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    Neuroblastoma is a childhood solid tumor that arises from neural crest cells. It has a wide range of clinical behaviors, from spontaneously regressing to highly resistant to chemotherapy. Metastasis and relapse are associated with the worst prognosis. Diagnosis is based on biopsy and urinary catecholamines. The International Neuroblastoma Staging System (INSS) stages neuroblastoma from 1 to 4, with stage 4 being disseminated disease and stage 4s indicating localized disease with limited metastasis. Treatment is based on patient and tumor characteristics.

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    Neuroblastoma

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    Neuroblastoma is a childhood solid tumor that arises from neural crest cells. It has a wide range of clinical behaviors, from spontaneously regressing to highly resistant to chemotherapy. Metastasis and relapse are associated with the worst prognosis. Diagnosis is based on biopsy and urinary catecholamines. The International Neuroblastoma Staging System (INSS) stages neuroblastoma from 1 to 4, with stage 4 being disseminated disease and stage 4s indicating localized disease with limited metastasis. Treatment is based on patient and tumor characteristics.

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    107 views10 pages

    Neuroblastoma

    Uploaded by

    dita
    Neuroblastoma is a childhood solid tumor that arises from neural crest cells. It has a wide range of clinical behaviors, from spontaneously regressing to highly resistant to chemotherapy. Metastasis and relapse are associated with the worst prognosis. Diagnosis is based on biopsy and urinary catecholamines. The International Neuroblastoma Staging System (INSS) stages neuroblastoma from 1 to 4, with stage 4 being disseminated disease and stage 4s indicating localized disease with limited metastasis. Treatment is based on patient and tumor characteristics.

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    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

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