Where do primitive neuroectodermal tumors (PNETs) most commonly manifest?

Updated: Feb 07, 2019
  • Author: Carlo P Honrado, MD, FACS; Chief Editor: Arlen D Meyers, MD, MBA  more...
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Answer

Answer

Most peripheral primitive neuroectodermal tumors (pPNETs) manifest in the thoracopulmonary region (Askin tumor), pelvis, abdomen, and extremities. In a large series of 26 cases, Jones and McGill reported 11 of 26 patients with disease in the head and neck. [10] Most other large published series, however, reveal a paucity of cases in the head and neck region. [11, 12, 13] Of the published cases involving the head and neck, the sites of presentation are diverse and include the paranasal sinuses, jugular foramen, oral cavity, nasal cavity, neck, skull, lingual nerve, parotid gland, larynx, retropharyngeal space, maxilla, mandible, masseter, temporal area, pterygomaxillary space, esophagus, and orbit.


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