Paediatrics - Oncology Flashcards
What are the most common childhood malignancies (7)?
- Leukaemias = MC
- Brain + CNS tumours
- Bone tumours
- Lymphoma
- Wilms tumours
- Hepatoblastoma
- Retinoblastoma
What are the most common forms of leukaemia in children?
- ALL (80%)
- AML (15%)
others e.g. CML
What age do ALL and AML most frequently occur?
- ALL = 2-5/10 years
- AML < 2 years
What are some risk factors for leukaemia (2)?
- Radiation exposure e.g. x-ray during pregnancy
- Genetic conditions - downs, kleinfelters, noonans
What effect does leukaemia have on cells in the blood?
Pancytopenia
What is pancytopenia?
- Anaemia
- Leukopenia
- Thrombocytopenia
How does leukaemia lead to pancytopenia?
Mutation in a precursor cell in the bone marrow leads to an excessive production of single type of abnormal WBC. This excessive production of cells in the bone marrow deprives the blood of the normal production of other blood cells –> pancytopenia
What are the signs/ symptoms of leukaemia in children (11)?
- Fatigue
- Fever
- Failure to thrive
- Weight loss
- Night sweats
- Recurrent infections
- Pallor
- Petechiae/ bruising
- Bleeding
- Lymphadenopathy
- Hepatomegaly
How soon should an FBC be done within when leukaemia is suspected?
48 hours
How should leukaemia be investigated (3)?
- FBC
- Blood film
- Bone marrow biopsy = diagnostic
What would be present on a blood film of those with leukaemia?
Blast cells
What further tests are often used to investigate leukaemia (4)?
- Chest X-ray
- CT scan
- Lumbar puncture
- Genetic analysis
imaging + lumbar used for staging
How is leukaemia treated (3)?
- Chemo = mainstay
- Radiotherapy
- Bone marrow transplant
What medical emergency is common in patients with leukaemia and other malignancies?
Febrile neutropenia
chemo can cause low neutrophils as well
What are some complications of chemo in children (5)?
- Neurotoxicity
- Immunodeficiency
- Infertility
- Stunted growth/ development
- Tumour lysis syndrome
What is the prognosis for childhood leukaemia?
- ALL = 80% cured
- AML = less positive
Are primary or secondary brain tumours more common in children?
Almost always primary in children
What are the 5 main types of brain tumours in children?
- Astrocytoma = MC
- Medulloblastoma
- Ependymoma
- Brainstem glioma
- Craniopharyngioma
What type of tumour are astrocytomas and ependyomas?
Gliomas
astrocytes and ependyma (lining of ventricles) are glial cells
What is a highly malignant form of astrocytoma?
Glioblastoma multiforme
Where is a medulloblastoma?
Midline of the posterior fossa
What is a craniopharyngioma?
Developmental tumour arising from an embryological remnant near the pituitary gland
Which brain tumours have a worse prognosis (2)?
- Glioblastoma multiforme
- Brainstem glioma
What are the signs/ symptoms of brain tumours (7)?
- Raised ICP headache
- Papilloedema
- N+V
- Seizures
- Mood/ personality change
- Visual field defects
- Focal neurological signs
What is characteristic of raised ICP headaches (2)?
- Wake up with headache
- Coughing/ straining/ bending forward worsen headache
What are the two types of lymphoma?
- Hodgkin lymphoma
- Non-hodgkin lymphoma
What cells are found in those with Hodgkin lymphoma?
Reed-Sternberg cells
What is a common type of non-hodgkin lymphoma in children?
Burkitts lymphoma
Which lymphocytes does burkitts lymphoma affect?
B-cells
Where is burkitts lymphoma most common?
Africa
What are some risk factors for lymphoma (3)?
- EBV
- Malaria
- HIV/ immunodeficiency
What are the signs/ symptoms of lymphoma (5)?
- Lymphadenopathy
- B symptoms (fever, night sweats, weight loss)
- Fatigue
- Recurrent infections
- SOB
What is a specific symptom of burkitts lymphoma?
Swollen jaw
Where is a neuroblastoma?
- Adrenal medulla
- Sympathetic nervous chain
tumour arises from neural crest tissue
What is the prognosis of neuroblastomas?
Very good - 80% cured
What age does neuroblastoma usually occur?
Under 5 years
How do neuroblastomas present (6)?
- Abdo lump
- Pallor (anaemia due to marrow infiltration)
- Weight loss
- Bone pain
- Hepatomegaly
- Lymphadenopathy
How are neuroblastomas investigated?
- Raised urine catecholamines due to increased production of catecholamines ((nor)adrenaline/ dopamine)
- Biopsy
- Imaging
What is the most common renal tumour in children?
Wilms tumour
What age does wilms tumour usually present?
< 5 years (80% of cases)
Where do wilms tumours come from?
Embryonal renal tissue
What are the signs/ symptoms of Wilms tumours (6)?
- Large abdo mass
- Haematuria
- Abdo pain
- Anorexia
- Hypertension
- Anaemia
How are Wilms tumours investigated?
CT/ MRI
biopsy can cause spread of cancer, so usually done post surgery
How is Wilms tumour managed (2)?
- Chemotherapy first
- Nephrectomy
What are cancers of connective tissue known as?
Sarcomas
What is the most common type of sarcoma in children?
Rhabdomyosarcoma (skeletal muscle cancer)
When do bone tumours most commonly occur in childhood?
After puberty
What is the most common childhood bone cancer overall?
Osteosarcoma
What childhood bone cancer is most common in younger children (before puberty)?
Ewing sarcoma
What is the presentation of bone cancers in children?
Persistent localised bone pain even when resting
most patients are otherwise well
Where are bone cancers most commonly found?
In the limbs
How are bone cancers investigated in children (2)?
- X-ray then MRI
- CT for metastises
What might an x-ray show in bone cancer (2)?
- Bone destruction and variable periosteal bone formation
- Ewing = soft tissue swelling/ mass
How is bone cancer managed in children (3)?
- Chemo first
- Surgical resection
- Radiotherapy if resection impossible
What cell do retinoblastoma come from?
Retinal cells
What percent of severe visual impairments in children are due to retinoblastomas?
5%
What age do retinoblastomas usually present?
< 3 years
What is the cause of bilateral retinoblastomas?
Hereditary genetic cause
What is the inheritance of hereditary retinoblastoma and which chromosome is affected?
Auto dom affecting chromosome 13
What is the presentation of retinoblastomas (2)?
- Loss of red reflex –> replaced by white reflex
- Squint
How are retinoblastomas investigated (2)?
- MRI
- Examination under local anaesthetic
How is retinoblastomas managed (2)?
- Chemo
- Laser therapy
What are some complications of retinoblastomas (2)?
- Visual impairments
- Recurrence/ secondary tumour common
What cancer often affects those with HIV?
Kaposi sarcoma
not a true sarcoma, comes from cells of blood/ lymph vessels
How does Kaposi sarcoma present (2)?
- Red/ purple lesions on skin/ GI tract
- Lymphadenopathy
What is the most common type of liver cancer in children/
Hepatoblastoma
How does hepatoblastoma present (2)?
- Abdo mass/ distension
- Rarely pain/ jaundice
What tumours sometimes form in the ovaries and testes amongst other places?
Germ cell tumours
How are germ cell tumours treated?
Chemo
usually very responsive to chemo
