Primary intracranial choriocarcinoma: a report of 8 cases with review of literature

Objective: Primary intracranial choriocarcinoma is a rare intracranial neoplasm. We discuss its clinical features and treatment in a pediatric population. Methods: We retrospectively studied choriocarcinoma cases in our center from May 2002 to January 2015. Eight patients were diagnosed with primary intracranial choriocarcinoma and included. Preand post-operative CT and MRI were examined and blood serum and cerebrospinal fluid (CSF) were sampled to measure alpha-fetoprotein (AFP), carcinoembryogenic antigen (CEA), and human chorionic gonadotrophin (HCG). Results: All patients were male, aged between 5-17 years, (mean 11 years). The major presenting symptoms were headache, and dizziness with vomiting. All patients presented with hydrocephalus. The duration of onset was 10 days to 3 months; five patients had eye movement disorder and diplopia. Two patients had precocious puberty; and one patient had diabetes insipidus. HCG was elevated in all patients (serum level ranged from 119012000 IU/L CSF level ranged from 990-4651.56 IU/L). Three patients received preoperative radiotherapy. Eight patients received ventriculo-peritoneal shunts immediately after admission. Seven patients underwent surgery the remaining patient received chemotherapy with radiotherapy. Seven patients received postoperative chemotherapy. Hemorrhage and tumor growth occurred in patients who underwent preoperative radiotherapy. Follow-up was from 1 to 60 months; three patients died during follow-up and four survived. The mean survival time was 23.6 months. Conclusions: Extremely elevated HCG levels in serum and CSF may help diagnosis of primary intracranial choriocarcinoma. Based on these cases, we are concerned that preoperative radiotherapy may increase the hemorrhage occurrence. Surgery combined with chemotherapy may be a more effective treatment.