neuroblastoma

neuroblastoma

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

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	Erstellt von v.djabatey vor fast 11 Jahre