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Flashcards in Oncology Deck (87)
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1

Who develops transient myeloproliferative disorder?

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

2

What is transient myeloproliferative disorder?

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

3

What genetic mutation is transient myeloproliferative disorder associated with?

GATA 1

4

How does rasburicase work?

Urate oxidase --> promotes degradation of uric acid.

5

Langerhans cell histiocytosis - what is this?

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

6

What are the most common adolescent breast lesions?

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

7

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

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)

8

5 childhood conditions that predispose to developing leukemia

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

9

What type of leukemias present in childhood?

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

10

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

80%

11

x-ray findings in bony leukemia involvement

-transverse radiolucent metaphyseal growth arrest lines
-periosteal elevation with reactive subperiosteal cortical thickening
-osteolytic lesions
-diffuse osteopenia/ osteoporosis

12

Other risk factors for leukemia (non-genetic conditions)

previous chemotherapy, radiation (including in utero), benzene

13

Prognosis of all

>90%

14

prognosis of AML

60%

15

ALL - poor prognostic factors

- 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)






16

What brain tumour do patients with tuberous sclerosis develop?

15% develop subependymal giant astrocytomas (SEGA)

17

What brain tumour do patients with tuberous sclerosis develop?

15% develop subependymal giant astrocytomas (SEGA)

18

What brain tumour can patients with NF1 develop?

optic nerve glioma

19

Risk for pediatric renal medullary carcinoma?

Sickle cell trait!

20

How many children with leukemia will not have circulating blasts?

20%

21

ALL vs JIA - 3 factors that predict ALL

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

(sensitivity 100% specificity 85%)

22

Poor prognosis in ALL is suggested by

Age < 1 yr, > 10yr

23

7 childhood conditions predisposing to leukemia

T21
NF1
Ataxia-telangiectasia
Fanconi
Bloom
Noonan
Li-Fraumeni

24

Most common type of childhood leukemia

ALL (80%)

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