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Primitive Neuroectodermal Tumor


D efinition — A rare, poorly differentiated embryonal neuroectodermal tumor sometimes associated with a teratoma that mimics the central type of primitive neuroectodermal tumor (PNET).

Clinical Features

Epidemiology

  • Extremely rare in the ovary.

  • Belongs in the Ewing's family of tumors

  • Ovarian tumors arise from neural tissue and are analogous to the “central” PNETs as opposed to the peripheral PNETs that contain a unique t(11:22)(q24;q12) translocation specific for the PNET/Ewing's sarcoma family.

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