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Royal College Exam > Oncology > Flashcards

Oncology Flashcards

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1
Q

Who develops transient myeloproliferative disorder?

A

10% of DS children develop transient abnormal myelopoeisis in the first three months of life

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2
Q

What is transient myeloproliferative disorder?

A

accumulation of immature megakaryoblasts in blood and liver (lesser degree in bone marrow)

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3
Q

What genetic mutation is transient myeloproliferative disorder associated with?

A

GATA 1

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4
Q

How does rasburicase work?

A

Urate oxidase –> promotes degradation of uric acid.

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5
Q

Langerhans cell histiocytosis - what is this?

A

Rare histiocytic disorder characterized by osteolytic bone lesions with infiltration by histiocytes. Can infiltrate nearly every organ, but most commonly bone and skin.

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6
Q

What are the most common adolescent breast lesions?

A

Juvenile fibroadenoma - Account for 30-50% of breast masses in adolescents.

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7
Q

What are most common x-ray findings in Ewings versus Osteosarcoma?

A

Ewing - most common X-ray finding is onion skinning or periosteal reaction (bone building on top of bone)

Osteosarcoma – most common X-ray finding is lytic lesions (sun burst pattern, building new born perpendicular to old bone)

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8
Q

5 childhood conditions that predispose to developing leukemia

A 
Neurofibromatosis  Type 1
Trisomy 21
Ataxia Telangiectasia
Fanconi anemia
Li-Fraumeni Syndrome
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9
Q

What type of leukemias present in childhood?

A

ALL (80%)
AML (15%)
CML
JMML

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10
Q

How many patients with leukemia will have circulating blasts at diagnosis?

A

80%

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11
Q

x-ray findings in bony leukemia involvement

A
  • transverse radiolucent metaphyseal growth arrest lines
  • periosteal elevation with reactive subperiosteal cortical thickening
  • osteolytic lesions
  • diffuse osteopenia/ osteoporosis
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12
Q

Other risk factors for leukemia (non-genetic conditions)

A

previous chemotherapy, radiation (including in utero), benzene

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13
Q

Prognosis of all

A

> 90%

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14
Q

prognosis of AML

A

60%

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15
Q

ALL - poor prognostic factors

A
  • AGE: < 1 and > 10 yrs*
  • WHITE CELL COUNT: > 50 *
  • CNS and/or TESTICULAR DISEASE
  • CYTOGENETICS (Good – t (12;21) = TEL-AML; hyperdiploidy / Poor – MLL gene rearrangement (11q23) / V. poor – t (9;22) = Ph chr and hypodiploidy)

-DISEASE RESPONSE
(MRD at the end of induction chemotherapy)

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16
Q

What brain tumour do patients with tuberous sclerosis develop?

A

15% develop subependymal giant astrocytomas (SEGA)

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17
Q

What brain tumour do patients with tuberous sclerosis develop?

A

15% develop subependymal giant astrocytomas (SEGA)

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18
Q

What brain tumour can patients with NF1 develop?

A

optic nerve glioma

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19
Q

Risk for pediatric renal medullary carcinoma?

A

Sickle cell trait!

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20
Q

How many children with leukemia will not have circulating blasts?

A

20%

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21
Q

ALL vs JIA - 3 factors that predict ALL

A

Low WBC < 4
Low-normal Plts 150-250
night time pain (hx)

(sensitivity 100% specificity 85%)

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22
Q

Poor prognosis in ALL is suggested by

A

Age < 1 yr, > 10yr

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23
Q

7 childhood conditions predisposing to leukemia

A 
T21
NF1
Ataxia-telangiectasia
Fanconi
Bloom
Noonan
Li-Fraumeni
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24
Q

Most common type of childhood leukemia

A

ALL (80%)

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25
Q

Treatment of CML

A

tyrosine kinase inhibitor (imatinib)

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26
Q

X-ray findings in leukemia

A

transverse metaphyseal growth arrest lines

osteolytic lesions

diffuse osteopenia

fractures

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27
Q

Extramedullary disease in leukemia

A 
chloroma
leukemia cutis
gingival hyperplasia
HSM
LNs
Lymphomatous masses
CNS disease*
Testicular disease*
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28
Q

Good cytogenetics

A

tel-aml

trisomy 4, 10

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29
Q

poor cytogenetics

A

Ph chromosome
hypodiploidy
MLL

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30
Q

Generalized lymphadenopathy - definition

A

> 2 contiguous groups

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31
Q

Chronic lymphadenopathy

A

> 4 weeks

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32
Q

Leading cause of death among survivors of hodgkin’s lymphoma > 15 yrs post treatment

A

second malignancy

then cardiovascular disease

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33
Q

Most common presentation of Burkitt’s lymphoma

A

abdominal mass

w/ pathologic lead point

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34
Q

Doubling time of Burkitt’s lymphoma and subsequent risk?

A

doubling time 24 hrs

risk of TLS

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35
Q

DDx anterior mediastinal mass

A 
lymphoma (hodgkins, NHL)
thymus
thymoma
teratoma
thyroid
36
Q

Presentation of SVC syndrome

A

plethora of the face/upper body

Change in LOC

Coma/stroke

37
Q

Causes of SVC syndrome

A

Mass (extra luminal)

Thrombus (intra luminal)

38
Q

Two most common posterior fossa tumours

A

Cerebellar astrocytoma

Medulloblastoma

39
Q

Symptoms of posterior fossa tumour

A

diplopia
headache
ataxia

40
Q

Most common malignancy childhood brain tumour

A

medulloblastoma

41
Q

What is posterior fossa?

