Paeds malignant diseases and haematology (ILA 1) Flashcards
What is the most common leukaemia in children?
Acute lymphoblastic leukaemia accounts for 80% of cases in children
What is leukaemia?
cancer of the white blood cells
overproduction of immature white blood cells -> inhibit production of normal cells in bone marrow -> infiltrates organs -> organ failure
How does leukaemia commonly present?
causes bone marrow infiltration which leads to…
- anaemia - pallor, lethargy
- thrombocytopenia - bruising, nose bleeds
- neutropenia - frequent infection
- bone pain
List poor prognosis factors for leukaemia
child <2 y/o or >10 y/o B/T cell surface markers WBC >20 x 10^9/L non caucasian male sex
Which cells would you expect to see in ALL?
BLAST CELLS
How is ALL diagnosed?
FBC - anaemia, thrombocytopenia, neutropenia, blast cells
bone marrow examination *
How is ALL treated?
REMISSION INDUCTION - correct anaemia, hydration, treat infections
INTENSIFICATION - intensive chemo
What are the most common brain tumours in children?
astrocytoma **
(most malignant form is a glioblastoma multiforme)
most commonly infratentorial - located below the tentorium cerebelli
Outline the symptoms that you might see in a brain tumour
symptoms of raised intracranial pressure…
- vomiting (early in morning)
- headache
- abnormal eye movements - papilloedema, squint, nystagmus
- seizures
- problems with balance/ walking
- lethargy
- developmental delay/ problems at school / delayed puberty
List the signs of a tumour in the cortex
seizures
hemiplegia
focal neurological signs
List the signs of a tumour in the midline
visual field loss = bitemporal hemianopia
pituitary failure
List the signs of a tumour in the cerebellum / 4th ventricle
truncal ataxia
coordination difficulties
abnormal eye movements
List the signs of a tumour in the brainstem
cranial nerve defects
pyramidal tract signs
ataxia
List the signs of a tumour in the spine
peripheral weakness of limbs
back pain
bladder/ bowl dysfunction
how is a brain tumour diagnosed?
MRI
When would you consider scanning a child presenting with a headache?
- papilloedema, visual loss
- neurological signs
- <2 y/o
- headache in early morning with vomiting preceding
- short stature
- neurofibromatosis
How does hodgkin lymphoma commonly present?
painless lymphadenopathy ***
- commonly in the neck
- lymph nodes larger and firmer
several month history
systemic symptoms - tiredness, weight loss, sweating
How is hodgkins lymphoma diagnosed?
lymph node biopsy
Which lymphoma presents more commonly in childhood?
non hodgkin lymphoma more common in childhood
How does non- hodgkin lymphoma commonly present?
mediastinal mass which can cause SVC obstruction - dyspnoea, facial swelling, flushing, distended veins
bone marrow infiltration
What are the signs of bone marrow infiltration?
- anaemia
- neutropenia
- thrombocytopenia
- bone pain
How do neuroblastomas present?
- abdominal mass - large, hepatomegaly
- bone pain
- limp
- bone marrow suppression
- malaise
- weight loss
Where do neuroblastoma arise from?
arise from neural tissue in the adrenal medulla and sympathetic nervous system
What is the prognosis for a neuronblastoma?
poor - often present late at advanced state and metastatic
What is the most common renal tumour in children and when do they present?
Wilms tumour = nephroblastoma
usually present before the age of 5
where do wilms tumour arise from?
embryonal renal tissue
how do wilms tumour commonly present?
large abdo mass
haematuria
How are nephroblastomas diagnosed?
ULTRASOUND +/- CT +/- MRI
How are nephroblastomas treated?
- chemotherapy - prior and after surgery
- delayed nephrectomy
- radiotherapy
What is the most common form of soft tissue sarcoma in childhood?
rhabdomyosarcoma
Where do soft tissue sarcomas arise from?
cancers of connective tissue e.g. muscle or bone
rhabdomyosarcoma originate from primitive mesenchymal tissue
How do soft tissue sarcomas present?
head and neck most common site of disease - proptosis, nasal obstruction, bloodstained nasal discharge
Genitourinary tumours - dysuria, urinary obstruction, scrotal mass, vaginal discharge
How does a child with a bone tumour present?
usually a well child
persistent localised bone pain
Which investigations should be carried out for bone tumours?
bone scan
x-ray
MRI
What are the causes of retinoblastoma?
family history!! - all children with families with it are screened, RB1 gene, autosomal dominant
multimodal therapy
sporadic
How does retinoblastoma present?
white pupillary reflex
squint
Where do germ cell tumours arise from?
arise from primitive germ cells which migrate from yolk sac endoderm to form gonads in the embryo
Which markers are used to diagnose germ cell tumours?
alpha fetoprotein
beta hCG
What are the 2 types of primary malignant liver tumours?
hepatoblastoma (65%) hepatocellular carcinoma (25%)
What are the common symptoms for a liver tumour?
- abdominal distension
- abdominal mass
- jaundice
- pain
How are liver tumours diagnosed?
elevated serum alpha fetoprotein
What are the symptoms of langerhans cell histiocytosis?
- bone lesions - pain, swelling, fracture
- diabetes insipidus
- systemic LCH - seborrhoea rash, soft tissue involvement
How is langerhans cell histiocytosis diagnosed and what would you see?
x-ray - lytic lesions with well defined border in bones, often involving skull (punched out osteolytic lesions)
What is langerhans histiocytosis?
abnormal proliferation of histiocytes
What are the most common cancers in children before the age of 5?
ALL
neuroblastoma
wilm tumour
retinoblastoma