Nervous System in Children
Online ISSN : 2435-824X
Print ISSN : 0387-8023
Long-term outcome of and prognostic factors for the primary central nervous system germ cell tumors
Fumiyuki YamasakiYasuyuki KinoshitaSatoshi UsuiTakeshi TakayasuMotoki TakanoUshio YonezawaAkira TaguchiSayuru TsuyuguchiAtsushi TominagaKaoru KurisuKazuhiko Sugiyama
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JOURNAL FREE ACCESS

2020 Volume 45 Issue 4 Pages 346-350

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Abstract

Background: Central nervous system (CNS) germinomas have favorable cure rates. However, long-term follow-up data are limited because of the rarity of these tumors. A recent report based on the SEER database investigated CNS-germ cell tumors (GCT) and showed that the 5-year survival outcomes of patients who developed germinomas were worse compared to that for patients with nongerminomatous germ cell tumors (NGGCT). In this study, we evaluated the long-term follow-up outcomes of germinoma and NGGCT survivors.

Materials and Methods: Our institutional review board approved this retrospective study. Between 1985 and 2018, 66 patients with germinomas (male: female = 55: 11, ages: 9-39, mean: 18, median: 15) were treated at Hiroshima University Hospital. In the same period, 20 patients with NGGCTs (male: female = 20: 0, ages: 5-51, mean: 18, median: 11) were treated in our institution. Long-term follow-up data of all patients was obtained from the medical records.

Results: The long-term follow-up on patients with germinomas was performed up to 28 years (median: 12 years) with a 10-year survival rate of 96% and a 20-year survival rate of 87%. The follow-up data for patients with NGGCT was up to 25 years (median: 6 years) with a 10-year survival rate of 85% and a 20-year survival rate of 85%. Factors that affected the course of treatment included progressive disease and associated teratoma syndrome, which resulted in decreased treatment intensity. The factor affecting the long-term outcome and quality of life in patients with GCTs was the site of tumor development. Those with multiple lesions/dissemination at the onset and neurohypophyseal location tended to have poor prognoses, and those with basal ganglia involvement tended to have a poor quality of life owing to cognitive impairment. Overall, four patients had secondary cancers and six patients experienced recurrence following germinoma treatment; one patient died from an adrenal crisis.

Conclusion: Long-term prognosis and the quality of life of patients are affected by tumor site. It is important to select an appropriate treatment intensity based on an accurate diagnosis. Multidisciplinary treatment that includes appropriate hormone replacement, obesity prevention and treatment for lifestyle-related diseases should be considered for patients with germinomas and NGGCT survivors.

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© 2020 The Japanese Society for Pediatric Neurosurgery
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