Central nervous system (CNS) germ cell tumors (GCTs) are rare malignant neoplasms that arise predominantly in adolescents and young adults. CNS GCTs demonstrate characteristic trends in national associations, with implications for both tumor incidence and genetics. Although the incidence of CNS GCTs is markedly higher in East Asia than Western countries, direct comparative analyses between these CNS GCT populations are limited.
In Japan, to facilitate the genomic analyses of CNS GCTs, the Intracranial Germ Cell Tumor Genome Analysis Consortium was established in 2011, and more than 200 cases of GCTs are available for both tumor tissue and clinical data, which is organized by the National Cancer Center (NCC) Japan. At the Mayo Clinic, there have been 98 cases of intracranial GCTs treated by the Department of Neurologic Surgery since 1988. In this paper, the authors compared the epidemiology, clinical presentation including location and histology, and prognosis between cases treated in the US and Japan.
There was no significant difference in age and sex distributions between the databases. However, there was a significant difference in the tumor locations; specifically, the frequency of basal ganglia was higher in the NCC database compared with the Mayo Clinic (8.4% vs 0%, p = 0.008), and bifocal location (neurohypophysis and pineal gland) was higher at the Mayo Clinic than at the NCC (18.8% vs 5.8%, p = 0.002). There was no difference in histological subdivisions between the databases. There was no difference in progression-free survival (PFS) and overall survival (OS) of germinoma cases and OS of nongerminomatous GCT (NGGCT) cases treated with chemotherapy and radiation therapy covering whole ventricles. However, PFS of NGGCTs differed significantly, and was better in the NCC cohorts (p = 0.04).
There appears to be a differential distribution of GCTs by neuroanatomical location between major geographic and national groups. Further study is warranted to better characterize any underlying genomic, epigenetic, or environmental factors that may be driving the phenotypic differences.
ABBREVIATIONSAFP = alpha-fetoprotein; BTRJ = Brain Tumor Registry Japan; CARE = carboplatin + etoposide; CNS = central nervous system; COG = Children’s Oncology Group; GCT = germ cell tumor; HCG = human chorionic gonadotropin; ICE = ifosfamide + carboplatin + etoposide; iGCT Consortium = Intracranial Germ Cell Tumor Genome Analysis Consortium; NCC = National Cancer Center; NGGCT = nongerminomatous GCT; NOS = not otherwise specified; OS = overall survival; PE = cisplatin + etoposide; PFS = progression-free survival; PGC = primordial germ cell; PRISMA = Preferred Reporting Items for Systematic Reviews and Meta-Analysis; WB = whole brain; WV = whole ventricle.