Oncology Flashcards
What is a wilms tumour (nephroblastoma)
mc renal tumour of childhood originating from embryonal renal tissue
At what age does a wilms tumour typically present
Before the age of 5
Give clinical features of a wilms tumour
- unilateral large abdominal mass
- painless haematuria
- Anaemia
- flank pain
How is a wilms tumour diagnosed
- Ultrasound and/or CT/MRI of abdomen - shows intrinsic renal mass
- children with an unexplained enlarged abdo mass should have a paeds review within 48hrs
How is a wilms tumour managed
- Initial chemo followed by delayed nephrectomy
- Staged histologicially then subsequent Tx is planned according to findings
What is a retinoblastoma
Malignant tumour of retinal cells
that can be bilateral or unilateral
What does a bilateral retinoblastoma indicate about the cause
Hereditary
What chromosome is the retinoblastoma susceptibility gene found and what is the pattern of inheritance
- chromosome 13
- Dominant inheritance pattern with incomplete penetrance
At what age do children typically present with retinoblastomas
first 3 years of life
Give 2 clinical features of a retinoblastoma
- white pupillary reflex (replaces normal red one)
- Squint
How is a retinoblastoma diagnosed
- ophthalmic A- and B-scan ultrasound
- fundoscopy and exam under anaesthesia
How is a retinoblastoma treated
- Aims to cure and preserve vision
- Chemotherapy to shrink tumour followed by local laser treatment
- Enucleation in advanced disease
What are the two most common leukaemias in children
- Acute lymphoblastic leukaemia (ALL) - most common
- Acute myeloid leukaemia (AML) - 2nd most common
Give 3 RFs for leukaemia in children
- Down’s syndrome
- Kleinfelter syndrome
- Noonan syndrome
- radiation exposure during pregnancy
Describe how leukaemia may present in children
- anaemia - lethargy and pallor
- neutropenia - frequent or severe infections
- thrombocytopenia - easy bruising, petechiae
- failure to thrive
- fever
- hepatosplenomegaly
How is leukaemia investigated in children
- NICE recommend referring any child with unexplained petechiae or hepatomegaly for specialist assessment
- FBC within 48h if leukaemia is suspected
- Bone marrow biopsy is diagnostic
Poor prognostic factors of acute lymphoblastic leukaemia
age < 2 years or > 10 years
WBC > 20 * 109/l at diagnosis
T or B cell surface markers
non-Caucasian
male sex
Give 5 types of brain tumour seen in children
- astrocytoma
- medulloblastoma
- ependymoma - Commonly seen in the 4th ventricle
- brainstem glioma
- craniopharyngioma - solid/ cystic tumour derived from embryological remnants of rathke’s pouch
What is a medulloblastoma
aggressive paediatric brain tumour that arises within the infratentorial compartment and spreads through the CSF
What is the most common primary brain tumour in children
Pilocytic astrocytoma
How may a brain tumour present in children
- raised intracranial pressure - Headache worse on waking, coughing, straining or bending forward
- Back pain
- Peripheral weakness of arms/legs
- Bladder/bowel dysfunction
How is a brain tumour managed
- diagnosed on MRI scan
- surgery - aimed at treating hydrocephalus
- chemo/ radiotherapy depending on tumour site and patient age
What is a neuroblastoma
tumour arising from neural crest tissue of the adrenal medulla (mc site) and sympathetic nervous system
Describe how a neuroblastoma may present in children
- abdominal mass
- pallor
- weight loss
- bone pain, limp
- hepatomegaly
- periorbital bruising and buldging
How is a neuroblastoma investigated
- raised catecholamine levels (urinary vanillylmandelic acid and homovanillic acid)
- biopsy
- abdo XR may show calcification
Give two types of bone cancers seen in children
- osteosarcoma
- ewing’s sarcoma
How may bone cancers present in children
- affecting pelvis and long bones in ewing’s
- affecting long bones prior to epiphyseal closure in osteosarcoma
- persistent bone pain, worse at night
- bone swelling
How are bone cancers investigated in children
- XR within 48h
- osteosarcoma: destruction and variable periosteal new bone formation and sunburst pattern
- ewing’s: soft tissue mass - onion skin appearance
How are bone cancers managed in children
- combination chemo before surgery
- surgical resection of lesions often with amputation
- MDT approach
How does a hepatoblastoma present in children
- abdo distension/ mass
- abdo pain
- jaundice (rare)
What tumour marker is nearly always raised in hepatoblastomas
alpha fetoprotein
