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Primitive neuroectodermal tumor

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 as primitive neuroectodermal tumor; Ewing' sarcoma or peripheral neuroepithelioma.

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.

Clinical Correlations: • 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: •Robbins "Pathologic Basis of Disease". (Cotran, Kumar and Robbins, Eds.) 5th Edition. pp 1244.