{"title":"Long-Term Outcome and Social-Intellectual Ability of Patients With Basal Ganglia Germinoma","authors":"Michinari Okamoto MD, PhD, Shigeru Yamaguchi MD, PhD, Ryosuke Sawaya MD, PhD, Yukitomo Ishi MD, PhD, Hiroaki Motegi MD, PhD, Yukayo Terashita MD, Minako Sugiyama MD, Yuko Cho MD, Kentaro Nishioka MD, PhD, Takashi Mori MD, PhD, Takayuki Hashimoto MD, PhD, Hidefumi Aoyama MD, PhD, Atsushi Manabe MD, PhD, Miki Fujimura MD, PhD","doi":"10.1016/j.pediatrneurol.2025.01.009","DOIUrl":null,"url":null,"abstract":"<div><h3>Background</h3><div>In this study, we aimed at delineating the still ambiguous clinical characteristics and long-term outcomes of basal ganglia (BG) germinoma from the aspect of recurrence-free survival as well as social and intellectual activity.</div></div><div><h3>Methods</h3><div>We retrospectively reviewed medical records and imaging data for 12 patients with BG germinoma diagnosed and treated between 1996 and 2020, collecting the most recent status via medical records or telephone from the patients.</div></div><div><h3>Results</h3><div>The tumors involved the right and left sides and bilateral locations in three, seven, and two cases, respectively. The median follow-up period was 179 months. Six patients exhibited onset neuropsychologic symptoms, for example, cognitive decline or behavior disorder. We evaluated the intelligence quotient (IQ) in 10 patients and observed significantly lower IQ scores in six patients with neuropsychologic symptoms. Patients with right-sided lesions displayed average IQ levels (median 106), whereas those with left-sided or bilateral lesions had reduced IQ (median 67). A complete response was achieved by primary chemoradiotherapy in all patients. Three patients who underwent focal or whole-ventricle irradiation experienced recurrence, whereas no recurrence was observed in patients who received whole-brain irradiation (WBI). Concerning the neurocognitive outcomes, three and six patients with unilateral right and left lesions were living self-independently (Karnofsky Performance Status [KPS] ≧ 70), whereas the three remaining patients (one with left and two with bilateral lesions) had a dependent status (KPS <70).</div></div><div><h3>Conclusions</h3><div>WBI is crucial for disease control in BG germinoma. Furthermore, lesion laterality might affect neuropsychologic symptoms including IQ at diagnosis and neurocognitive outcomes in BG germinoma.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"165 ","pages":"Pages 16-21"},"PeriodicalIF":3.2000,"publicationDate":"2025-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Pediatric neurology","FirstCategoryId":"3","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0887899425000177","RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Background
In this study, we aimed at delineating the still ambiguous clinical characteristics and long-term outcomes of basal ganglia (BG) germinoma from the aspect of recurrence-free survival as well as social and intellectual activity.
Methods
We retrospectively reviewed medical records and imaging data for 12 patients with BG germinoma diagnosed and treated between 1996 and 2020, collecting the most recent status via medical records or telephone from the patients.
Results
The tumors involved the right and left sides and bilateral locations in three, seven, and two cases, respectively. The median follow-up period was 179 months. Six patients exhibited onset neuropsychologic symptoms, for example, cognitive decline or behavior disorder. We evaluated the intelligence quotient (IQ) in 10 patients and observed significantly lower IQ scores in six patients with neuropsychologic symptoms. Patients with right-sided lesions displayed average IQ levels (median 106), whereas those with left-sided or bilateral lesions had reduced IQ (median 67). A complete response was achieved by primary chemoradiotherapy in all patients. Three patients who underwent focal or whole-ventricle irradiation experienced recurrence, whereas no recurrence was observed in patients who received whole-brain irradiation (WBI). Concerning the neurocognitive outcomes, three and six patients with unilateral right and left lesions were living self-independently (Karnofsky Performance Status [KPS] ≧ 70), whereas the three remaining patients (one with left and two with bilateral lesions) had a dependent status (KPS <70).
Conclusions
WBI is crucial for disease control in BG germinoma. Furthermore, lesion laterality might affect neuropsychologic symptoms including IQ at diagnosis and neurocognitive outcomes in BG germinoma.
期刊介绍:
Pediatric Neurology publishes timely peer-reviewed clinical and research articles covering all aspects of the developing nervous system.
Pediatric Neurology features up-to-the-minute publication of the latest advances in the diagnosis, management, and treatment of pediatric neurologic disorders. The journal''s editor, E. Steve Roach, in conjunction with the team of Associate Editors, heads an internationally recognized editorial board, ensuring the most authoritative and extensive coverage of the field. Among the topics covered are: epilepsy, mitochondrial diseases, congenital malformations, chromosomopathies, peripheral neuropathies, perinatal and childhood stroke, cerebral palsy, as well as other diseases affecting the developing nervous system.