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.