Objective: Primary intracranial choriocarcinoma (PICCC)/germ-cell tumors (GCTs) with high levels of human chorionic gonadotropin (HCG) (PICCC/GCTs with HL-HCG) are rare and malignant. The goal of this study was to report our 3 cases of PICCC/GCTs with HL-HCG and to review the literature to elucidate the clinical problems and prognostic factors and to discuss the therapeutic modalities of this rare tumor.
Methods: Survival was analyzed in 66 previously reported PICCC/GCTs with HL-HCG including our 3 cases, of which the clinical results have been described in the literature since 1975. In the 66 cases (mean age: 12.1 years; male/female: 45/21), 35 were verified histologically as pure choriocarcinoma, 23 were as mixed GCTs with choriocarcinoma element, and 8 were not verified as including choriocarcinoma element histologically but with very high HCG levels. Significance of the differences among survival curves concerning each parameter (age, sex, tumor location, serum HCG/beta-HCG level, precocious puberty, extent of surgery, radiotherapy, chemotherapy, mixture of other non-germinomatous GCT elements and extraneural metastasis) was tested using univariate and multivariate analyses.
Results: The median survival time and the 1- and 2-year survival rates were 22 months, 61.2% and 49.8%, respectively. In univariate analysis, male, subtotal removal or more, radiotherapy and chemotherapy were revealed to be significantly good prognostic factors. However, suprasellar region and tumor hemorrhage were poor prognostic factors. Multivariate analysis showed that extent of surgery, radiotherapy and chemotherapy were independent prognostic factors.
Conclusions: Although, we should mind the limitations of this study design because of case selection bias, different treatment protocols and incomplete follow-up of patients, this study led the following results and suggestive conclusions. Tumor hemorrhage and progressive extraneural and cerebrospinal fluid metastasis were characteristic clinical problems of PICCC/GCTs with HL-HCG. In the cases with extremely elevated levels of HCG, biopsy for histological diagnosis may be no longer needed. Initial biopsy and radiotherapy may lead to tumor hemorrhage. To prevent tumor hemorrhage, gross tumor removal followed by radiotherapy and chemotherapy should be aimed for. A few courses of chemotherapy before surgery may prevent metastasis. Stereotactic radiotherapy and high dose chemotherapy may be promising options for treatment.