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PAEDIATRICS > MALIGNANCY IN CHILDREN > Flashcards

MALIGNANCY IN CHILDREN Flashcards

1
Q

What are the top 8 cancers seen in children in the UK?

A

Leukaemia - 32% of cancers in children

Brain and spinal tumours - 24%

Lymphomas - 10%

Neuroblastomas - 7%

Soft tissue sarcomas - 7%

Wilms tumour - 6%

Bone tumours - 4%

Retinoblastoma - 3%

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2
Q

What is the most common leukaemia in children?

A

Acute lymphoblastic leukaemia - account for 80%

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3
Q

What is the peak age of incidence of ALL in children?

A

2-5 years of age

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4
Q

What are the different system that are infiltrated in ALL in children?

A

Bone marrow infiltration

Reticulo-endothelial infiltration

Other organ inflitration (more common in relapse than original presentation)

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5
Q

What are the clinical features of ALL in children associated with infiltration of the bone marrow?

A

Anaemia - pallor, lethargy

Neutropenia - Infection

Thrombocytopenia - bruising, petechiae, nose bleeds

Bone pain

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6
Q

What are the clinical features of ALL in children associated with infiltration of the reticulo-endothelial system?

A

Hepatosplenomegaly

Lymphadenopathy

Superior mediastinal obstruction (uncommon)

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7
Q

What are the other organs that are commonly infiltrated in ALL in children and what are the clinical features associated with infiltration of such organs?

A

CNS - headache

Testes - enlargement

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8
Q

What investigations should you do in a child who presents with signs and symptoms associated with leukaemia?

A

FBC

Blood film

Bone marrow examination

Chest x-ray - to look for mediastinal mass

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9
Q

What will be seen on the blood film of a child with ALL?

A

Blast cells

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10
Q

What will the FBC of a child with ALL show?

A

Low RCC

High or low WCC

Low platelets

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11
Q

What are the prognostic factors to consider in ALL and what are the high risk features?

A

Age - less than 1 or more than 10

Tumour load (WCC) more than 50 x 10^9/L

Cytogenic / molecular genetic abnormalities - MLL rearrangement t(4;11)

Speed of response to initial chemotherapy - Persistence of leukaemic blasts in bone marrow

Minimal residual disease assessment (levels of leukaemia detected by PCR) - High

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12
Q

How long does treatment for ALL tend to last?

A

Remission treatment usually last 23 weeks and then maintenance treatment is given for another 2 years in girls and 3 years in boys.

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13
Q

What are the four original drugs used to induce remission in children with ALL?

A

Vincristine

Dexamethasone

L-Asparginase

Intrathecal methotrexate

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14
Q

What rate of remission does induction treatment of ALL achieve?

A

95% with four weeks of chemotherapy

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15
Q

How do we treat a relapse of ALL?

A

High dose chemotherapy

Bone marrow transplantation

Total body irradiation

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16
Q

What is the genetic disease associated with ALL?

A

Down syndrome

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17
Q

Where in the brain do most tumours develop in children?

A

60% are infratentorial whereas most adult tumours are supratentorial

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18
Q

What are the different types of brain tumour found in children?

A

Astrocytoma (40%)

Medulloblastoma (20%)

Ependymoma (8%)

Brainstem glioma (6%)

Craniopharyngioma (4%)

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19
Q

What are the clinical features of raised intracranial pressure caused by brain tumours in infants?

A

Vomiting

Separation of sutures/tense fontanelle

Increased head circumference

Head tilt/posturing

Development delay/regression

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20
Q

What are the clinical features of raised intracranial pressure caused by brain tumours in children over 1 years old?

A

Headache - worse in the morning

Vomiting - especially waking in the morning

Behaviour / personality change

Visual disturbance

Papilloedema

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21
Q

What is the best imaging technique for viewing brain tumours?

A

MRI

Magnetic resonance spectroscopy can be used to examine biological activity of a tumour

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22
Q

Should an LP be performed on someone with signs and symptoms of brain tumour?

A

Not without advice from a neurosurgeon if there is any suspicion of raised intracranial pressure

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23
Q

How do we manage a child with a brain tumour?

