517471
Description
Mind Map by v.djabatey, updated more than 1 year ago
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Created by v.djabatey
almost 11 years ago
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neuroblastoma
- arise from neural crest
tissue in adrenal medulla &
sympathetic NS
- biologically weird tumour
- spontaneous remission
sometimes occurs in very
young infants
- spontaneous remission
sometimes occurs in very
young infants
- spectrum of disease from benign
(ganglioneuroma) to highly malignant
(neuroblastoma)
- most common before 5 years old
- clinical presentation
- common
- sx from metastatic
disease
(predominate in > 2
years old)
- pallor
- weight loss
- due to bone marrow suppression
- due to bone marrow suppression
- bone pain
- malaise
- due to bone
marrow
suppression
- due to bone
marrow
suppression
- pallor
- abdo mass
- most kids have this, but
primary tumour can lie
anywhere along sympathetic
chain from neck to pelvis
- classically of adrenal origin
- but at presentation tumour mass often large,
crossing midline & surrounding major
vasculature & lymph nodes
- but at presentation tumour mass often large,
crossing midline & surrounding major
vasculature & lymph nodes
- most kids have this, but
primary tumour can lie
anywhere along sympathetic
chain from neck to pelvis
- hepatomegaly
- limp
- sx from metastatic
disease
(predominate in > 2
years old)
- less common
- paraplegia
- from spinal cord
compression from
invasion of
paravertebral tumours
invading through
intervertrebral foramen
- from spinal cord
compression from
invasion of
paravertebral tumours
invading through
intervertrebral foramen
- cervial
lymphadenopathy
- proptosis
- periorbital brusing
- skin nodules
- paraplegia
- common
- Ix
- characteristic clinical +
radiological features w/
raised urinary
catecholamines
- confirmatory biopsy
- bone marrow sampling
- to detect evidence of mets
- to detect evidence of mets
- metaiodobenzylguanidine
(MIBG) scan +/- bone
scan
- characteristic clinical +
radiological features w/
raised urinary
catecholamines
- prognostic factors
- age at diag
- stage of
disease at
diagnosis
- most kids > 1
year old pw
advanced
disease & have
poor prog
- most kids > 1
year old pw
advanced
disease & have
poor prog
- genetic factors in
tumour cells- assoc w/
poor prognosis
- over
expression of
N-myc
oncogene
- evidence of del
1p (some
material deleted
from chromsome
1)
- gain of genetic material on chromosome 17q
- over
expression of
N-myc
oncogene
- age at diag
- Mx
- localised primaries w/o mets
- often surgery alone enough
- often surgery alone enough
- metastatic disease (mets)
- chemo- high dose
- w/ autologous stem cell
rescue, surgery &
radiotherapy
- w/ autologous stem cell
rescue, surgery &
radiotherapy
- chemo- high dose
- immunotherapy & long term
'maintenance' Rx w/ retinoic
acid (differentiating agent)
- for high risk disease
- for high risk disease
- localised primaries w/o mets
- prospect of
cure w/ mets
~30%
- high risk of relapse
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