Abstract

We experienced a rare case of primary intracranial choriocarcinoma treated successfully with synchronous chemotherapy and radiotherapy followed by three consecutive courses of chemotherapy without surgery. A 19-year-old male patient presented with a two-week history of diplopia, headache, nausea and vomiting. Neurological examination revealed visual disturbance, bilateral hearing loss, bilateral sixth nerve palsy, left skew deviation, downgaze limitation and clockwise torsional nystagmus on the left upgaze. During image evaluation, the patient suddenly presented with a confused mental status, bradycardia and decreased respiration. An emergency third ventriculostomy was conducted, followed by synchronous ifosfamide, cisplatin and etoposide chemotherapy (ICE; I, 900 mg/m(2); C, 20 mg/m(2); and E, 60 mg/m(2)) on days 1-5, and external radiotherapy (whole brain, 30 Gy/15 Fxs; local boost, 30 Gy/15 Fxs) followed again by three consecutive courses of chemotherapy. This therapy resulted in tumor regression by 63% and full improvement in the patient's neurological condition. However, it was difficult to remove the remaining tumor using a surgical approach due to the risk of postoperative hemorrhage and limited access. We planned to observe the remaining mass without surgery. This patient is now alive and the mass size has not changed for 18 months since treatment. This is the first report of the effectiveness of synchronous chemotherapy and radiotherapy followed by consecutive chemotherapy without surgery in a patient with primary intracranial choriocarcinoma.

Highlights

  • Primary intracranial germ cell tumors (GCTs) are associated with many unanswered questions due to their low incidence [1]

  • While germinomas may be cured by chemotherapy and/or radiotherapy, non‐germinatous malignant GCTs are highly resistant to standard treatments, and their prognosis is extremely poor

  • A statistical analysis report published by the Committee of Brain Tumors Registry of Japan indicated that the incidence of choriocarcinoma was 3.2% (36 cases) out of 1127 primary intracranial GCT cases [5]

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Summary

Introduction

Primary intracranial germ cell tumors (GCTs) are associated with many unanswered questions due to their low incidence (approximately 1.8‐3.0% of all primary brain tumors) [1]. The patient underwent synchronous ifosfamide, cisplatin and etoposide chemotherapy (ICE; I, 900 mg/m2; C, 20 mg/m2; and E, 60 mg/m2) on days 1‐5, and external radiotherapy (whole brain, 30 Gy/15 Fxs; local boost, 30 Gy/15 Fxs) followed by ventriculoperitoneal CSF shunting. Following completion of the first course of synchronous chemotherapy and radiotherapy, the serum β‐HCG and AFP were normalized at 0.2 mIU/ml and 2.2 ng/ml, respectively (Fig. 2). Imaging studies (CT and MRI) demonstrated an ~45% reduction in the tumor mass (21x16 mm) and disappearance of the hydrocephalus (Fig. 1B). We aimed to observe the remaining mass without surgery, and since the patient has undergone regular CT/MRI follow‐up as well as serum β‐HCG and AFP level examinations every three months. The patient is alive, the mass size has not changed for 18 months since treatment, and he is leading a normal life

Discussion
The Committee of the Brain Tumor Registry of Japan
12. Kyritsis AP
Findings
14. Yamamoto I
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