Paeds malignancy, haem, derm Flashcards Preview

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Flashcards in Paeds malignancy, haem, derm Deck (245)
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1

What type of tumour are the majority of CNS tumours in children?

Astrocytoma (pilocystic astrocytoma = most common)

2

Where are astrocytomas found?

Cerebellum

3

Where are medulloblastomas found?

Cerebellum

4

Where are ependymomas found?

Posterior fossa

5

What is the WHO grade for pilocystic astrocytoma?

Grade I

6

What syndrome is associated with pilocystic astrocytoma?

Neurofibromatosis 1

7

How will pilocystic astrocytoma appear on MRI?

Cerebellar, well-circumscribed, cystic and enhancing

8

What mutation is most common in pilocystic astrocytoma?

BRAF

9

Recall the signs and symptoms of pilocystic astrocytoma

Headaches (worst in morning)
Gait problems, co-ordination problems
Visual changes
Vomiting on waking
Failure to thrive
Behaviour/ personality change
Later sign = papilloedema due to raised ICP

10

What is the typical picture of benign intracranial HTN (not malignant cause)?

Normal MRI, normal exam, papilloedema, 14y/o, high BMI

11

Which investigation is most appropriate in investigating an astrocytoma?

MRI

12

What is the specialised member of the MDT in childhood cancer cases?

CLIC Sargent (cancer and leukaemia in children social worker)

13

What is the first-line management for astrocytoma?

Surgery

14

Which CNS tumours can be treated with radiotherapy?

Gliomas and metastases

15

Which CNS tumours can be treated with chemotherapy?

High grade gliomas

16

What % of ALL is B lineage vs T lineage?

85% B, 15% T

17

What % of leukaemia is ALL vs AML in children?

80% ALL, 20% AML

18

Recall the signs and symptoms of ALL

BM failure (anaemia, thrombocytopaenia, neutropaenia)
Local infiltration --> lymphadenopathy, hepatosplenomegaly, bone pain, testes/CNS
Leukaemia cutis = petechial rash on face + trunk

19

What is raised in tumour lysis syndrome?

Potassium, LDH, phosphate, uric acid

20

What will be seen on CXR in ALL?

Enlarged thymus

21

What results of BM biopsy would be diagnostic of ALL?

>20% blasts in BM/ peripheral blood
Immunological and cytological characteristics

22

What should be the immediate management of a high WCC in ALL?

TLS needs to be reduced: allopurinol and hyperhydration

23

How many years of chemotherapy are necessary in ALL?

2-3 years
Boys are treated for longer because testes are a site of accumulation of lymphoblasts

24

What therapies can be given alongside chemotherapy in ALL?

CNS-directed therapy (if LP is negative initially)
Molecular treatment
Transplantation

25

What types of molecular treatment are available for ALL?

Imatinib (TK inhibitor) for Ph +ve cases
Rituximab (monoclonal antibodies against CD20 for B cell depletion)

26

What age group are NHL/HL most common in?

NHL = childhood
HL = adolescence

27

Which of NHL/HL is more likely to be localised to one nodal site?

Hodgkin's lymphoma

28

Compare the spread of HL vs NHL

HL = spreads contiguously to adjacent lymph nodes
NHL involves multiple sites and spreads sporadically

29

Recall the signs and symptoms of HL

Painless cervical lymphadenopathy
B symptoms are uncommon, even in advanced disease
Painful on drinking alcohol (in 10%)

30

Recall the investigations and results that are standard for HL

LN biopsy: Reed Sternberg cells
PDG-PET/ CT staging: Ann Arbor
Bloods: FBC, ESR, LFTs, LDH, Alb (prognostic markers)
Immunophenotyping (CD30, CD15 - diagnostic markers)