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Peadiatrics > Malignant Disease > Flashcards

Malignant Disease Flashcards

1
Q

What are the important causative factors in malignant disease

A

Genetics, infections, environmental

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2
Q

What effect on genes causes cancer? Eg of some diseases?

A

Activation of oncogenes & loss of tumour suppressor

retinoblastoma, Down’s syndrome, Ataxia-telangiectasia, Li-Fraumeni

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3
Q

Which two infections can lead to increased risk of malignancy? What malignancy?

A

Epstein Barr virus - transforms B cells -> Burkitt’s lymphoma
HIV - targets T helper (CD4) cells -> susceptibility to lymphoid malignancies

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4
Q

Important environmental risk factor for malignancy

A

Previous treatment for malignancy in a child

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5
Q

Localised mass in Abdo? Thorax? Soft tissue ? ….makes you think of ?

A

A- wilm’s tumour, neuroblastoma
T- non-hodgkins lymphoma
S- rhabdomyosarcomas

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6
Q

Which malignancy if constitutional symptoms ? What are constitutional Sx?

A

Weight loss, night sweats, fever

Lymphoma

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7
Q

Which malignancy if bone marrow failure? Bone pain?

A

Acute leukaemia

Bon tumours , leukaemia

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8
Q

Give some egs of consequences of mass effect?

A

Air way obstruction due to enlarged lymph nodes

Increased ICP due to CNS tumours

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9
Q

Malignancy investigations

A

Histology - biopsy / bone marrow aspiration
Imaging - USS, X-ray, MRI
Tumour markers (in some)

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10
Q

Which tumours do you not take a biopsy from?

A

CNS

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11
Q

Egs of some tumour markers

A

A-fetoprotein (in liver Ca)

Increased urinary catecholamines (in neuroblastoma)

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12
Q

What is the most common childhood malignancy ? What characterises it?

A

Leukaemias

proliferation of immature WBCs

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13
Q

What is the commonest childhood leukaemia? Which only affects adults?

A

Acute - 97%

CML - rare, CLL only adults

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14
Q

Which cells are included in the ALL lineage of leukaemia ? % of leukaemia ?

A

Lymphoid
T cells, B cells, natural killer cells
80%

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15
Q

Which cells are in the AML cells of leukaemia

A

Granulocytic / macrocytic

Eosinophils, basophils, monocytes, platelets, RBCs

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16
Q

Usual onset? Features of leukaemia and causes?

A

Insidious with pallor & malaise (anaemia); haemorrhagic diathesis (purpura, easy brusing, epistaxis - thrombocytopenia) ; hepatosplenomegaly / lymphadenopathy (macrocyte infiltration) ; bone pain (marrow cavity expansion); infections (neutropenia)

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17
Q

Investigations for leukaemia

A 
FBC - anaemia, thrombocytopenia, WBCs variable
Blood film - *blast cells*
*bone marrow aspirate* 
(Chest X-ray - ?mediastinal mass
Serum chemistry 
LP)
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18
Q

Management of ALL?

A

Chemotherapy (5 phases)

Haemopoietic stem cell transplantation

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19
Q

What are the 5 phases of chemo for ALL

A

Induction (3-5 drugs to decrease tumour load)
Interim management
Consolidation (continued systemic therapy after remission)
Intensification
Maintenance (for 2 years)

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20
Q

When does haemopoietic stem cell transplantation get used?

A

Only in high risk cases in 1st remission / relapse

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21
Q

What drug is used to protect kidneys from what in ALL management?

A

Allopurinol protects kidneys from

Effects of rapid cell lysis

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22
Q

Which has worst prognosis ALL/ AML?

A

AML - more intensive treatment

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23
Q

What is associated strongly with AML

A

80% have chromosomal abnormalities eg. Downs

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24
Q

What are lymphomas? 2 types and their usual ages?

A

Malignancies of the cells of the immune system
Non-hodgkins (young children)
Hodgkins (adolescents & young adults)

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25
Q

Predominant site of disease in NHL? What do the clinical features reflect?

A

Lymph nodes

Pattern of normal lymphoid cell migration

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26
Q

Features of NHL depending on what is affected?

A

T- cell malignancies - present as ALL or NHL usually with a mediastinal mass (+/- pleural effusion)
B-cell malignancies - NHL with localised lymph node disease (head/neck/abdo)
Abdominal disease - pain (+/- palpable mass) - usually advanced B cell

27
Q

Treatment of NHL

A

Combination of chemo / radio

+/- surgical debunking for abdominal tumours

28
Q

What is SVC syndrome?

A 
Complication of (usually) a tumour compressing SVC
-> oedema of face and arms
29
Q

Complication of therapy for lymphomas / Leukaemia ? What is it?

A

Tumour lysis syndrome

Due to release of cellular contents into blood stream when cells die

30
Q

What changes in blood are characteristic for tumour lysis syndrome

A

Hyperkalaemia
Hypocalcaemia
Hyperphosphataemia
Hyperuricaemia

31
Q

Where are most CNS tumours

A

60% infratentorial

32
Q

Presentation of CNS tumours

A 
Most have general features of raised ICP, but may get focal signs 
Vomiting (esp. Early morning)
Headache ( worse lying down / early morning)
Papilloedema 
Strabismus (VI nerve palsy)
Nystagmus
Ataxia
Behavioural / personality change
33
Q

What causes strabismus

A

Paralysed lateral rectus -> eye drawn in nasally

34
Q

Investigations for CNS tumours

A

MRI
LP - Not advised if raised ICP
Biopsy (not always possible eg. Brainstem tumour)

35
Q

2 types of CNS tumours and egs.

