ESPE Abstracts

Background:In children and adolescents suffered form primary intracranial germ cell tumors, bifocal GCTs are rare and were reported a bad prognosis. In this study, we report a case and summarize the clinical characteristics and prognosis.

Methods: A boy suffered form bifocal GCTs (Basal ganglia and pineal region) was diagnosed in our hospital and accepted chemotherapy+radiotherapy, and was followed up.

Results: 1. A 8 year and 2 month old boy came to our hospital because of enlargement of the penis for 5 months with pubic hairgrowth. Facial acne, beard, depening of the voice, gynecomastia and enlarged penis(9 cm *3 cm) were found. Bilaterallytesticular volume :8mL. Tanner stage of pubic hair: stage 3. No headache, nausea or vomiting, and no abnormal signs ofnervous system. 2. Laboratory data: Basal FSH and LH levels decreased, with the elevated serum testosterone levels: 9.35ng/ml. And the gonadotrophin-releasing hormone agonist(GnRHa) stimulation test showed GnRH independent precocious puberty. Serum β-human chorionic gonadotropin(β-hCG) increased (22.3mIU/ml), along with elevated cerebrospinal fluid (CSF) β-hCG (21.31mIU/ml), but both serum and CSFlevels of the AFP were normal. MRI scans revealed bifocal germ cell tumors located in the pineal gland and basal ganglia. No other extracranial metastases were found. When compared to other primary solitary intracranial GCTs cases(published before by our team), no differences of the serum and CSF β-hCG levels, serum testosterone levels and clinical manifestations were found. 3. The boy accepted regular chemotherapy and radiotherapy. After one course of chemotherapy, the serum levels of β-hCG, as well as the CSF β-hCG levels decreased significantly. Serum testosterone levels returned normal.

Conclusion: Peripheral precorcious puberty is usually the first or one of the typical symptoms in boys with hCG-secreting GCTs. There are no differences in clinical manifestations between solitary and bifocal primary intracranial tumors. Patients with basal ganglia lesions do not necessarily have neurological symptoms. The combination of the detection serum hCG and CSF hCG levels may be significant for early diagnosis, the determination of the tumor locations.