- 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