MALIGNANCY IN CHILDREN Flashcards by Alex Kinsky

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

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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What is the most common leukaemia in children?

Acute lymphoblastic leukaemia - account for 80%

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

2-5 years of age

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

Bone marrow infiltration

Reticulo-endothelial infiltration

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

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

Anaemia - pallor, lethargy

Neutropenia - Infection

Thrombocytopenia - bruising, petechiae, nose bleeds

Bone pain

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

Hepatosplenomegaly

Lymphadenopathy

Superior mediastinal obstruction (uncommon)

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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?

CNS - headache

Testes - enlargement

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

FBC

Blood film

Bone marrow examination

Chest x-ray - to look for mediastinal mass

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

Blast cells

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What will the FBC of a child with ALL show?

Low RCC

High or low WCC

Low platelets

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

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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How long does treatment for ALL tend to last?

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

Vincristine

Dexamethasone

L-Asparginase

Intrathecal methotrexate

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What rate of remission does induction treatment of ALL achieve?

95% with four weeks of chemotherapy

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How do we treat a relapse of ALL?

High dose chemotherapy

Bone marrow transplantation

Total body irradiation

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What is the genetic disease associated with ALL?

Down syndrome

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Where in the brain do most tumours develop in children?

60% are infratentorial whereas most adult tumours are supratentorial

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

Astrocytoma (40%)

Medulloblastoma (20%)

Ependymoma (8%)

Brainstem glioma (6%)

Craniopharyngioma (4%)

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

Vomiting

Separation of sutures/tense fontanelle

Increased head circumference

Head tilt/posturing

Development delay/regression

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

Headache - worse in the morning

Vomiting - especially waking in the morning

Behaviour / personality change

Visual disturbance

Papilloedema

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What is the best imaging technique for viewing brain tumours?

MRI

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

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

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

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How do we manage a child with a brain tumour?

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

Non-Hodgkin lymphoma

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

Hodgkin lymphoma

What are the clinical features of Hodgkin lymphoma?

Painless, large, firm lymphadenopathy Lymph nodes may cause airway obstruction More often than not there are no B symptoms

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

In the neck

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

Lymph node biopsy Radiological assessment of all nodal sites Bone marrow biopsy - to stage disease and determine treatment

How do we treat Hodgkin lymphoma in adolescents?

Combination chemotherapy with or without radiotherapy

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

PET scanning

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

80% | even 60% of those with disseminated disease will be cured

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

Mediastinal mass that may cause superior vena cava obstruction

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

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.

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

Biopsy Radiological assessment of all nodal sites Examination of the bone marrow Examination of CSF

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

Multi-agent chemotherapy

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

Over 80%

Where do neuroblastomas arise from in children?

Neural crest tissue in the adrenal medulla and sympathetic nervous system

What is the name for the benign type of neuroblastoma?

Ganglioneuroma

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

Under 5 years

What are the common presenting features of neuroblastoma in children?

Abdominal mass (remember that primary tumour can actually lie anywhere along sympathetic chain from neck to pelvis) Pallor Weight loss Hepatomegaly Bone pain Limp

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

Paraplegia - spinal cord compression Cervical lymphadenopathy Proptosis Periorbital bruising Skin nodules

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

BP USS MRI Urinary catecholamine levels Biopsy

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

MIBG (metaiodobenzylguanidine) scan with or without a bone scan

What is the prognosis for a child with neuroblastoma?

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

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

MDT Surgery

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

MDT Chemotherapy Stem-cell rescue Surgery Radiotherapy

What is the other name for a Wilms tumour?

Nephroblastoma

What cells do nephroblastomas originate from?

Embryonal renal tissue

What is the peak age of incidence of Wilms tumour?

Over 80% will present before their 5th birthday

What is the most common clinical feature of Wilms tumour?

Large abdominal mass found incidentally

What are the less common features of Wilms tumour?

Abdominal pain Anorexia Anaemia - due to haemorrhage into mass Haematuria Hypertension Associated with hemihypertrophy

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

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

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

Lung 20% of patients will have metastases

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

What is the management of Wilms tumour?

Initial chemotherapy followed by delayed nephrectomy Radiotherapy is restricted to those with more advanced disease

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

More than 80% are cured Even those with metastatic disease at presentation have a 60% cure rate Relapse carries poor prognosis

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

Rhabdomyosarcoma

Where are the most common sites for rhabdomyosarcoma to develop?

Head and neck Genitourinary tumours

What are the clinical features of head and neck rhabdomyosarcoma?

Palpable mass Proptosis Nasal obstruction Blood stained nasal discharge

What are the overall cure rates of rhabdomyosarcoma in children?

65%

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

Adolescents

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

Osteogenic sarcoma

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

Uncommon before puberty

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

Ewing sarcoma

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

Males

Which bones are most often affected by malignancy in children?

Bones of the limbs

What are the clinical features of bone malignancy in children?

Persistent localised pain Pathological fracture

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

Plain x-ray MRI Bone scan Chest CT - for lung metastasis

How do we manage bone tumours in children and adolescents?

Surgery followed by chemotherapy Radiotherapy used in Ewing sarcoma

What percentage of retinoblastomas are hereditary?

All bilateral retinoblastomas 20% of unilateral retinoblastomas

What is the inheritance pattern of retinoblastoma?

Autosomal dominant

On which chromosome is the susceptible gene for retinoblastoma found?

Chromosome 13

What is the peak age of incidence of retinoblastoma?

Around 18 months most cases will present in the first 3 years of life

What are the features of retinoblastoma?

White pupillary reflex (leukocoria) or absent red pupillary reflex Squint Strabismus Visual problems

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

MRI Examination under anaesthetic

How do we manage a child with retinoblastoma?

Enucleation Chemotherapy - particularly for bilateral disease Radiotherapy - often reserved for recurrence

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

ALL - peak incidence in this age group Non-Hodgkin lymphoma Neuroblastoma Wilm tumour Retinoblastoma

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

ALL - although peak age is under 5s Brain tumours

What are the paediatric malignancies that affect adolescents?

ALL - although peak age is under 5s Malignant bone tumours Soft tissue sarcoma - rhabdomyosarcoma