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MRCPCH Clinicals > Oncology > Flashcards

Oncology Flashcards

1
Q

Which cells are affected by ALL?

A

B or T cell, but B cell is more common

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2
Q

How does ALL present?

A 
2-5 years
Insidious
Malaise, anorexia
Pallor
Lethargy
Bruising, petechiae, nose bleeds
Bone pain
Hepatosplenomegaly
Lymphadenopathy
Headache, vomiting, nerve palsies
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3
Q

What investigations should be done for suspected aLL?

A

FBC - low Hb, thrombocytopenia, blast cells
Bone marrow investigation
Chest X-ray

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4
Q

What are the poor prognostic signs in ALL?

A 
Presenting WCC >50
<2 and >9
Boys
Chromosomal abnormalities/translocations, particularly Philadelphia chromosome
Hypidiploidy
Afro-Caribbean ethnicity
CNS disease
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5
Q

How is ALL managed?

A

Remission induction
Intensification
Intrathecal chemo in some
Continuing therapy

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6
Q

How might an ALL Relapse be managed?

A

High dose chemo with total body irradiation and BMT

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7
Q

What is AML?

A

Heterogeneous group of disorders involving precursors of myeloid, monocyte, erythroid and megakaryocyte lines

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8
Q

How is AML subdivided?

A

7 types according to morphology and immunophenotyping

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9
Q

How common are chromosomal abnormalities in AML?

A

At least 80% have chromosomal abnormalities, with translocations the most common

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10
Q

How does management of AML compare to ALL?

A

Treatment is more intensive, but shorter than ALL (usually 6 months)
BMT plays a much more prominent role

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11
Q

What is the prognosis in ALL?

A

80% 5 year survival

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12
Q

What is the prognosis in AML?

A

50% 5 year survival

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13
Q

What are the immediate dangers at presentation of a leukaemia?

A 
Infection
Hyperleucocytosis/hyperviscosity
Tumour lysis syndrome
Bleeding
Obstruction from mediastinal mass
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14
Q

What are the broad types of brain tumour?

A 
Asytrocytoma (40%)
Medulloblastoma (20%)
Ependymoma (8%)
Brain stem glioma (6%)
Craniopharyngioma (4%)
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15
Q

Where do medulloblastomas come from?

A

Midline of the posterior fossa

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16
Q

How common are spinal mets at diagnosis in medulloblastoma?

A

Up 2o 20%

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17
Q

Where do ependymomas arise?

A

Mostly in posterior fossa

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18
Q

Where do craniopharyngiomas arise from?

A

Squamous remnant of Rathke pouch

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19
Q

What are the clinical features of a supratentorial tumour?

A

○ Seizures
Hemiplegia
Focal neurological signs

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20
Q

What are the clinical features of a midline brain tumour?

A

Visual field loss - bitemporal hemianopia

Pituitary failure - growth failure, diabetes insipidus, weight gain

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21
Q

What are the clinical features of a cerebellar or fourth ventricle tumour?

A

Truncal ataxia
Coordination difficulties
Abnormal eye movements

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22
Q

What are the clinical features of a brainstem tumour?

A

Cranial nerve defects
Pyramidal tract signs
Cerebellar signs e.g. ataxia
Often no raised ICP

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23
Q

Which tumours are seen in the posterior fossa?

A

Cerebellar astrocytoma
Medulloblastoma
Ependymoma

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24
Q

What is the most common tumour in children?

A

Cerebellar astrocytoma

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25
Q

What might a cerebellar astrocytoma involve?

A

Vermis, cerebellar hemispheres or both

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26
Q

What are the features of a cerebellar astrocytoma?

A

Cystic, slow growing

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27
Q

What are the features of a medulloblastoma?

A 
Highly malignant, rapidly growing
Arises from cerebellar vermis
Often causes hydrocephalus
Can metastasize along CSF pathways
Often solid
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28
Q

What is the prognosis for medulloblastoma?

A

75% 5 year survival

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29
Q

Who has a poorer prognosis in medulloblasoma?

A

Younger children

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30
Q

Where do ependymomas come from?

A

4th ventricle

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31
Q

What can an ependymoma cause?

A

Hydrocephalus

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32
Q

What is the prognosis in an ependymoma?

A

Poor, due to localisation of tumour

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33
Q

What are the features of brainstem tumours?

A 
Varies in degree of malignancy
Peak incidence 5-9 years
Presents with multiple cranial nerve palsies and long tract signs, possible vomiting
Treatment is with radiotherapy
Poor survival
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34
Q

What are the kinds of tumours seen in the supratentorial region?

