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3a > Paed:Malignancy > Flashcards

Paed:Malignancy Flashcards

1
Q

What is the most common malignancy in children?

A

Leukaemia followed by brain tumours

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2
Q

What are the general clinical presentations of malignancy in a child?

A
  • localised mass (e.g. lymphadenopathy, ,organomegaly, soft tissue or body mass)
  • consequences of disseminated disease (e.g. bone marrow infiltration causing systemic ill-health)
  • consequences of pressure from a mass on local structures or tissue (airway obstruction secondary to enlarged lymph nodes in mediastinum)
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3
Q

What investigations are carried out if suspecting malignancy?

A

Radiology: xray, US, CT, MRI
Pathoogy: bone marrow biopsy/aspiration, tumour biopsy
Tumour marker studies:
- increased urinary catecholamine creation can confirm neuroblastoma
- increased alpha-fetoprotein can confirm germ cell tumour and liver tumour

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4
Q

What is the general management for cancer in children?

A

Chemo, radio, surgery, high-dose therapy with bone marrow rescue

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5
Q

What are the different types of leukaemia and their prevelances in children?

A

ALL 80%
AML 15%
CML 5%

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6
Q

What is the clinical presentation of leukaemia?

A 
2-5years old
General: malaise, anorexia, fever
Bone marrow infiltration causes:
- anaemia: pallor, lethargy
- neutropenia: infection
- thrombocytopenia: bruising, petechiae, nose bleeds and bone pain

Reticulo-endotehlia infiltration causes:

  • hepatosplenomegaly
  • lymphadenopathy
  • superior mediastinal obstruction

Other organ infiltration:

  • CNS: headaches, vomiting, nerve palsies
  • Testes: testicular enlargement
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7
Q

What investigations are done in leukaemia?

A

FBC: low Hb + thrombocytopenia + evidence of circulating leukaemia blast cells
Bone marrow exam: confirm diagnosis and identify immunological + cryogenic characteristics
CXR: mediastinal mass characteristic of T-cell disease
Lumbar puncture

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8
Q

What are the 5 phases of chemo in leukaemia?

A

1) induction
2) consolidation + CNS protection
3) interim maintenance
4) delayed intensification
5) continuing maintenance

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9
Q

When is a haemopoietic cell transplantation used?

A

For high risk leukaemia pt in first remission or relapsed pt

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10
Q

What is the predominant type of brain tumour?

A

Infratentorial involving cerebellum, midbrain, brainstem

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11
Q

What are the different types of brain tumours?

A 
Astrocytoma (40%)
Medulloblastoma (20%)
Ependymoma (8%)
Brainstem glioma (6%)
Craniopharyngioma (4%)
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12
Q

What is a craniopharyngioma?

A

A development tumour arising from the squamous remnant of the Rathke much. Not truly malignant but locally invasive and grows slowly in the suprasellar region.

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13
Q

What are clinical features of brain tumours?

A 
Raised ICP Sx
- nystagmus, ataxia, early morn headache worse on lying down, vomiting, papillodema, squint, personality/behavioural change
Focal neurological signs
Back pain
Peripheral weakness of arms and legs
Bladder/bowel dysfunction
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14
Q

What investigations are done for a brain tumour?

A

MRI scan
Magnetic resonance spectroscopy
Lumbar puncture (only with neurosurgical advice if suspicion of raised ICP)

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15
Q

Mx of brain tumour

A

Surgery: treat hydrocephalus, provide tissue diagnosis and attempt maximum reception
–> resection + VP shunt

Chemo + radio

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16
Q

What is lymphoma?

A

malignancy of the cells of the immune system

  • non-hodgkins more common in childhood
  • Hodgkins in adolescence
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17
Q

Clinical features of Hodgkins lymphoma

A

painless lymphadenopathy in neck

lymph nodes may cause airway obstruction

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18
Q

investigations for lymphoma

A

lymph node biopsy

radiological assessment of all nodal sites + bone marrow biopsy

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19
Q

mx of lymphoma

A

combo chemo + radio

PET scanning for monitoring

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20
Q

Causes of lymphadenopathy?

A

HIV infetion
Autoimmune conditions
Storage disorders
Malignancy

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21
Q

Clinical presentation of non-hodgkins lymphoma

A

Localised lymph node disease in head, neck or abdomen

Abdo disease: pain from intestinal obstruction, palpable mass + intersussception

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22
Q

What is a neuroblastoma?

A

Neural crest tube tumour arising from sympathetic tissue of the adrenal medulla
Median age of onset: 20 months

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23
Q

Clinical features of neuroblastoma?

A

Abdo mas, neck mass, chest mass
- pallor, weight loss, hepatomegaly, bone pain, limp, paraplegia, cervical lymphadenopathy, proptosis, periorbital bruising, skin nodules

24
Q

What investigations are done for neuroblastoma?

