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YR5 Specialties > Paeds malignancy > Flashcards

Paeds malignancy Flashcards

1
Q

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

A

Astrocytoma (pilocystic astrocytoma = most common)

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2
Q

Where are astrocytomas found?

A

Cerebellum

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3
Q

Where are medulloblastomas found?

A

Cerebellum

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4
Q

Where are ependymomas found?

A

4th Ventricle

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5
Q

What is the WHO grade for pilocystic astrocytoma?

A

Grade I

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6
Q

What syndrome is associated with pilocystic astrocytoma?

A

Neurofibromatosis 1

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7
Q

How will pilocystic astrocytoma appear on MRI?

A

Cerebellar
Well-circumscribed
Cystic
Enhancing

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8
Q

What mutation is most common in pilocystic astrocytoma?

A

BRAF

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9
Q

Recall the 7 signs and symptoms of pilocystic astrocytoma

A

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

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10
Q

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

A

Normal exam
14y/o
High BMI
Normal MRI
Papilloedema

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11
Q

Which investigation is most appropriate in investigating an astrocytoma?

A

MRI

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12
Q

What histopathological feature characterises pilocystic astrocytomas?

A

Rosenthal fibres: corkscrew eosinophilic fibre bundles

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13
Q

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

A

CLIC Sargent (cancer + leukaemia in children social worker)

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14
Q

What is the most common malignant brain tumour in children?

A

Medulloblastoma

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15
Q

What is the first-line management for astrocytoma?

A

Surgery

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16
Q

Which CNS tumours can be treated with radiotherapy?

A

Gliomas + metastases

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17
Q

Which CNS tumours can be treated with chemotherapy?

A

High grade gliomas

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18
Q

What % of ALL is B lineage vs T lineage?

A

85% B
15% T

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19
Q

What % of leukaemia is ALL vs AML in children?

A

80% ALL
20% AML

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20
Q

Recall the signs and symptoms of ALL

A

BM failure (anaemia, thrombocytopaenia, neutropaenia)

Local infiltration: lymphadenopathy, hepatosplenomegaly, bone pain, testes/ CNS

Leukaemia cutis = petechial rash on face + trunk

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21
Q

What is raised in tumour lysis syndrome?

A

Potassium
LDH
Phosphate
Uric acid

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22
Q

What may be seen on CXR in ALL?

A

Enlarged thymus (Mediastinal mass)

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23
Q

What investigations are used for ALL?

A

FBC: Thrombocytopenia, Anaemia, abnormal WCC
CRP
Coagulation profile
U+Es: TLS

Peripheral blood smear: >20% blasts

BMA: >20% blasts

Immunophenotyping using flow cytology

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24
Q

What results of BM biopsy would be diagnostic of ALL?

A

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

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25
Q

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

A

TLS needs to be reduced: allopurinol + hyperhydration

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26
Q

How many years of chemotherapy are necessary in ALL?

A

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

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27
Q

What therapies can be given alongside chemotherapy in ALL?

A

CNS-directed therapy
Molecular tx
Transplantation

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28
Q

What types of molecular treatment are available for ALL?

A

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

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29
Q

What age group are NHL/HL most common in?

A

NHL = childhood
HL = adolescence

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30
Q

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

A

Hodgkin’s lymphoma

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31
Q

Compare the spread of HL vs NHL

A

HL = spreads contiguously to adjacent LNs
NHL involves multiple sites + spreads sporadically

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32
Q

Recall the signs and symptoms of HL

A

Painless cervical lymphadenopathy
Painful on drinking alcohol (in 10%)

Mediastinal mass

B Sx uncommon, even in advanced disease

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33
Q

Recall the investigations and results that are standard for HL

A

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

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34
Q

How should HL be managed?

A

Combination chemotherapy (ABVD) + radiotherapy

35
Q

Recall the chemotherapy regime used in paediatric HL

A

ABVD
Adriamycin
Bleomycin
Vincristine
Dacarbazine

36
Q

Recall the signs and symptoms of NHL

A

Painless lymphadenopathy +/- compression syndrome

Extra-nodal involvement

B Sx indicate worse prognosis: fever, weight loss, night sweats

37
Q

Recall the useful investigations and their results in NHL

A

LN/BM biopsy for cytology, histology + immunophenotyping
PDG-PET/ CT staging: Ann Arbor
Bloods: FBC, ESR, LFTs, LDH, Alb (prognostic)

38
Q

Recall the management approach dependent on the type of NHL

A
  1. Urgent chemo
  2. Monitor only
  3. Abx eradication: H pylori gastric MALToma
39
Q

What chemotherapy regime is used in NHL?

A

R-CHOP
Rituximab
Cyclophosphamide
H: Doxorubicin
O: Vincristine
Prednisolone

40
Q

What is Burkitt’s lymphoma a type of?

A

B cell NHL

41
Q

What are the different variants of burkitt’s lymphoma - and what are their different causes?

A

Endemic (EBV)
Sporadic (EBV)
Immunodeficiency (HIV)

42
Q

Why is endemic Burkitt’s so common in Africa?

A

Chronic malaria reduced EBV resistance

43
Q

What part of the body does Burkitt’s lymphoma affect?

