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| Adrenal Neuroblastoma |
| Etiology • May be congenital |
| Pathogenesis • Loss of neuroblastoma suppressor gene on distal short arm of chromosome 1 • Amplification of N-myc oncogene (seen in cases that are more aggressive), |
| Epidemiology • Disease of children generally < 5 yrs. of age • May be congenital |
| General Gross Description • Soft • Tan with areas of hemorrhage and necrosis |
| General Microscopic Description • Small cells with scant cytoplasm • Hyperchromatic nuclei • May be in sheets or nests • Homer-Wright pseudo-rosettes may surround neuropil (stroma like that of white matter) • Cells are positive with neuroendocrine markers such as NSE, chromogranin, and synaptophysin |
| Clinical Correlation • Present with bone pain, mass, or symptoms attributed to excess catechols • Usually metastatic disease at presentation • Prognosis related to extent of disease and improved by age <1 yr. • Hyperdiploidy associated with better prognosis; N-myc amplification with poor outcome • Treatment with chemotherapy |
| References • Rosai J. Ackerman's Surgical Pathology. 8th ed. St. Louis, Mosby-YearBook, 1996, pp. 1026-1033. |
| Adrenal Neuroblastoma |
| Synopsis by: Melinda Sanders M.D. (T93000M95003)[158] |
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