A

First treatment is usually surgery to treat hydrocephalus, provide a tissue diagnosis and attempt maximum resection.

Chemotherapy and radiotherapy are used but they are dependent on child’s age and the tumour type

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24
Q

Which is more common in younger childhood: Hodgkin lymphoma or non-Hodgkin lymphoma?

A

Non-Hodgkin lymphoma

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25
Q

Which is more common in adolescence: Hodgkin lymphoma or non-Hodgkin lymphoma?

A

Hodgkin lymphoma

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26
Q

What are the clinical features of Hodgkin lymphoma?

A

Painless, large, firm lymphadenopathy

Lymph nodes may cause airway obstruction

More often than not there are no B symptoms

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27
Q

Where in the body is the lymphadenopathy associated with Hodgkin lymphoma most commonly found?

A

In the neck

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28
Q

What investigations would you do for an adolescent that presents with large, firm lymph nodes in the neck suspicious of Hodgkin lymphoma?

A

Lymph node biopsy

Radiological assessment of all nodal sites

Bone marrow biopsy - to stage disease and determine treatment

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29
Q

How do we treat Hodgkin lymphoma in adolescents?

A

Combination chemotherapy with or without radiotherapy

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30
Q

How do we monitor response to treatment for Hodgkin lymphome in an adolescent?

A

PET scanning

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31
Q

What is the cure rate of adolescents diagnosed with Hodgkin lymphoma?

A

80%

even 60% of those with disseminated disease will be cured

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32
Q

What is the characteristic feature of T-cell non-Hodgkin lymphoma?

A

Mediastinal mass that may cause superior vena cava obstruction

33
Q

What are the characteristic features of B-cell non-Hodgkin lymphoma?

A

Localised lymph node usually in the head, neck or abdomen.

Abdominal disease will present with pain from intestinal obstruction, a palpable mass or even intussusception.

34
Q

What are the investigations that should be done in someone with suspected non-Hodgkin lymphoma?

A

Biopsy

Radiological assessment of all nodal sites

Examination of the bone marrow

Examination of CSF

35
Q

How do we manage non-Hodgkin lymphoma in a child?

A

Multi-agent chemotherapy

36
Q

What is the survival rate of non-Hodgkin lymphoma in a child?

A

Over 80%

37
Q

Where do neuroblastomas arise from in children?

A

Neural crest tissue in the adrenal medulla and sympathetic nervous system

38
Q

What is the name for the benign type of neuroblastoma?

A

Ganglioneuroma

39
Q

What is the peak age of incidence of neuroblastoma in children?

A

Under 5 years

40
Q

What are the common presenting features of neuroblastoma in children?

A

Abdominal mass (remember that primary tumour can actually lie anywhere along sympathetic chain from neck to pelvis)

Pallor

Weight loss

Hepatomegaly

Bone pain

Limp

41
Q

What are the less common presenting features of neuroblastoma in children?

A

Paraplegia - spinal cord compression

Cervical lymphadenopathy

Proptosis

Periorbital bruising

Skin nodules

42
Q

What are the investigations that you would do for a child who presents with a large abdominal mass suggestive of neuroblastoma?

A

BP

USS

MRI

Urinary catecholamine levels

Biopsy

43
Q

How would you look confirm metastases in a child with confirmed neuroblastoma?

A

MIBG (metaiodobenzylguanidine) scan with or without a bone scan

44
Q

What is the prognosis for a child with neuroblastoma?

A

Unfortunately, those over 1 years old normally present with quite advanced disease and prognosis is not good.

45
Q

How do we manage a child with neuroblastoma with no signs of metastasis?

A

MDT

Surgery

46
Q

How do we manage a child with neuroblastoma with confirmed metastasis?

A

MDT

Chemotherapy

Stem-cell rescue

Surgery

Radiotherapy

47
Q

What is the other name for a Wilms tumour?

A

Nephroblastoma

48
Q

What cells do nephroblastomas originate from?

A

Embryonal renal tissue

49
Q

What is the peak age of incidence of Wilms tumour?

