MALIGNANCY IN CHILDREN Flashcards
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%
What is the most common leukaemia in children?
Acute lymphoblastic leukaemia - account for 80%
What is the peak age of incidence of ALL in children?
2-5 years of age
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)
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
What are the clinical features of ALL in children associated with infiltration of the reticulo-endothelial system?
Hepatosplenomegaly
Lymphadenopathy
Superior mediastinal obstruction (uncommon)
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
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
What will be seen on the blood film of a child with ALL?
Blast cells
What will the FBC of a child with ALL show?
Low RCC
High or low WCC
Low platelets
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
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.
What are the four original drugs used to induce remission in children with ALL?
Vincristine
Dexamethasone
L-Asparginase
Intrathecal methotrexate
What rate of remission does induction treatment of ALL achieve?
95% with four weeks of chemotherapy
How do we treat a relapse of ALL?
High dose chemotherapy
Bone marrow transplantation
Total body irradiation
What is the genetic disease associated with ALL?
Down syndrome
Where in the brain do most tumours develop in children?
60% are infratentorial whereas most adult tumours are supratentorial
What are the different types of brain tumour found in children?
Astrocytoma (40%)
Medulloblastoma (20%)
Ependymoma (8%)
Brainstem glioma (6%)
Craniopharyngioma (4%)
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
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
What is the best imaging technique for viewing brain tumours?
MRI
Magnetic resonance spectroscopy can be used to examine biological activity of a tumour
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
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
Which is more common in younger childhood: Hodgkin lymphoma or non-Hodgkin lymphoma?
Non-Hodgkin lymphoma
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?
5%
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
