Increased Survival Associated with Experience-based Treatment in Primary Intracranial Yolk Sac Tumor and a systematic review

OBJECTIVE Primary intracranial yolk sac tumors are rare neoplasms with poor prognosis. The study aimed to identify adverse factors affecting survival and to propose an appropriate treatment. METHODS We reviewed the clinical data of 68 patients with primary intracranial yolk sac tumors in our cohort and performed a systematic review of 28 cases published between 1976 and 2019. RESULTS Our series included 54 male (79.4%) and 14 female (20.6%) patients, with a median age at diagnosis of 12 years. Twenty-eight patients experienced recurrence, 6 patients developed metastasis, and 28 patients died at the final follow-up. Multivariate analysis revealed that surgery combined with chemotherapy and radiation significantly increased both progression-free survival (p<0.001) and overall survival (p<0.001). As noted in our prior literature, 10 patients developed recurrence, 4 patients experienced metastasis, and 7 patients died during their follow-up time. In the pooled cases, multivariate analysis revealed that surgery with chemotherapy and radiation significantly prolonged progression-free survival (p<0.001). and increasing age (p=0.009) and surgery with chemotherapy and radiation (p<0.001) significantly increased overall survival. CONCLUSIONS Surgery plus adjuvant therapy was a favorable factor for both progression-free survival and overall survival in patients with yolk sac tumors.