A

Over 80% will present before their 5th birthday

50
Q

What is the most common clinical feature of Wilms tumour?

A

Large abdominal mass found incidentally

51
Q

What are the less common features of Wilms tumour?

A

Abdominal pain

Anorexia

Anaemia - due to haemorrhage into mass

Haematuria

Hypertension

Associated with hemihypertrophy

52
Q

What investigations would be done for a child who present with a large abdominal mass but is otherwise well suggestive of Wilms tumour?

A

USS / CT / MRI - shows an intrinsic renal mass distorting the normal structure

53
Q

What is the most common location of distant metastasis from Wilms tumour?

A

Lung

20% of patients will have metastases

54
Q

What percentage of children have bilateral disease at diagnosis of Wilms tumour?

A

5%

55
Q

What is the management of Wilms tumour?

A

Initial chemotherapy followed by delayed nephrectomy

Radiotherapy is restricted to those with more advanced disease

56
Q

What is the prognosis for a child diagnosed with Wilms tumour?

A

More than 80% are cured

Even those with metastatic disease at presentation have a 60% cure rate

Relapse carries poor prognosis

57
Q

What is the most common form of soft tissue sarcoma in childhood?

A

Rhabdomyosarcoma

58
Q

Where are the most common sites for rhabdomyosarcoma to develop?

A

Head and neck

Genitourinary tumours

59
Q

What are the clinical features of head and neck rhabdomyosarcoma?

A

Palpable mass

Proptosis

Nasal obstruction

Blood stained nasal discharge

60
Q

What are the overall cure rates of rhabdomyosarcoma in children?

A

65%

61
Q

Children in what age group are most commonly affected by rhabdomyosarcoma?

A

Adolescents

62
Q

What is the most common type of bone tumour in children?

A

Osteogenic sarcoma

63
Q

What is the peak age of incidence of malignant bone tumours in children?

A

Uncommon before puberty

64
Q

What type of bone cancer is most common in younger children?

A

Ewing sarcoma

65
Q

Which sex is most often affected by bone malignancy in children?

A

Males

66
Q

Which bones are most often affected by malignancy in children?

A

Bones of the limbs

67
Q

What are the clinical features of bone malignancy in children?

A

Persistent localised pain

Pathological fracture

68
Q

What investigations would you do for a child who presents with persistent localised bone pain suggestive of bone tumour?

A

Plain x-ray

MRI

Bone scan

Chest CT - for lung metastasis

69
Q

How do we manage bone tumours in children and adolescents?

A

Surgery followed by chemotherapy

Radiotherapy used in Ewing sarcoma

70
Q

What percentage of retinoblastomas are hereditary?

A

All bilateral retinoblastomas

20% of unilateral retinoblastomas

71
Q

What is the inheritance pattern of retinoblastoma?

A

Autosomal dominant

72
Q

On which chromosome is the susceptible gene for retinoblastoma found?

A

Chromosome 13

73
Q

What is the peak age of incidence of retinoblastoma?

A

Around 18 months

most cases will present in the first 3 years of life

74
Q

What are the features of retinoblastoma?

A

White pupillary reflex (leukocoria) or absent red pupillary reflex

Squint

Strabismus

Visual problems

75
Q

What investigations would you do in a child with a white pupillary reflex suggestive of retinoblastoma?

A

MRI

Examination under anaesthetic

76
Q

How do we manage a child with retinoblastoma?

A

Enucleation

Chemotherapy - particularly for bilateral disease

Radiotherapy - often reserved for recurrence

77
Q

What are the paediatric malignancies that affect the under 5 year olds?

A

ALL - peak incidence in this age group

Non-Hodgkin lymphoma

Neuroblastoma

Wilm tumour

Retinoblastoma

78
Q

What are the paediatric malignancies that affect school aged children (5-12)?

A

ALL - although peak age is under 5s

Brain tumours

79
Q

What are the paediatric malignancies that affect adolescents?

A

ALL - although peak age is under 5s

Malignant bone tumours

Soft tissue sarcoma - rhabdomyosarcoma

PAEDIATRICS (41 decks)

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