NEUROBLASTOMA

NEUROBLASTOMA
- Embryonal tumor malignant tumor
of sympathetic nervous system
derived from primitive neural crest
The most common:
- extracranial childhood cancer
- of nervous system solid tumor
Incidence: 8.7 10.5 per million
children < 15 years of age
two-thirds of cases:
< 5 years of age

LOCATION OF PRIMARY TUMOR

can arise from any site
along the sympathetic nervous system
-

adrenal (suprarenal) the most common

paraspinal ganglia
pelvic
cervical
thoracic

1% cases primary tumor cannot

be found

CLINICAL FEATURES
Depends on lacalisation of:
- primary tumor
- metastasis
Primary tumor:
Abdomen:
a tumor mass
abdominal pain
Paraspinal gangglia:
compress the spinal cord
paralysis

CLINICAL FEATURES
Neck/thoracic:
Horners syndrome & swelling
mediastinal mass
Pelvic: constipation
bladder dysfunction

Symptoms due to metabolic activity:

sweating
hypertension
irritation
diarrhoea

CLINICAL FEATURES
Metastasis:
Bone/bone marrow:
bone pain
pancytopenia
Retrobulbar:
proptosis
periorbital ecchymosis
Liver: abdominal distention
liver dysfunction
Lung: respiratory symptoms

DIAGNOSTIC PROCEDURES
-

Clinical features
Radiodiagnostic
Tumor markers
Pathologytissue biopsy:
- open biopsy
- FNA

- Bone marrow aspiration

- Nuclear scanning with MIBG
(meta-iodobenzylguanidine)

- Genetic examination

Radiology:
a mass or multiple masses
plain radiograph, CT or MRI
IVP: dysplacement
of calyces (renal pelvic)

Tumor markers:
Elevated levels:
- Serum catecholamine,
urine catecholamine metabolites:
vanillymandelic acid (VMA)
- Ferritin

Cytogenetics analysis:
Deletion of distal short arm of chromosome 1
in 70% cases
The amplification of MYCN (proto-oncogen
derived from the distal short arm
of chromosom 2)
in 30% cases

70% patients have metastatic at diagnosis

The minimum criterion for diagnosis:

1. An unequivocal pathologic
from tissue tumor (histopathology)
2. The combination:
- Bone marrow: unequivocal tumor cells
- Increased level serum catecholamine or
urine (VMA)

International Neuroblastoma Staging System (INSS)

Stage 1:
Localized tumor with complete gross excision
Ipsilateral & contralateral lymph nodes: negative

Stage 2a:
Localized tumor with incomplete gross excision
Ipsilateral lymph nodes: negative

Stage 2b:
Localized tumor with or without complete
gross excision; ipsilateral lymph nodes: positive;
contralateral lymph nodes: negative

International Neuroblastoma Staging System (INSS)

Stage 3:
- Unresectable unilateral tumor infiltrating across
the midline with or without regional lymph node
involvement; or
- Localized unilateral tumor with contralateral
regional lymph node involvement; or
- Midline tumor with bilateral extension by infiltration
(unresectable) or by lymph node involvement.

Stage 4:
Any primary tumor with dissemination to distant lymph node,
bone marrow, liver, skin and/or other organs

TREATMENT
Dependent on:
- Stage
- Biological characteristics by biopsy
- Catecholamine metabolites
determine the persistence of disease
Treatment modalities:
- Surgery
- Chemotherapy
- Radiation
- New therapeutics:
- bone marrow transplantation
- immunomodulation
- differentiation therapy

PROGNOSIS
Related to:
-

age
stage
pathology
urine VMA
ferritin
MYCN
1p deletion