Intracranial Primitive Neuroectodermal Tumors: A Retrospective Systematic Study Of Clinical Characteristics In 10 Patients

Objective To discuss the clinicopathological features,therapy,outcome,and prognosis of cen-tral primitive neuroectodermal tumors(PNET)so as to improve diagnostic and therapeutic levels.Background Intracranial primitive neuroectodermal tumors(PNETs)were a rare variant of malignant tumor.The study aims to retrospectively analyze the clinical characteristics of PNET treated in Qilu Hospital during 10 years.Methods A total of 10 patients with PNET treated with operation in our department during a 10-year period were identified.Clinical records and follow-ups were reviewed.Results Intracranial PNETs patients tended to have a predilection toward younger patients at the first diagnosis with mean and median age of 30.4 and 34.5 years,respectively.And there was obvious male preponderance with male to female ratio of 9:1.The most frequently affected location was convexity(6/10,60%).The most common symptom was headache(7/10,70%),and other lesser common symptoms were dizziness(4/10,40%),nausea and vomiting(4/10,40%),aphasia(1/10,10%),absence seizure(1/10,10%),and version loss(1/10,10%).The postoperative follow-up period varied from 10 to 25 months(median of 14.5 months).Death was noted in three patients after 12,20 and 12 months of surgery,respectively.Seven other patients all had obtained more relative satisfactory treatment effect without any further recurrences.Conclusions Intracranial PNETs were a rare variant of malignant tumors.The tumors were usually seen in younger patients and have a male predilection.Convexity was the most frequently affected location,and the most common symptom was headache.Histopathological examination and immunohistochemistry was considered the gold standard diagnosis.Maximal surgical resection with postoperative adjuvant treatment,including radiotherapy,and chemotherapy,should be an optimal therapeutic modality.