Cancer in Children

NEUROBLASTOMA

Introduction

Neuroblastoma derived from the primitive

neural crest
The greatest diversity in clinical behavior of all
childhood solid tumor,some tumor regressing
spontaneously ,some chemo-cureble whilst
others resistant to intensive chemotherapy
Metastases neuroblastoma associated with
worst prognosis. Relapse and drug resistant is
major problems
Therapeutic is based on patient characteristics
and tumor feature.

Epidemiologi

The incidence of neuroblastoma per year is

10.5permillion children less than 15 years.
No significant geographical variation in the
incidence
Peak of age of incidence 0 4 years, with
median age of 23 months. 40% < 1 year
More frequently in males than female ( 1.2:1)
Screening of the disease might be of value in
reducing mortality by detecting poor prognosis
disease.

Embryology,pathology and
genetics

Embryology:
Neural crest is the embryonic structure which
gives rise to the sympathetic nervous system
and also part of the facial skeleton, the
thymus,parathyroids,the
enteric
nervous
system and skin melanocytes.
Pathology:
Histologically: vary heterogenous and are
compose of two predominant cell types: thr
neuroblast/ganglion cell and the Schwann cell.
Genetics:
A genetics predisposition exists in families
Genetic studies: amplification of oncogens or
chromosomal and chromosomal losses.

Clinical presentation

Clinical presentation depend on the site of the

primary tumor and whether there is metastatic
disease.
The classical presentation in a3 4 years old is a
pale, irritable and orbital ecchymoses, whilst in
infant may present with a grossly enlarged liver
with subcutaneous nodules.
Primary tumor:
Coinciding with normal sympathetic nervous
system
structure,
for
example
adrenas,
abdominal para gangglia, and sympathetic chain
About 25% of primaries are foun in the neck or
thorax,70% in the abdomen and 5% in the pelvic.

Clinical presentation (cont)

A hard, fixwd ,abdominal mass causing only

mild abdominal discomfort.
Hypertension can result from compression of
the renal vessels by the tumor.
Gastrointestinal symptoms are rare.
Manifestation on cervical region only as a mass
Mediastinal mass cause of cough and superior
mediastinal obstruction.
Tumor can extend into the neural foramina and
compress nerve roots and spinal cord,
resulting: radicular pain; paraplegia and bowel
and bladder symptoms.

Diagnosis and staging

Diagnostic criteria
International Neuroblastoma Staging
System (INSS) working party have been
clearly that diagnosis of neuroblastoma
by a tissue biopsy, with a histological
appearance of neuroblastoma or by the
presence of a non-haemopoietic tumor
in the bone marrow , together with
raised urinary cathecolamines. In the
bone marrow , neuroblastoma often has
the appearance of pseudorosettes.

Staging system

INSS for Neuroblastoma

Stage 1

Localized tumor with complete gross excision,with or wthout

microscopic residual disease.

Stage 2a

Localized tumor with incomplete gross excision;representative

ipsilateral and non adherent lymp node negative for tumor
microscopically

Stage 2b

Localized tumor with complete or incomplete gross

excision;with ipsilateral non adherent lympnone positive
tumor.Enlarged contralateral lymph none must be negative
microscopically

Stage 3

Unresectable unilateral tumor infiltrating across the midline

with or without regional lymp node involvement;or localized
tumor with contralateral regional lympnode involvement; or
mild line tumor with bilateral extension by infiltration or by
lymp node involvement

Stage 4

Any primary tumor with dissemination to distant lymp node,

bone, bone marrow, liver, skin and/or others organ

Stage 4s

Localized primary tumor with dissemination limited to skin,

liver and/or bone marrow