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- Discussion:
    - most common tumor that causes skeletal metastasis in children;
    - frequency of neuroblastoma is about 1 per 100,000 persons;
    - originates in neural-crest cells of sympathetic nervous system;
    - majority of patients have tumor involvment of regional lymph nodes or have metastasis to bone at time of diagnosis;
          - only 20% of children present w/ local or regional disease;
    - skeletal involvement is usually diffuse;

- Presentation:
    - neuroblastoma may originate anywhere along sympathetic nervous system;
    - typically presents w/ abdominal mass in 2 year old child arising either in adrenal gland (40%) or in a paraspinall ganglionn (25%);
    - if bones are involved, bone pain may be present;
    - bone marrow involvement may cause pallor (anemia), petechiae (thrombocytopenia), and leukopenia;

- Radiographs:
    - small, punctate osteolytic lesions with poorly defined borders involve skull, ribs, and long-bone metaphyses;

- Labs:
    - anemia,  thrombocytopenia, and leukopenia;
    - elevated urinaryy vanillylmandelic acid (VMA) is diagnostic;

- Histology:
    - neuroblastoma is one of "small blue round cell tumors" of childhood
    - in its primative form it consists of dense nests of cells separated by fibular bundles;
    - frequently demonstrates hamorrhage, necrosis, and calcification;
    - characteristic finding is the presence of rosettes in which tumor cells surround a pink fibrillar center;

- Treatment:
    - infants under one year;
         - generally can be cured regardless of disease stage;
    - older children:
         - older patients with advanced disease have a poor prognosis in all treatment programs;
                - most pts > 1 yr of age,  present w/ metastatic dz of liver, lung, or bone;
                - with regional lymph-node involvement or metastases at  presentation, less than 20% will survive;
    - surgical excision of localized primary lesions provides best chance for cure;
    - chemotherapy and XRT may be used for advanced dz

Spinal deformity in children treated for neuroblastoma.

Radionuclide skeletal survey in neuroblastoma.

Chromosome 6p22 Locus Associated with Clinically Aggressive Neuroblastoma

Neuroblastoma — Linking a Common Allele to a Rare Disease

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