A

JAW/ facial bones

44
Q

What type of cell does burkitt’s lymphoma arise from?

A

Germinal centre cells

45
Q

What is the histopathological appearance of burkitt’s lymphoma?

A

Starry sky

46
Q

What translocation is most common in burkitt’s lymphoma?

47
Q

What is the prognosis for burkitt’s lymphoma?

A

Really bad as fastest growing human tumour known

48
Q

What is the most common primary bone cancer in adolescents?

A

Osteosarcoma

49
Q

Where does osteosarcoma tend to occur?

A

Long bones: 60-75% in the knee

50
Q

Recall 3 signs and symptoms of osteosarcoma

A

Pain: worse at night
Mass/ swelling- tender
Restricted movement/ limp

51
Q

Give 2 genetic abnormalities give predisposition to osteosarcoma

A

RB1 abnormality (Retinoblastoma)
Li-Fraumeni syndrome (TP53 tumour suppressor gene mutation)

52
Q

Where does osteosarcoma tend to metastasise to?

53
Q

What will be shows on XR in osteosarcoma?

A

Soft tissue calcification: sunburst appearance
Elevated periosteum = Codman’s triangle

Normal XR: does NOT r/o osteosarcoma

54
Q

What investigations should be done following XR in suspected osteosarcoma?

A

MRI
Biopsy: definitive dx

55
Q

How should osteosarcoma be managed?

A

Specialised sarcoma team
Surgery + chemo
Post-treatment OT/PT/ orthotics etc

56
Q

What is the surgical management of osteosarcoma?

A

Limb-sparing surgery +/- amputation

57
Q

What is the prognosis for osteosarcoma?

A

Poor: 60% 5y survival

58
Q

How does Ewing’s sarcoma appear under the microscope?

A

Small round blue cell tumour

59
Q

What is the median age of Ewing’s sarcoma development?

60
Q

Where does Ewing’s sarcoma develop?

A

Femur
Tibia
Fibula
Humerus
Ileum of pelvis

61
Q

What translocation is associated with Ewing’s sarcoma?

62
Q

What are the signs and symptoms of Ewing’s sarcoma?

A

Bone pain: often worse at night
Mass
Restricted movement of joint
Constitutional Sx: fatigue, fever, weight loss

63
Q

What investigations are appropriate to investigate Ewing’s sarcoma?

A

XR

CT/PET/MRI

Biopsy: definitive dx

64
Q

What would be seen on XR in Ewing’s sarcoma?

A

Bone destruction with overlying onion-skin layers of periosteal bone formation

65
Q

What is the main management of Ewing sarcoma?

A

Surgery + VIDE chemotherapy + radiotherapy

66
Q

What management should be given post-treatment for osteosarcoma?

A

OT, PT, dietician, orthotics/ prosthetics

67
Q

Which cells have undergone malignant transformation in retinoblastoma?

A

Retinal cells

68
Q

How does retinoblastoma often present?

A

Leukocoria <3y

69
Q

What is the main difference in presentation between hereditary and spontaneous retinoblastoma?

A

Hereditary is nearly always bilateral
Spontaneous is unilateral

70
Q

Recall the pattern of inheritance of retinoblastoma

A

Autosomal dominant mutation on chr13
RB1: Tumour suppressor gene inactivation

71
Q

Recall the 2 key signs and symptoms of retinoblastoma

A

Negative red reflex: white pupillary reflex (Leukocoria)
Strabismus (Squint)

72
Q

Recall 2 investigations in retinoblastoma

A

MRI + EUA (examination under anaesthetic)

73
Q

How should retinoblastoma be treated?

A

Minimal/ no seeding present: systemic chemo + focal therapy

Laser therapy
External beam radiotherapy

Gross seeding (tumour cells floating in vitreous cavity):
Enucleation (eye removal) + Chemo

74
Q

What is the average age of diagnosis of retinoblastoma?

75
Q

What is the prognosis in retinoblastoma?

A

90% cured + survive into adulthood

Many will be visually imparted

Significant risk of 2nd malignancy (esp. sarcoma) among survivors of hereditary retinoblastoma

76
Q

What type of tissue and where does neuroblastoma arise?

A

Neural crest tissue in adrenal medulla + SNS

77
Q

What is a benign tumour of the same tissue as neuroblastoma originates from called?

A

Ganglioneuroma

78
Q

Which gene is associated with a poor prognosis for neuroblastoma?

79
Q

Where is the mass most likely to be found in neuroblastoma?

80
Q

Recall 5 symptoms of neuroblastoma

A

Abdo distension
Fatigue
WL
Sweating, agitation
Bone pain (mets)

81
Q

What additional symptoms of neuroblastoma are likely in people under 2 years?

A

Sx of metastases (poorer prognosis in under 1s)

82
Q

What urinary abnormality may be seen in neuroblastoma?

A

Urinary catecholamine metabolites eg VMA/HVA

83
Q

How should neuroblastoma be managed?

A

In very young infants spontaneous regression can occur
If localised primaries with no mets: surgery alone
If metastatic: chemotherapy + radio (with autologous stem cell rescue) + surgery

YR5 Specialties (27 decks)

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