BACKGROUND & OBJECTIVE: Primary central nervous system (CNS) germ cell tumors (GCTs) are rare malignant neoplasms with various histological types. Excluding pure germinoma and mature teratoma, other types carry a poor prognosis. Previous investigations focused on combined modality treatment including chemotherapy to improve survival. This study was to analyze the efficacy and toxicity of chemotherapy combined with surgery and/or radiotherapy on CNS GCTs.

METHODS

A total of 23 patients with CNS GCTs were treated in Cancer Center of Sun Yat-sen University from May 2002 to Jun. 2006. The median age at diagnosis was 16 years. Alpha-fetoprotein (AFP) and/or beta-human chorionic gonadotropin (beta-HCG) levels were elevated in 19 patients (82.6%). All patients were treated with chemotherapy of PEB regimen combined with surgery and/or radiotherapy. PEB regimen was administered every 3 weeks with 20 mg/m(2) cisplatin (DDP) at Days 1-5 or 80-100 mg/m(2) at Day 1, 60-100 mg/m(2) etoposide (VP-16) or teniposide (VM-26) at Days 1-5, 10 mg/m(2) bleomycin (BLM) at Days 1 and 5.

RESULTS

Of the 23 patients, 17 newly diagnosed patients received induction chemotherapy followed by radiotherapy or surgery/radiotherapy followed by adjuvant chemotherapy; 6 recurrent patients received salvage chemotherapy, of which 3 patients with disseminated tumor received salvage chemotherapy followed by craniospinal irradiation. The 23 patients completed a total of 61 cycles of PEB regimen with a median of 3 cycles. Chemotherapy alone gained a response rate of 87.0% and a complete remission rate of 30.4%. Craniospinal irradiation was performed in 14 patients and focal irradiation in 8 patients. One patient did not receive irradiation. Incomplete tumor resection or biopsy was performed in 13 patients. Fourteen patients (60.9%) were alive without evidence of diseases after combined modality treatment. With a median follow-up of 24 months, the 2-year survival rate was 67.4%. Main adverse events were late irradiation injury including aplastic anemia and hypothalamus syndrome (1 case), irradiation encephalopathy (1 case), and hypopituitarism (2 cases).

CONCLUSIONS

The combined modality treatment including PEB regimen is highly effective in treating CNS GCTs patients, especially in the patients with elevated tumor markers. However, the long-term toxicities which related with craniospinal irradiation should not be ignored.