A

Astrocytic (eg high grade (supratentorial), low grade (Cerebellar), brainstem glioma)

Neuroepithelial eg. Primitive neuroectodermal tumours (PNET) (including Cerebellar medulloblastoma)

36
Q

Commonest brain tumour ? Grade / growth / age usually?

A

Astrocytomas 40%

Cerebellar astrocytomas, low grade, slow growing cystic gliomas in 6-9 year olds

37
Q

Presentation of astrocytomas

A

Headache, vommiting (+- pappilodema) - obstructive hydrocephalus
Cerebellar signs - ataxia, nystagmus, uncoordination
Diplopia, strabismus ( CN VI palsy)

38
Q

What are primitive neuroectodermal tumours also called? % of brain tumours? Which age commonest?

A

Medulloblastoma, 20%

Most common malignant brain tumour of childhood - peak in boys 2-6

39
Q

Usual location of medulloblastoma

A

Midline & invade 4th ventricle & Cerebellar hemispheres

40
Q

What is often found at diagnosis of medulloblastoma

A

Spinal mets in 20%

41
Q

Presentation of medulloblastoma

A

Vomiting, headache, Ataxia

42
Q

Treatment of medulloblastoma

A

Surgical excision + adjuvant radiotherapy of whole of CNS

Chemo in +++ risk

43
Q

What % of CNS tumours are craniopharyngiomas? What do they arise from?

A

4%

squamous remnant of Rathke’s pouch

44
Q

Presentation of craniopharyngiomas ? Why /how are they easily seen?

A

Visual field loss (optic chiasm compression), pituitary dysfunction (DI / growth failure)
calcified and so visible on skull radiographs

45
Q

Treatment of craniopharyngiomas ? Complication?

A

Surgical excision - good

Risk of pan hypopituitarism / visual impairment

46
Q

What is a neuroblastoma ? Usual age?

A

Malignancy of neural crest tissue in the adrenal medulla & sympathetic nervous system

47
Q

Features of neuroblastoma depend on location, but give some anyway?

A

Abdo mass - most common (firm non tender)
Systemic signs - pallor, weight loss, bone pain
Unilateral proptosis - pre orbital swelling & eccymosis due to eye mets
Mediastinal mass on chest X-ray

48
Q

Diagnosis of neuroblastoma

A

Increased urinary catecholamines (vanillymandelic acid, hormovanillic acid)
Biopsy (usually possible) & MIBG scan

49
Q

What happens in MIBG scan

A

Radio labeled tumour specific agent

50
Q

Treatment of neuroblastoma

A

Surgical resection, chemotherapy, irradiation

51
Q

What gives a poorer prognosis in neuroblasoma

A

Over expression of N-myc oncogene (arise being older & metastatic disease)

52
Q

What’s the proper name for Wilms’ tumour ? What tissue is it from? Usual age? And where?

A

Nephroblastoma
Embryonal renal tissues of metanephros
Rare >5 years \unilateral in 95%

53
Q

Features of nephroblastoma

A

Most common is asymptomatic abdominal mass that doesn’t cross the midline
Can get abdo pain, heamaturia, hypertension (25% cases)
anirida (absent iris)

54
Q

Diagnosis of Wilms tumour ? Where do mets commonly go?

A

Made from characteristic appearance on CT (intrinsic renal mass with mixed solid & cystic densities)
Lungs and liver

55
Q

Treatment of nephroblastoma

A

Surgical resection of primary tumour

Chemo according to stage of disease

56
Q

Most important soft tissue sarcoma ? Where do they arise from?

A

Rhabdomyosarcomas

Primitive mesenchyme

57
Q

Soft tissue Sarcoma location and effects

A

Head & neck - proptosis, nasal obstruction

GU - obstruction , dysuria

58
Q

When are bone tumours most common? What % of childhood cancer ?

A

Uncommon before puberty

4%

59
Q

2 types of bone tumours

A

Osteogenic sarcoma

Ewing’s sarcoma

60
Q

What is an osteogenic sarcoma? What age? Features?

A

Tumour of bone producing mesenchyme
Older children
Local pain & swelling

61
Q

Where is the most common place for osteogenic sarcoma ? Diagnosis? Treatment?

A

50% around knee joint in the metaphysis of the distal femur / proximal tibia
X-ray shows sunburst appearance - tumour breaks through cortex & spiralates as new bone forms
Surgery

62
Q

Age of Ewing’s sarcoma? Tissue origin? Where does it affect?

A

Younger children , less common then osteogenic, Unknown tissue origin
Usually affects long bones (mid-proximal femur) but does affect flat ones eg pelvis

63
Q

Features of Ewing’s ? Diagnosis? Treatment?

A

Pain and localised swelling are usual presenting complaints
X-ray shoes destructive lesion with periosteal elevation or a soft tissue mass (onion skin appearance )

Radio + chemo

64
Q

What is the cause of a absent red reflex in neonate ? Cause? How often is it bilateral and what does this mean?

A

Retinoblastoma
Deletion of RB gene on chromosome 13
40% - all bilateral are hereditary

Peadiatrics (14 decks)

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