A

Cerebral astrocytoma
Ependymoma
Optic glioma

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35
Q

What are the features of a cerebral astrocytoma?

A

○ Presentation depends on location, but often leads to seizures
Low grade tumours are benign and more common
High grade tumours are rarer

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36
Q

What are the features of a supratentorial ependymoma?

A

30-40% of ependymomas are supratentorial
More malignant than the infratentorial ones
Tendency to metastasize
Poor prognosis

37
Q

What are the features of an optic glioma?

A 
1/2 pre chiasmatic, 2/3 post chiasmatic
Generally pilocytic astrocytomas
1/4 occur with NF type 1
Pre chiasmatic: proptosis, visual loss (late)
Post chiasmatic: visual loss
38
Q

What are the clinical features of craniopharyngiomas?

A

Endocrine:
Delayed growth
Hypothyroidism
Diabetes insipidus

Raised ICP:
Headache
Ataxia

Local features
Bitemporal hemianopia
Depressed consciousness
Vomiting
Nystagmus
39
Q

Which tumours are associated with posterior fossa syndrome and when?

A

Medulloblastoma - 12-48 hours postoperatively

40
Q

What are the cardinal features of posterior fossa syndrome?

A

Hemiparesis
Mutism
Cerebellar dysfunction
Supranuclear cranial nerve palsy

41
Q

In how many patients does posterior fossa syndrome persist?

A

50%

42
Q

Which groups get non-Hodgkin lymphoma vs Hodgkin?

A

Non-Hodgkin is more common in childhood, and Hodgkin is more frequently seen in adolescence.

43
Q

How does Hodgkin lymphoma present?

A

Painless lymphadenopathy, usually in the neck
Lymph nodes are larger and firmer than benign ones
Often a long clinical history
‘B’ symptoms are uncommon but include sweating, pruritus, weight loss, fever

44
Q

How should a Hodgkin lymphoma be investigated?

A

Lymph node and bone marrow biopsy

45
Q

What is the prognosis for Hodgkin lymphoma?

A

> 80% can be cured, 60% with disseminated disease

46
Q

How does a non-Hodgkin lymphoma present?

A

Abdominal mass - usually B cell disease
Mediastinal mass in T cell
Can cause SVC obstruction
Head and neck masses - no specific cell

47
Q

What is the prognosis for non-Hodgkin lymphoma?

A

> 80% in T and B cell disease

48
Q

What is the pathophysiology of tumour lysis syndrome?

A

Occurs with bulky disease e.g. high count ALL, B cell NHL; undergoes lysis with treatment, resulting in the intracellular contents of potassium, phosphate and nuclear debris being released into the circulation
Uric acid crystals and phosphate precipite out with calcium into crystals.

49
Q

What are the biochemical and clinical features of tumour lysis syndrome?

A

Fluid overload
High phosphate, potassium, urea and creatinine
Hypocalcaemia

50
Q

How is tumour lysis syndrome treated?

A

Hyperhydration
Uric acid lowering agents e.g. urate oxidase or allopurinol
Treatment of hyperkalaemia
Consideration of fluid filtration or dialysis

51
Q

Where does a neuroblastoma arise from?

A

Arises from neural crest tissue in the adrenal medulla and sympathetic nervous system

52
Q

Who gets neuroblastoma?

A

Mostly under 5 years

53
Q

How does neuroblastoma present?

A

Abdominal mass most commonly, but primary tumour can be anywhere along the sympathetic chain from neck to pelvis
Can cross the midline, enveloping major blood vessels and lymph nodes
May have paravertebral tumours causing spinal cord compression
Over 2 years, symptoms are mostly from metastatic disease: bone pain, bone marrow suppression causing weight loss, malaise

54
Q

What investigations should be done in neuroblastoma?

A

Raised urinary catecholamines
Biopsy
Bone marrow sampling
MIBG scan

55
Q

How is a neuroblastoma managed?

A

Surgery
Chemotherapy - may need high dose therapy with autologous stem cell rescue
Radiotherapy

56
Q

What is the prognosis for neuroblastoma and what affects this?

A

Most children over 1 present with advanced disease and have a poor prognosis
Overexpression of N-myc, evidence of deletion on chromosome 1 and gain of material on chromosome 17q in tumour cells are associated with poorer prognosis
Risk of relapse is high
Cure rate is about 30%

57
Q

Where does a Wilms tumour originate from?

A

Embryonal renal tissue

58
Q

How does a Wilms tumour present?

A

Over 80% are under 5; very rare over 10 years
Large abdominal mass, often found incidentally in an otherwise well child
Haematuria
Hypertension

59
Q

How is a Wilms tumour managed?

