Neuroblastoma 4xn1t

Cancer in Children

NEUROBLASTOMA

Introduction  

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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 

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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 

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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