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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
Mx of neuroblastoma
Surgery, radiation + chemo | Maybe bone marrow transplantation
26
What indicates poor prognosis of neuroblastoma?
- 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
What is nephroblastoma also known as?
Wilms tumour
28
What is wilms tumour?
Originates from embryonal renal tissue and commonest cause of renal tumour of childhood
29
What is clinical presentation of wilms?
80%: asymptomatic abdominal mass | Uncommon sx: abdo pain, anorexia, anaemia, haemturia, HTN
30
DD of nephroblstoma
hydronephrosis, PCK, renal cell carcinoma, neuroblastoma, lymphoma
31
Ix of wilms
US/CT/MRI: shows an intrinsic renal mass distorting the normal structure Staging: lung mets
32
Mx of wilms
initial chemo then nephrectomy
33
What other congenital abnormalities might be assoc with wilms tumour?
sporadic anridia, hemihyperthrophy, genitourinary abnormalities
34
What is a retinoblastoma?
Malignant tumour of retinal cells and accounts for 5% of vision impairment in children
35
What's the difference between bilateral and unilateral retinoblastoma?
Bilateral: hereditary Unilateral: 20% hereditary, dominant incomplete penetrance on Chr 13, mostly present in first 3years
36
What are the clinical features of retinoblastoma?
White pupillary reflex instead of red | Squint
37
Ix for retinoblastoma
MRI + examine under anaesthetic | Tumours often multifocal
38
Tx for retinoblastoma
Depends on findings - enuculation of eye - chemo + radio - laser treatment of retina
39
What are the different types of bone tumours?
Osteogenic sarcoma: more common | Ewing sarcoma: more often in younger children
40
Clinical features of bone tumours
Limbs most common site Persistent, localised bone pain Mass
41
Ix of bone tumours
``` Plain x-ray: destruction + variable periostel new bone formation MRI Bone scan Chest CT Bone marrow sampling ```
42
Mx of bone tumours
Chemo + surgery | Amputation
43
What is the most common soft tissue sarcoma in children?
rhabdomyosarcoma (originates from primitive mesenchymal tissue)
44
What are the clinical features of soft tissue sarcoma?
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
Ix of sarcoma
Biopsy | MRI
46
What are the types of malignant liver tumours?
liver tumours are rare! Hepatoblastoma (65% - better prognosis) Hepatocellular carcinoma (25%)
47
Clinical presentation of liver tumours
abdominal distension mass pain + jaundice (rare)
48
Ix of liver tumour
Elevated serum alpha fetoprotein (detected in nearly all heaptoblasoma and some hepatocellular)
49
Mx of liver tumour
Chemo, surgery, liver transplantaiton
50
What are germ cell tumours?
Arise from primitive germ cells which migrate from yolk sac endoderm to form gonads in the embryo. Can be benign or malignant.
51
Where are benign germ cell tumours usually found?
Sacrococcygeal region
52
Where are malignant germ cell tumours usually found?
Gonads | - v sensitive to chemo so good outcome
53
Ix for germ cell tumours
Serum markers alpha fetopretein and beta hCG confirm diagnosis
54
What is langerhans cell histiocytosis? (LCH)
A rare disorder characterised by an abnormal proliferation of histiocytes. A disorder of dendritic (antigen-presenting) cells.
55
How does LCH manifest?
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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