A

Characteristic clinical + radiological features with raised urinary catecholamine levels suggest neuroblastome

  • confirmatory biopsy
  • bone marrow sampling
  • MIBG scan
  • bone scan
  • bone marrow scan for neuroblastoma cells in ‘rosettes’
25
Q

Mx of neuroblastoma

A

Surgery, radiation + chemo

Maybe bone marrow transplantation

26
Q

What indicates poor prognosis of neuroblastoma?

A
  • over expression of N-myx oncogene
  • evidence of deletion of material on Chr 1
  • again of genetic material on Chr 17q in tumour cells
27
Q

What is nephroblastoma also known as?

A

Wilms tumour

28
Q

What is wilms tumour?

A

Originates from embryonal renal tissue and commonest cause of renal tumour of childhood

29
Q

What is clinical presentation of wilms?

A

80%: asymptomatic abdominal mass

Uncommon sx: abdo pain, anorexia, anaemia, haemturia, HTN

30
Q

DD of nephroblstoma

A

hydronephrosis, PCK, renal cell carcinoma, neuroblastoma, lymphoma

31
Q

Ix of wilms

A

US/CT/MRI: shows an intrinsic renal mass distorting the normal structure
Staging: lung mets

32
Q

Mx of wilms

A

initial chemo then nephrectomy

33
Q

What other congenital abnormalities might be assoc with wilms tumour?

A

sporadic anridia, hemihyperthrophy, genitourinary abnormalities

34
Q

What is a retinoblastoma?

A

Malignant tumour of retinal cells and accounts for 5% of vision impairment in children

35
Q

What’s the difference between bilateral and unilateral retinoblastoma?

A

Bilateral: hereditary
Unilateral: 20% hereditary, dominant incomplete penetrance on Chr 13, mostly present in first 3years

36
Q

What are the clinical features of retinoblastoma?

A

White pupillary reflex instead of red

Squint

37
Q

Ix for retinoblastoma

A

MRI + examine under anaesthetic

Tumours often multifocal

38
Q

Tx for retinoblastoma

A

Depends on findings

  • enuculation of eye
  • chemo + radio
  • laser treatment of retina
39
Q

What are the different types of bone tumours?

A

Osteogenic sarcoma: more common

Ewing sarcoma: more often in younger children

40
Q

Clinical features of bone tumours

A

Limbs most common site
Persistent, localised bone pain
Mass

41
Q

Ix of bone tumours

A 
Plain x-ray: destruction + variable periostel new bone formation
MRI
Bone scan
Chest CT
Bone marrow sampling
42
Q

Mx of bone tumours

A

Chemo + surgery

Amputation

43
Q

What is the most common soft tissue sarcoma in children?

A

rhabdomyosarcoma (originates from primitive mesenchymal tissue)

44
Q

What are the clinical features of soft tissue sarcoma?

A

Head + neck
- proptosis, nasal obstruction or blood stained nasal discharge

Genitourinary tumours

  • bladder, paratesticular structures or female genitourinary tract
  • dysuria, urinary obstruction, scrotal mass, blood stained vaginal discharge

Mets
- lung, liver, bone, marrow

45
Q

Ix of sarcoma

A

Biopsy

MRI

46
Q

What are the types of malignant liver tumours?

A

liver tumours are rare!
Hepatoblastoma (65% - better prognosis)
Hepatocellular carcinoma (25%)

47
Q

Clinical presentation of liver tumours

A

abdominal distension
mass
pain + jaundice (rare)

48
Q

Ix of liver tumour

A

Elevated serum alpha fetoprotein (detected in nearly all heaptoblasoma and some hepatocellular)

49
Q

Mx of liver tumour

A

Chemo, surgery, liver transplantaiton

50
Q

What are germ cell tumours?

A

Arise from primitive germ cells which migrate from yolk sac endoderm to form gonads in the embryo. Can be benign or malignant.

51
Q

Where are benign germ cell tumours usually found?

A

Sacrococcygeal region

52
Q

Where are malignant germ cell tumours usually found?

A

Gonads

- v sensitive to chemo so good outcome

53
Q

Ix for germ cell tumours

A

Serum markers alpha fetopretein and beta hCG confirm diagnosis

54
Q

What is langerhans cell histiocytosis? (LCH)

A

A rare disorder characterised by an abnormal proliferation of histiocytes. A disorder of dendritic (antigen-presenting) cells.

55
Q

How does LCH manifest?

A

bone lesions

  • present at any age with pain, swelling or fracture
  • xray: lytic lesion with well-defined border, often involving skull

diabetes insipidus
- may be assoc with skull disease, proptosis + hypothalamic infiltration

systemic LCH

  • most aggressive form
  • present in infancy with seborrheic rash and sub tissue involvement of gums, ears, lungs, liver, spleen, lymph nodes and bone marrow

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