Abstract
Primary intracranial primitive neuroectodermal tumors (PNETs) are
extremely rare malignancies, which affects children and adolescents with
only 10 cases has been reported over 33 years old. They have an
aggressive behavior and a high tendency for local recurrence and distant
metastasis. Here we present a case of supratentorial PNET which occurred
in left frontoparietal lobe of a 36 years old female patient with the
chief complaint of aphasia for 2 month duration. Aphasia is being
reported for the first time as a consequence of a PNET brain tumor.
T1-weighted MRI showed a large mass with mixed isointense to hypointense
signals. The tumor was completely removed. Histopathologic examination
was indicative of a small round cell tumor. Immunohistochemical analysis
showed positivity for CD99. Presence of EWSR1 gene rearrangement
confirmed the diagnosis. The patient’s aphasia gradually resolved post
operation. Six months follow-up showed no evidence of local recurrence
or metastasis.