Incidence of Primary Central Nervous System Germ Cell Tumors in Childhood: A Regional Survey in Kumamoto Prefecture in Southern Japan

Abstract

Background/Aims: Central nervous system germ cell tumors (CNS-GCTs) are relatively rare. While their incidence was thought to be higher in East Asia than the USA, recent evidence suggests the difference between Japan and the USA is not statistically significant. The aim of this study was to determine the rate of pediatric primary CNS-GCTs in Kumamoto prefecture, Japan. Methods: We surveyed 6,615 new cases of primary intracranial tumors diagnosed in Kumamoto prefecture between 1989 and 2011. Among these, 251 (3.8%) occurred in patients younger than 15 years. The age-adjusted incidence rates were calculated by the direct method using 5-year age groupings; the incidence in the total Japanese population in the year 2000 was the standard. Results: During the 23-year period, 70 cases of primary GCT were diagnosed. Of these tumors, 31 (44.3%) arose in patients aged between 0 and 14 years (22 boys, 9 girls). Their tumor location was pineal in 45.2%; the other sites were nonpineal. There were more germinomas (64.5%) than nongerminomas (35.5%) in this group. The age-adjusted annual incidence rate was 0.45 cases (boys: 0.64, girls: 0.28) per 10⁵ children. At 2.29, the ratio of CNS-GCTs was higher in these boys than girls. Our data showed higher rates than data from CBTRUS 2012 (0.18/10⁵), SEER 2008 (0.15/10⁵) and Germany (0.10/10⁵). Conclusions: Our survey showed that the incidence of primary CNS-GCTs in children was higher in Kumamoto prefecture than in the USA and other Western countries, suggesting that racial backgrounds play a role.

Nationwide Population-Based Incidence and Survival Rates of Malignant Central Nervous System Germ Cell Tumors in Korea, 2005-2012

“…Goodwin et al [21] also described differences in the incidence of pediatric GCTs according to race (Caucasian, 1.29 per 100,000; African-American, 0.33 per 100,000; and Asian, 2.6 per 100,000). Moreover, the childhood incidence of primary CNS GCTs in a regional survey in Kumamoto Prefecture in southern Japan was found to be 0.45 per 100,000 [4]. These data suggest that an Asian genetic background is strongly associated with the development of CNS GCT.…”

Critical review of the management of primary central nervous nongerminomatous germ cell tumors

“…Central nervous system (CNS) germ cell tumors (GCT) constitute ≤4% of all primary CNS tumors among individuals under 15 years of age in the United States . CNS‐GCT are more common among males, individuals younger than 20 years old, and East‐Asian populations …”

A case of germinoma located in the fornix inducing transsynaptic atrophy of the Papez circuit

“…Germinoma is the most common subtype of CNS germ cell tumors and estimated in the annual incidence rate of 0.12 cases per 100,000 population aged under 15 in Japan [4] . Germinomas are thought to originate from primordial germ cells entrapped during embryonic migration and predominantly arise from the midline structures such as the pineal gland and neurohypophysis.…”