspirate with <25% blasts
large mediastinal mass
elevated white count

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35
Q

how does Burkitts lymphoma present?

A

abdominal mass 80%

waldeyer’s ring 20%

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36
Q

what is waldeyer’s ring?

A

The palatine tonsils, nasopharyngeal tonsil (adenoid) and lingual tonsil constitute the major part of Waldeyer’s ring

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37
Q

what is the most important thing to worry about for burkitts lymphoma

A

Tumor lysis syndrome

fastest proliferating malignancy that exists

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38
Q

what are the concerns with a mediastinal mass?

A

airway compromise- CXR, CT, PFTs
SVC syndrome- echo
RVOT obstruction- echo
cardiac tamponade- pulses paradoxus, ekg, echo

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39
Q

what is the ddx for anterior mediastinal mass?

A

thyroid
terrible lymphoma
teratoma
thymus

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40
Q

how does superior vena cava syndrome present?

A

dilated neck veins
facial swelling
altered mental status

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41
Q

what are some management options for a patient with a mediastinal mass

A 
DO NOT LAY FLAT
biopsy under local
CT neck/chest/abdo/pelvis
PET scan
bilateral bone marrow aspirate/biopsy
Echo
consult anesthesia, ICU, Oncology
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42
Q

for what tumors do we worry about tumor lysis syndrome

A

Burkitts, other NHL
lymphoma
leukemia
has been reported in solid tumors

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43
Q

what are the laboratory features associated with tumor lysis syndrome

A

elevated k
elevated phosphate
elevated urate
low calcium

can have a metabolic acidosis and therefore low bicarb

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44
Q

what are 2 treatment options for high urate?

A

rasburicase

allopurinol

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45
Q

what are the most common solid tumors in children?

A

brain tumors

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46
Q

what is the most common type of glioma

A

astrocytoma

sometimes these terms are used interchangeably

47
Q

what are common low grade astrocytomas in supratentorial area? infratentorial area?

A

supratentorial- optic pathway glioma

infratentorial- pilocytic astrocytoma

48
Q

what is the most common malignant brain tumor

A

medulloblastoma

49
Q

what is the most common type of brain tumor

A

glial tumors

50
Q

where are most brain tumors found in children?

A

infratentorial

51
Q

what are the common infratentorial brain tumors in children

A

1) astrocytomas
- cerebellar
- brainstem (diffuse intrinsic pontine glioma)
2) medulloblastoma
3) ependymoma

52
Q

what are signs of supratentorial brain tumor

A 
seizures**
visual changes
hemiparesisis
hemisensory loss
hyperreflexia
53
Q

DI can indicate what type of brain tumor?

A

germinoma

54
Q

what are signs of infratentorial brain tumor

A

raised ICP- h/a, N/V, 6th nerve palsy, papilledema, lethargy, cushings triad
ataxia

55
Q

what syndromes are associated with gliomas/astrocytomas

A

NF1

TS

56
Q

what syndromes are associated with medulloblastoma

A 
FAP
Gorlin
Rubinstein- Taybi
Li fraumeni
Fanconi anemia
57
Q

gorlin syndrome is associated with which cancers?

A

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
Q

Li- Fraumeni syndrome

A 
mutation in tumor suppressor gene, p53. 
breast cancer
osteosarcoma
sarcomas
brain tumors
leukemias
59
Q

what could a 6th nerve palsy indicate?

A

brain tumor!!

60
Q

what is considered the gold standard treatment for pediatric oncology patients

A

participation in clinical trials

61
Q

which tumor is associated with claw sign on CT

A

wilm’s tumor

62
Q 
Which tumour diagnosis would be supported by a
raised AFP level?
a) Hepatoblastoma
b) Wilms tumour
c) Neuroblastoma
d) Burkitt’s lymphoma
A

hepatoblastoma

63
Q 
What is the commonest site for metastases in
Osteosarcoma?
a) Lymph nodes
b) Adjacent bone (“skip lesions”)
c) Brain
d) Lung
A

lung

64
Q 
Which of these IS a long long-term side effect of
Cisplatin?
a) Hepatic fibrosis
b) Hearing loss
c) Infertility
d) Cardiomyopathy
A

hearing loss

65
Q 
Which of these is NOT a short short-term side effect of
Doxorubicin
a) Myelosuppression
b) Hair loss
c) Nausea/vomiting
d) Haemorrhagic cystitis
A

hemorrhagic cystitis

66
Q

what type of cell do these suggest:
CD19
CD3
MPO

A

CD19- B cell
CD3- T cell
MPO- myeloid marker

67
Q 
Acute Lymphoblastic Leukaemia comprises
what proportion of childhood cancer cases?
a) 10%
b) 15%
c) 25%
d) 40%
A

25%

68
Q

Which of these drugs MUST NOT be given intrathecally?

a) Hydrocortisone
b) Vincristine
c) Methtrexate
d) Cytarabine

A

Vincristine- huge neurotoxin

Intrathecal administration:

  • Severe demyelination
  • Severe encephalopathy
  • Severe pain
  • Death, usually
69
Q

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

A

persistent itch

70
Q

what are the 2 treatment options for SVC syndrome

A
  1. steroids

2. radiotherapy

71
Q

staging for lymphoma (stage 1, 2, 3, 4)

A

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
Q

what is the definition of febrile neutropenia

A

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
Q

what are some long term side effects from radiation therapy

A 
Fibrosis/scarring
Growth impairment (children)
Endocrinopathies
Second malignancies
Xerophthalmia
Xerostomia
Cataract
Intellectual impairment
74
Q

what are some short term side effects from radiation therapy

A

Nausea/vomiting
Fatigue
Radiation “burns”

75
Q

what is an emergent treatment for hydrocephalus secondary to brain tumor

A

dexamethasone

76
Q

A 6 yo girl presents with 2 month hx of head tilt,
diplopia, papilledema and ataxia
Where is the lesion?

