The good prognosis of those rare cases with pPNET and embryonal neuroblastomas
elucidates questions concerning the tumor's biological behaviour and role of different
factors affecting cell differentiation, tumour growth and dissemination.
Primitive neuroectodermal tumor (PNET) is a small round cell malignancy of presumedly primitive, neuroectodermal tissue or pluripotential, migratory neural crest cells arising from the soft tissue or bone, predominantly in older children and adults. The term, “PNET” includes malignant small round cell tumors of the thoracopulmonary region (Askin's tumor), extraskeletal Ewing's sarcoma, peripheral neuroblastoma, and peripheral neuroepithelioma.
Neuroblastoma is the most common extracranial solid tumor of infancy. It is an embryonal malignancy of the sympathetic nervous system arising from neuroblasts (pluripotent sympathetic cells). In the developing embryo, these cells invaginate, migrate along the neuraxis, and populate the sympathetic ganglia, adrenal medulla, and other sites
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