A

○ Chemotherapy
Delayed nephrectomy
Radiotherapy for more advanced disease

60
Q

What is the prognosis in Wilms tumour?

A

> 80% cure rate

61
Q

What is Wilms tumour also known as?

A

Nephroblastoma

62
Q

What is the commonest soft tissue sarcoma in childhood?

A

Rhabdomyosarcoma

63
Q

Where does a rhabdomyosarcoma originate from?

A

Primitive mesenchymal tissue

64
Q

How does a rhabdomyosarcoma present?

A

Head and neck are the site in 40%:
Proptosis
Nasal obstruction
Bloodstained nasal discharge

Genitourinary:
Dysuria
Urinary obstruction
Scrotal mass
Bloodstained vaginal discharge

Metastatic disease
Present in approx 15% at diagnosis

65
Q

What is the cure rate for rhabdomyosarcoma?

A

About 65%

66
Q

What is langerhans cell histiocytosis?

A

Clonal accumulation and proliferation of abnormal bone marrow-derived Langerhans cells
These cells, functioning as potent antigen-presenting cells, along with white cells cause inflammatory tissue damage

67
Q

What is the prognosis for langerhans cell histiocytosis?

A

Very variable - spontaneous regression to death

68
Q

How is langerhans cell histiocytosis managed?

A

Corticosteroids
Vinblastine
Methotrexate
6-mercaptopurine

69
Q

What is haemophagocytic lymphohistiocytosis?

A

Rare disease caused by abnormal proliferation of histiocytes in tissues and organs, causing an uncontrolled and ineffective immune response with high fatality rate

70
Q

How is familial haemophagocytic lymphohistiocytosis inherited?

A

AR

71
Q

What is the defect in familial haemophagocytic lymphohistiocytosis

A

5 genetic mutations which cause a defect in NK and T cell cytotoxic function, leading ot strong immunological activation of phagocytes and inflammatory mediators

72
Q

How is haemophagocytic lymphohistiocytosis treated?

A

○ Active treatment with corticosteroids and chemotherapy first
Definitive treatment is bone marrow transplant

73
Q

What is secondary haemophagocytic lymphohistiocytosis also known as?

A

Macrophage activating syndrome

74
Q

What can precipitate haemophagocytic lymphohistiocytosis?

A

Infection, rheumatological, immune deficiencies, malignant and metabolic disorders

75
Q

What are the typical findings in haemophagocytic lymphohistiocytosis?

A 
Fever
Hepatosplenomegaly
Cytopenia
Hypertriglyceridaemia
Coagulopathy
Hypofibrogenaemia
Elevated soluble CD25
Liver dysfunction
Elevated ferritin
Neurological symptoms
76
Q

Who doesn’t get bone tumours?

A

Pre pubertal children

77
Q

What is the most common kind of bone tumour?

A

Osteogenic sarcoma

78
Q

Which group is Ewing sarcoma more common in ?

A

Younger children

79
Q

Where does osteogenic sarcoma often occur?

A

Predominantly in metaphyses of long bones
0% occurring at the knee joint
Commonly leads to lung metastasis

80
Q

Where does Ewing sarcoma commonly occur?

A

More often in flat bones

Can be extra osseous rarely

81
Q

What are the clinical features of bone tumours?

A

Limbs are the most common site
Persistent localised bone pain
At diagnosis, most are otherwise well

82
Q

What investigations should be done if there is a suspected bone tumour?

A

Plain XR followed by MRI And bone scan
In Ewing sarcoma, there is often a substantial soft tissue mass
Chest CT to look for lung mets
Bone marrow sampling to exclude involvement

83
Q

How are bone tumours managed?

A

○ Combination chemotherapy before surgery

Radiotherapy in Ewing sacoma

84
Q

What is a retinoblastoma?

A

Malignant tumour of retinal cells

85
Q

How is retinoblastoma inherited?

A

All bilateral tumours are hereditary, and 20% of unilateral cases
Gene is on chromosome 13q14
Dominant inheritance with incomplete penetrance

86
Q

How does retinoblastoma present?

A

Usually within the first 3 years; children with hereditary form should be screened regularly from birth
White pupillary reflex instead of red
Squint

87
Q

How is retinoblastoma managed?

A

Chemotherapy to shrink the tumours
Local laser treatment to the retina
Enucleation may be needed in more advanced disease, or radiotherapy

88
Q

What is the prognosis in retinoblastoma?

A

Most patients are cured, but there is a significant risk of secondary malignancy among survivors of hereditary retinoblastoma

MRCPCH Clinicals (17 decks)

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