A

posterior fossa

77
Q

what signs would suggest brainstem involvement

A

head tilt

cranial nerve palsies

78
Q

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

A
  1. hepatoblastoma
  2. neuroblastoma
  3. wilm’s tumor
  4. lymphoma
  5. AFP
  6. urine VNA, HVA
  7. ultrasound
79
Q

what are some physical exam

features that may be associated with neuroblastoma?

A 
hypertension
abdominal mass
bruising below eyes
bone pain
nystagmus
80
Q 
Opsoclonus-myoclonus-ataxia (OMA) is seen in
which of the following malignancies?
a) Leukemia
b) Rhabdomyosarcoma
c) Neuroblastoma
d) Medulloblastoma
A

neuroblastoma

81
Q

what are two unique paraneoplastic syndromes associated with neuroblastoma?

A

• Opsoclonus-Myoclonus-Ataxia
Rapid eye movements, ataxia, myoclonus
• VIP-induced diarrhea
get hypokalemia and dehydration

82
Q 
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
A

age <12- 18 months

83
Q

what is the most common renal tumor in children

A

wilm’s

84
Q

what are 3 predisposing syndromes for wilm’s tumor

A

Beckwith-Wiedemann
Hemi-hyperplasia
–WAGR

85
Q

what are 3 predisposing conditions for hepatoblastoma

A

BWS
Hemihyperplasia
FAP – family history of polyps
Prematurity/LBW

90% have ↑↑↑ alphafetoprotein=marker of embryonic liver tissue

86
Q

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

A

Urine VMA/ HVA

87
Q

what is the most common intraabdominal tumor in children

A

Neuroblastoma is the most common intraabdominal
tumor in children
wilm’s= second most common

88
Q

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

A

osteosarcoma

89
Q

how does an osteoid osteoma present

A

dull pain at night
improves with NSAIDS
on x ray: well defined lucency surrounded by sclerotic bone

90
Q

where does osteosarcoma occur

A

metaphysis of long bones

91
Q

what is the xray appearance for osteosarcoma

A

sunburst appearance

92
Q

what is the xray appearance for Ewings sarcoma

A

onion skinning

93
Q

what are two predisposing conditions for osteosarcoma

A

LFS, RB

94
Q

where does Ewings sarcoma occur?

A

diaphysis of long bones

axial skeleton

95
Q

what are the treatment options for osteosarcoma

A

chemotherapy and surgery

96
Q

what are the treatment options for Ewings sarcoma

A

chemo, surgery and radiation

97
Q 
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
A

Ewing sarcoma

98
Q 
List 3 side effects of each of the
following drugs:
• Vincristine
• Asparaginase
• Prednisone
A

Vincristine- SIADH, peripheral neuropathy, constipation

Asparaginase- Allergic reaction, hepatopathy, coagulopathy/thrombosis, pancreatitis

prednisone- Hypertension, hyperglycemia, AVN, cataracts

99
Q 
The most common organism isolated in episodes
of febrile neutropenia is?
a) PCP
b) E.Coli
c) CONS
d) Pseudomonas
e) Candida
A

CONS

gram positives> gram negatives

100
Q 
Wilms tumor is associated with all of the
following except?
a) 11p deletion
b) BWS
c) Wiskott Aldrich syndrome
d) NF-1
e) Anirida
A

WAS

101
Q 
Which condition is most associated with
childhood leukemia?
a) Electromagnetic field exposure during
pregnancy
b) NF-1
c) Family history of adult leukemia
A

NF1

102
Q

Child with hemi-hypertrophy. What
condition would you suspect that would
require serial follow-up?

A

Beckwith Wiedemann syndrome
Hepatoblastoma: AFP q 3 months until age 4
Wilms’ Tumor: Abdo U/S q 3 months until age 8

103
Q

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

A

Langerhands cell histocytosis

  • can have CNS involvement and present with DI
104
Q

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?

A

LCH

skin biopsy

105
Q 
Poor prognosis (mortality risk) in LCH
is associated with
a) Mastoiditis
b) Pancytopenia
c) Lymphadenopathy
d) Chronic lung disease
e) Vertebral body involvement
A

pancytopenia

106
Q

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

A

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
Q 
what are the common side effects for:
bleomycin
cisplatin/carboplatin
MTX
Cyclophosphamide
Vincristine
A

bleomycin- pulmonary fibrosis
cisplatin/carboplatin- ototoxicity, renal toxicity
MTX- myelosuppression
Cyclophosphamide- hemorrhagic cystitis
vincristine- peripheral neuropathy, SIADH, constipation

108
Q

6 month old baby with pallor, hepatosplenomegaly, irritable. Xray shown with very white bones. Diagnosis:

A

osteoporosis

109
Q

Complication of doxorubicin?

  1. Restrictive cardiomyopathy
  2. Dilated cardiomyopathy
  3. Hypertrophyic cardiomyopathy
  4. Pericarditis
A

dilated cardiomyopathy

110
Q

Newborn has a flank mass. What is the most likely cause

a. Wilms tumour
b. Hydronephrosis
c. Polycystic kidney

A

hydronephrosis

111
Q 
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
A

main adverse effect is cardiotoxicity

112
Q

Which is the most permanent toxicity of vincristine?
Peripheral neuropathy
Seizures
Constipation

A

peripheral neuropathy

113
Q

Child with pancytopenia, short stature, abnormal thumbs. What is the diagnosis?

A

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

Pediatrics- Noelle (28 decks)

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