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PNET/ESFT of the cranial vault: a case report
M Mellai1, V Caldera, A Comino
1Neuro-bio-oncology Center, Policlinico di Monza Foundation, University of Turin (Vercelli), U.O. Neurochirurgia, Cuneo, Italy.
Abstract:
A case of peripheral PNET (PNET/ESFT) of the cranial vault is described. A 56-year-old woman showed a mass with a large cyst in the right temporal region, adherent to the meninges, which caused a left hemiparesis with headache and confusion. The mass was totally removed. The histological examination showed a dense proliferation of small elements, organized in lobules separated by reticulin septa. Many circumscribed necroses, vessels with a thick handcuff of reticulin, a diffuse mucous degeneration and abundant mitoses were present. The cells were positive for Vimentin and CD99. RT-PCR revealed the EWS/FLI1 fusion transcript of the t(11,22) (q24;q12) translocation. The patient presented is the oldest one of the rare cases of dura-based meningioma-mimicking pPNETs till now described. In line with the possible origin from peripheral nerves or roots of cauda equina of non-intracranial tumors, those of the vault may derive from peripheral sensory nerves of the dura. The differential diagnosis must be made with cPNETs which show a worse prognosis and both can benefit from a different chemotherapy.
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