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517471
neuroblastoma
Descripción
(Malignancy) Paediatrics Mapa Mental sobre neuroblastoma, creado por v.djabatey el 31/01/2014.
Sin etiquetas
malignancy
paediatrics
paediatrics
malignancy
Mapa Mental por
v.djabatey
, actualizado hace más de 1 año
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Creado por
v.djabatey
hace casi 11 años
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Resumen del Recurso
neuroblastoma
arise from neural crest tissue in adrenal medulla & sympathetic NS
biologically weird tumour
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
bone pain
malaise
due to bone marrow suppression
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
hepatomegaly
limp
less common
paraplegia
from spinal cord compression from invasion of paravertebral tumours invading through intervertrebral foramen
cervial lymphadenopathy
proptosis
periorbital brusing
skin nodules
Ix
characteristic clinical + radiological features w/ raised urinary catecholamines
confirmatory biopsy
bone marrow sampling
to detect evidence of mets
metaiodobenzylguanidine (MIBG) scan +/- bone scan
prognostic factors
age at diag
stage of disease at diagnosis
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
Mx
localised primaries w/o mets
often surgery alone enough
metastatic disease (mets)
chemo- high dose
w/ autologous stem cell rescue, surgery & radiotherapy
immunotherapy & long term 'maintenance' Rx w/ retinoic acid (differentiating agent)
for high risk disease
prospect of cure w/ mets ~30%
high risk of relapse
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