Introduction
Primary intracranial primitive neuroectodermal tumors (PNETs) are
extremely rare malignancies that usually originate from the meninges and
show varying degrees of neuroectodermal differentiation [1-4]. About
66 cases of these tumors have been reported so far. PNETs are poorly
differentiated, highly malignant, and aggressive small round cell
neoplasms. They had a high propensity for local recurrence and distant
metastasis. Intracranial PNETs commonly affect children and adolescents
[1, 2, 5]. It is noteworthy that the incidence of PNETs in adults is
exceedingly rare. Only about ten cases of these tumors have been
reported over the age of 33 years [1, 2]. Fusion of the EWSR1 gene
with a member of the gene family is considered the primary cause of the
PNETs [1, 4]. Due to the scarcity of cases of patients with PNETs,
the clinical and imaging features, treatment and prognosis are not clear
[1, 2].