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| Primitive NeuroectodermalTumor | ||
| Etiology •Unknown | ||
| Pathogenesis •Unknown , | ||
| Epidemiology •Tumor is rare, representing less than 1% of all sarcomas. •May occur at any age; three quarters of cases appear before 35 years of age. | ||
| General Gross Description •Also known Ewing' sarcoma or peripheral neuroepithelioma. •Large fleshy mass with areas of hemorrhage and necrosis. •Examples: | ||
| General Microscopic Description •Microscopically, the tumors resemble neuroblastomas. •They are composed of sheets of small round cells that resemble lymphocytes. •The cytoplasm is indistinct. •Diagnostic features include the presence of rosettes. •Tumors express neuron-specific enolase, and are characterized by other neuron-specific markers. •Examples: | ||
| Clinical Correlation •Mostly present as a mass attached to a nerve. •As a result of attachment to nerve, neurological symptoms are often a presenting feature. •The tumors are highly aggressive, and rapidly give rise to metastases and death. •Survival at 3 years has been reported at 50% or less. | ||
| References • Cotran RS, Kumar V, Robbins SL: Robbins Pathologic Basis of Disease. 5th edition. Philadelphia, W.B. Saunders, 1994, pp. 1244. Please be patient during transfer. Medline will open in a new window. To return, close the Medline Window Primitive NeuroectodermalTumor
| Synopsis by: T.V. Rajan M.D. Ph.D. (T1X100M92603)[88]
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