A

cerebellum

brainstem

42
Q

Signs of supratentorial mass

A

behavioural problems
neuroendocrine deficits
seizures
visual changes

43
Q

Brainstem triad

A

Ataxia

Long tract signs (hyperreflexia)

Cranial nerve involvement

44
Q

Dull bone pain worse at night, better with NSAIDs

X-ray: well-defined lucency surrounded by sclerotic bone

A

Osteoid Osteoma

45
Q

Non-painful, bony mass

Often in metaphysis of long bones

May have stalk-like base

A

Osteochondroma

46
Q

Expansile lesion within bone filled with blood/solid components

often in metaphysis

A

aneurysmal bone cyst

47
Q

Most common bone tumour in adolescents

A

osteosarcoma

48
Q

Osteosarcoma - sites of metastases

A

lung, bone

49
Q

Ewing’s sarcoma involves which bones?

A

Lots!

flat bones of axial skeleton, long bones (diaphysis)

50
Q

Features of Ewing’s on x-ray

A

moth-eating appearance w/ “onion skin” periosteal reaction

associated soft tissue mass

51
Q

x-ray appearance Osteosarcoma

A

sunburst reaction

“bone outside of bone”

52
Q

Paraneoplastic syndromes in neuroblastoma

A

Opsoclonus-myoclonus-ataxia

Vaso-active intestinal peptide (VIP) secretion - leading to diarrhea and hypokalemia

53
Q

Prognosis of a child with neuroblastoma < 12-18 months?

A

Favourable

54
Q

Staging of neuroblastoma

A 
CT/MRI
MIBG
Bone scan
Bone marrows
Urine HVA/VMA
Biopsy (for biology, n-myc)
55
Q

Infant with stage 4S neuroblastoma

A

Skin involvement (blue spots)
Hepatomegaly
Bone marrow < 10%
NO mets to bone

May not require treatment!

56
Q

Four risk factors for hepatoblastoma

A

BWS

hemihypertrophy

family hx of FAP

prematurity

57
Q

Tumour lysis

A

hyperkalemia
hypocalcemia
hyperphosphatemia
hyperuricemia

58
Q

Definition of hyperleukocytosis, organs that it can affect

A

WBC > 100

Brain (intracranial hemorrhage)
Lungs
Kidney

59
Q

Management of hyperleukocytosis

A

Hydration!

Rasburicase

Platelets - at risk of bleeding (plts and FFP do not increase viscosity)

60
Q

Patient with hyperleukocytosis. When would you transfuse pRBCs?

A

Only if symptomatic from anemia (heart failure) - give small amount of blood (2-5 cc/kg)

61
Q

Oncologic emergencies

A 
TLS
Fever + Neutropenia
Cord compression
Mediastinal mass
APML
62
Q

Most common bug in febrile neutropenia

A

gram positive organisms

63
Q

How do you manage an immunocompromised child exposed to varicella? (with no symptoms)

A

VZIG

64
Q

3 side effects of vincristine

A

siADH
constipation
jaw pain

65
Q

3 side effects of peg-asparaginase

A

pancreatitis
anaphylaxis
thrombosis

66
Q

3 side effects of bleomycin

A

pulmonary fibrosis

hypersensitivity, rash

67
Q

side effects of cyclophosphamide

A

hemorrhagic cystitis

infertility

68
Q

side effects of etoposide

A 
allergic reaction
secondary malignancies (leukemia)
69
Q

Side effects of cisplatin

A

N/V
hearing loss
nephrotoxicity

70
Q

methotrexate side effects

A

renal failure
mucositis
leukoencephalopathy

71
Q

late effects after neck radiation

A

hypothyroidism

72
Q

poor prognosis in LCH is associated with…

A

bone marrow involvement (pancytopenia)

hemorrhagic skin lesions

hepatomegaly

young age at diagnosis (< 18 mos)

73
Q

diagnostic test for LCH?

A

biopsy of involved bone/skin

74
Q

Autologous transplant - what is the principle

A

Give them back their own bone marrow as a “rescue”

75
Q

Allogeneic transplant side effect

A

Graft versus host disease

76
Q

Acute GVHD commonly affects what organs

A

Liver
Gut
Skin

77
Q

RFs for bad GVHD

A

mismatched/unrelated donor
bone marrow transplant
older age

78
Q

when does chronic GVHD occur?

A

> 100 days

can affect any organ!

79
Q

What is osteopetrosis?

A

inherited bone disease involving dense, sclerotic bones prone to fracture

Cure: BMT

80
Q

What is Kasabach-Merritt

A

DIC associated with capillary hemangiomas

81
Q

What investigation does a child with WAGR need regularly?

A

Abdominal ultrasound for Wilm’s tumour

82
Q

Wilm’s tumour is associated with

A 
NF
11p
Denys-Drash
Aniridia, WAGR
B-W
Hemihypertrophy
83
Q

What predisposes to leukemia:

  • exposure to rads
  • mom or dad with leukemia
  • NF1
A

NF1

84
Q

Two tumours that children with Beckwith-Wiedemann are at increased risk of?

A

hepatoblastoma

Wilms tumour

85
Q

Retinoblastoma - increased risk of what other cancers?

A

Osteosarcoma

Melanoma

86
Q

MEN2 - what types of cancers are they at risk of?

A

medullary thyroid cancer

pheochromocytoma

87
Q

familial adenomatous polyposis - at risk of what type of cancers?

A

colorectal ca
hepatoblastoma
thyroid ca

Royal College Exam (32 decks)

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