Malignancy Flashcards
What is the mutation in retinoblastoma
RB gene, Chr 13
What syndrome is leukaemia associated with
Downns
What syndrome is glioma associated with
neurofibromatosis
What malignancies are associated to EBV
Burkitt
Hodgkins
nasopharyngeal cancer
What marker will be raised for a neuroblastoma
VMA (vannilllylmandelic acid) & homovanillic acid (HVA)
What is a neuroblastoma
Tumour arising from neural crest tissue in adrenal medulla and sympathetic nervous system
What. can a neuroblastoma do/ evolve
can spontaneously regress
can become highly malignant
what are clinical features of a neuroblastoma
pallor
WL
abdo mass (anywhere along the sympathetic chain)
What is the classical primary abdominal tumour in neuroblastoma
ADRENAL TUMOUR
large. and complex at presentation
crosses the midline, envelops major blood vessels and nodes
What is the key problem from neuroblstomas
can cause spinal cord compression
can cause metastatic disease
what are investigations for neuroblastoma
CT/MRI
high urine catecholamine metabolites (VMA, HVA)
biopsy
bone marrow sampling
MIBG scan (MIBG is taken up by neuroblastoma, so this s can identifies areas of high uptake)
What is prognosis of neuroblastoma
if presenting at >1 year of age: advance disease, poor prognosis
What is a wilms tumour
Neophroblastoma (originates from embryonal renal tissue)
What is the most common renal tumour in children
wilms tumour
when does wilms tumour present
UNDER 5 years old
What are Clin features of wilms tumour
large abdo mass - usually child is WELL
abdo pain, anorexia, anaemia, haematuria
how do you investigate a wilms tumour’
ULTRASOUND
CT/MRI
how do you manage a neuroblastoma
surgery - if not metastasised
chemo - if metastasised
high risk of relapse
how do you manage wilms tumour
surgery + chemo
most patients are cure
what is a retinoblastoma
a malignant tumour of retinal cell
HEREDITARY
what are clinical features of a retinoblastoma
WHITE pupillary reflex (instead of red)
squint
how don you ix retinoblastoma
MRI
Exam under anaesthetic
How do you mx retinoblastoma
CURE but PRESERVE VISION
chemotherapy shrinks tumour
Laser tx
Radiotherapy
what are complications of retinoblastoma
visual impairment
enucleation (removal of eye)
