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Royal College Exam > Oncology > Flashcards

Oncology Flashcards

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
Treatment of CML
tyrosine kinase inhibitor (imatinib)
26
X-ray findings in leukemia
transverse metaphyseal growth arrest lines osteolytic lesions diffuse osteopenia fractures
27
Extramedullary disease in leukemia
``` chloroma leukemia cutis gingival hyperplasia HSM LNs Lymphomatous masses CNS disease* Testicular disease* ```
28
Good cytogenetics
tel-aml | trisomy 4, 10
29
poor cytogenetics
Ph chromosome hypodiploidy MLL
30
Generalized lymphadenopathy - definition
> 2 contiguous groups
31
Chronic lymphadenopathy
> 4 weeks
32
Leading cause of death among survivors of hodgkin's lymphoma > 15 yrs post treatment
second malignancy | then cardiovascular disease
33
Most common presentation of Burkitt's lymphoma
abdominal mass | w/ pathologic lead point
34
Doubling time of Burkitt's lymphoma and subsequent risk?
doubling time 24 hrs risk of TLS
35
DDx anterior mediastinal mass
``` lymphoma (hodgkins, NHL) thymus thymoma teratoma thyroid ```
36
Presentation of SVC syndrome
plethora of the face/upper body Change in LOC Coma/stroke
37
Causes of SVC syndrome
Mass (extra luminal) Thrombus (intra luminal)
38
Two most common posterior fossa tumours
Cerebellar astrocytoma | Medulloblastoma
39
Symptoms of posterior fossa tumour
diplopia headache ataxia
40
Most common malignancy childhood brain tumour
medulloblastoma
41
What is posterior fossa?
cerebellum | brainstem
42
Signs of supratentorial mass
behavioural problems neuroendocrine deficits seizures visual changes
43
Brainstem triad
Ataxia Long tract signs (hyperreflexia) Cranial nerve involvement
44
Dull bone pain worse at night, better with NSAIDs X-ray: well-defined lucency surrounded by sclerotic bone
Osteoid Osteoma
45
Non-painful, bony mass Often in metaphysis of long bones May have stalk-like base
Osteochondroma
46
Expansile lesion within bone filled with blood/solid components often in metaphysis
aneurysmal bone cyst
47
Most common bone tumour in adolescents
osteosarcoma
48
Osteosarcoma - sites of metastases
lung, bone
49
Ewing's sarcoma involves which bones?
Lots! | flat bones of axial skeleton, long bones (diaphysis)
50
Features of Ewing's on x-ray
moth-eating appearance w/ "onion skin" periosteal reaction associated soft tissue mass
51
x-ray appearance Osteosarcoma
sunburst reaction "bone outside of bone"
52
Paraneoplastic syndromes in neuroblastoma
Opsoclonus-myoclonus-ataxia Vaso-active intestinal peptide (VIP) secretion - leading to diarrhea and hypokalemia
53
Prognosis of a child with neuroblastoma < 12-18 months?
Favourable
54
Staging of neuroblastoma
``` CT/MRI MIBG Bone scan Bone marrows Urine HVA/VMA Biopsy (for biology, n-myc) ```
55
Infant with stage 4S neuroblastoma
Skin involvement (blue spots) Hepatomegaly Bone marrow < 10% NO mets to bone May not require treatment!
56
Four risk factors for hepatoblastoma
BWS hemihypertrophy family hx of FAP prematurity
57
Tumour lysis
hyperkalemia hypocalcemia hyperphosphatemia hyperuricemia
58
Definition of hyperleukocytosis, organs that it can affect
WBC > 100 Brain (intracranial hemorrhage) Lungs Kidney
59
Management of hyperleukocytosis
Hydration! Rasburicase Platelets - at risk of bleeding (plts and FFP do not increase viscosity)
60
Patient with hyperleukocytosis. When would you transfuse pRBCs?
Only if symptomatic from anemia (heart failure) - give small amount of blood (2-5 cc/kg)
61
Oncologic emergencies
``` TLS Fever + Neutropenia Cord compression Mediastinal mass APML ```
62
Most common bug in febrile neutropenia
gram positive organisms
63
How do you manage an immunocompromised child exposed to varicella? (with no symptoms)
VZIG
64
3 side effects of vincristine
siADH constipation jaw pain
65
3 side effects of peg-asparaginase
pancreatitis anaphylaxis thrombosis
66
3 side effects of bleomycin
pulmonary fibrosis | hypersensitivity, rash
67
side effects of cyclophosphamide
hemorrhagic cystitis | infertility
68
side effects of etoposide
``` allergic reaction secondary malignancies (leukemia) ```
69
Side effects of cisplatin
N/V hearing loss nephrotoxicity
70
methotrexate side effects
renal failure mucositis leukoencephalopathy
71
late effects after neck radiation
hypothyroidism
72
poor prognosis in LCH is associated with...
bone marrow involvement (pancytopenia) hemorrhagic skin lesions hepatomegaly young age at diagnosis (< 18 mos)
73
diagnostic test for LCH?
biopsy of involved bone/skin
74
Autologous transplant - what is the principle
Give them back their own bone marrow as a "rescue"
75
Allogeneic transplant side effect
Graft versus host disease
76
Acute GVHD commonly affects what organs
Liver Gut Skin
77
RFs for bad GVHD
mismatched/unrelated donor bone marrow transplant older age
78
when does chronic GVHD occur?
> 100 days | can affect any organ!
79
What is osteopetrosis?
inherited bone disease involving dense, sclerotic bones prone to fracture Cure: BMT
80
What is Kasabach-Merritt
DIC associated with capillary hemangiomas
81
What investigation does a child with WAGR need regularly?
Abdominal ultrasound for Wilm's tumour
82
Wilm's tumour is associated with
``` NF 11p Denys-Drash Aniridia, WAGR B-W Hemihypertrophy ```
83
What predisposes to leukemia: - exposure to rads - mom or dad with leukemia - NF1
NF1
84
Two tumours that children with Beckwith-Wiedemann are at increased risk of?
hepatoblastoma | Wilms tumour
85
Retinoblastoma - increased risk of what other cancers?
Osteosarcoma | Melanoma
86
MEN2 - what types of cancers are they at risk of?
medullary thyroid cancer | pheochromocytoma
87
familial adenomatous polyposis - at risk of what type of cancers?
colorectal ca hepatoblastoma thyroid ca

Royal College Exam (32 decks)

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