Intracranial Peripheral Primitive Neuroectodermal Tumor Arising from the Clivus with Intracranial Metastasis in an...

Intracranial peripheral primitive neuroectodermal tumors (pPNETs) are rare lesions, accounting for only 1% of all pPNETs, and usually arise from bone and soft tissue. These tumors primarily affect young patients (<30 years old) and rarely affect older patients. The common intracranial locations of these lesions are frontal dura and tentorium. These lesions rarely may involve the cerebellopontine angle, cavernous sinus, and jugular foramen. Involvement of clivus has not been reported to date. Intracranial metastasis from primary intracranial pPNET is a further rare occurrence.

A 74-year-old woman presented with gradually progressive painless diminution of vision in both eyes alongwith headache for 6 months duration. Radiology demonstrated a clival mass as well as a suspected intracranial metastasis. Histopathology was suggestive of pPNET. We also present a brief review of the literature on pPNETs.

pPNETs of the clival region are exceptionally rare. In elderly patients with clival mass lesions, the rare possibility of pPNET should be kept in mind.