A case of fatal late vasospasm in a patient with a recurrent, supratentorial, rhabdoid primitive neuroectodermal tumor: possible molecular implications.

Abstract

We report the case of a 44-year-old man who experienced a fatal and untreatable delayed vasospasm after resection of a recurrent temporal IV grade primitive neuroectodermal tumor (PNET). The histological analysis demonstrated a rare rhabdoid variant of the tumor with a diffuse myxoid degeneration; molecular investigations demonstrated an upregulation of IL-1β and IL-6 expression in the recurrence.We reviewed the pathophysiology of the vasospasm that occurs after tumors resection, and due to the rarity of case, we speculated on the possibility that specific histological and molecular features of the tumor could have contributed to the delayed and fatal complication.

Low grade astrocytoma transformating primitive neuroectodermal tumour in an adult? A case report and review of literature

“…An unusual somatic p53 mutation of a type specific to radiationinduced DNA damage in a secondary glioblastoma identified by Tada [12]. Also IL-1βm-RNA was seven-fold higher in the recurrent tumor of PNET and it could have triggered the fatal vasospasm [19]. Signaling pathways, sonic hedgehog (Shh), Wnt, and Notch pathways, especially the Shh pathway, are considered playing an important role in the pathogenesis and biological behavior of desmoplastic PNET [20].…”