2. Neuroblsatoma is a malignant tumor from neural
crest of
- adrenal medulla (35%)
- sympathetic ganglia
- abdomen 35%
- thoracic 25%
- cervical 5%
Microscopically contain: rossete shaped cells and
ganglion cells
3. Clinical picture:
Commonest malignancy in first year of life,
about 8-10% of children are effected.
The five common presentation are
1- abdominal mass
2- mediastinal mass and may manifest with
Dyspnea , superior vena cava obstruction and
horner syndrome
4. 3- spinal cord compression if sympathetc chain near
cord effected and present with:
Back pain and tenderness, parapalegia and
sphincteric dysfunction
4- metastatic neuroblastoma
- if bone marrow 70% = pancytopenia
- if bone = pain and limbing or refuse to walk
and mouth eaten appearance in dipolic space
5. - liver = hepatomegaly
- orbit = proptosis and raccoon like appearance
- skin = blue berry muffin lesions
5- paraneoplastic syndrome are 3 :
a- excessive catecholamine secretion will lead
to intermittent attacks of sweating,
palpitation, HTN, flushing, polyuria and
polydypsia
6. b- VIP secretion may lead to kerner morison
syndrome which is triad of:
1- intractable diarrhoea
2- abdominal distension
3- hypokalemia
c- acute myoclonic encephalopathy syndrome
- also called dancing eye syndrome
- consist of opsclonus and myoclonus
- treated by dexamethazone and IVIG
8. Diagnosis
1- detect the origin of primary site by
- CT and U/S of abdomen , chest X ray
- MRI of spine
- biobsy
- MIBG scan
2- screen for urinary catecholamines
by VMDA and homovallinilic
9. Treatment
1- supportive care
2- surgery
3- combination chemotherapy
4- irradiation
5- Bone Marrow Transplantation
Nb: ttt vary according to age, cytogenetics and
stage
