Neurological and endocrinological manifestations of 49 children with intracranial pure germinoma at initial diagnosis in Taiwan

Background: Intracranial pure germinoma is a rare extragonadal neoplasm. Affected patients may have motor impairment, visual disturbance, neurological signs, and endocrine disorder, depending on the size and location of the tumor. This study investigated and analyzed patients’ demographic data and n...

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Main Authors: Hsin-Yuan Chang, Chiao-Fan Chiu, Shih-Ming Jung, Alex Mun-Ching Wong, Chieh-Tsai Wu, Fu-Sung Lo
Format: Article
Language:English
Published: Elsevier 2021-01-01
Series:Pediatrics and Neonatology
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Online Access:http://www.sciencedirect.com/science/article/pii/S1875957220301650
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author Hsin-Yuan Chang
Chiao-Fan Chiu
Shih-Ming Jung
Alex Mun-Ching Wong
Chieh-Tsai Wu
Fu-Sung Lo
author_facet Hsin-Yuan Chang
Chiao-Fan Chiu
Shih-Ming Jung
Alex Mun-Ching Wong
Chieh-Tsai Wu
Fu-Sung Lo
author_sort Hsin-Yuan Chang
collection DOAJ
description Background: Intracranial pure germinoma is a rare extragonadal neoplasm. Affected patients may have motor impairment, visual disturbance, neurological signs, and endocrine disorder, depending on the size and location of the tumor. This study investigated and analyzed patients’ demographic data and neuroimaging, clinical, laboratory, and endocrinological findings. Methods: We performed a retrospective chart review of 49 children diagnosed with pure germinoma in Taiwan from 1990 to 2018. The initial clinical presentation, tumor markers (beta-hCG, alpha fetoprotein, and carcinoembryonic antigen), pituitary function, and brain images were reviewed and analyzed. Results: This study included 49 patients (37 boys and 12 girls). Their ages ranged from 7.5 to 17.9 years, and the mean age at diagnosis was 13.6 years. Initial symptoms included visual disturbance (n = 23, 47.9%), motor impairment (n = 20, 40.8%), polyuria (n = 20, 40.8%), headache (n = 17, 34.7%), dizziness or vertigo (n = 14, 28.6%), nausea/vomiting (n = 13, 26.5%), and short stature (n = 8, 18.2%). Laboratory data indicated growth hormone deficiency or low IGF-1 levels (n = 18, 85.7%), adrenal insufficiency (n = 21, 77.8%), central diabetes insipidus (n = 27, 55.1%), central hypothyroidism (n = 15, 48.4%), and hypogonadotropic hypogonadism (n = 4, 44.4%). Conclusion: Intracranial pure germinomas may initially manifest as neurological symptoms or endocrinological findings at diagnosis. As endocrinologic presentation is related to delayed diagnosis, clinicians should be aware of patients with such complaints. Laboratory data should be surveyed carefully, and neuroimaging must be considered if the result is abnormal.
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spelling doaj-art-2441dbfe13964aacb6ea0c49cf48a39a2025-08-20T02:04:41ZengElsevierPediatrics and Neonatology1875-95722021-01-0162110611210.1016/j.pedneo.2020.07.017Neurological and endocrinological manifestations of 49 children with intracranial pure germinoma at initial diagnosis in TaiwanHsin-Yuan Chang0Chiao-Fan Chiu1Shih-Ming Jung2Alex Mun-Ching Wong3Chieh-Tsai Wu4Fu-Sung Lo5Division of Pediatric Endocrinology and Genetics, Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, TaiwanDivision of Pediatric Endocrinology and Genetics, Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, TaiwanDepartment of Pathology, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, TaiwanDepartment of Medical Imaging and Intervention, Chang Gung Memorial Hospital, Keelung/Linkou, and Chang Gung University, TaiwanDepartment of Neurosurgery, Chang Gung Memorial Hospital, Linkou Medical Center, Taoyuan, TaiwanDivision of Pediatric Endocrinology and Genetics, Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University College of Medicine, Taiwan; Corresponding author. Department of Pediatrics, Chang Gung Memorial Hospital, Chang Gung University, College of Medicine, No.5, Fusing St., Gueishan Township, Taoyuan County, 333, Taiwan. Fax: +886 3 3288957.Background: Intracranial pure germinoma is a rare extragonadal neoplasm. Affected patients may have motor impairment, visual disturbance, neurological signs, and endocrine disorder, depending on the size and location of the tumor. This study investigated and analyzed patients’ demographic data and neuroimaging, clinical, laboratory, and endocrinological findings. Methods: We performed a retrospective chart review of 49 children diagnosed with pure germinoma in Taiwan from 1990 to 2018. The initial clinical presentation, tumor markers (beta-hCG, alpha fetoprotein, and carcinoembryonic antigen), pituitary function, and brain images were reviewed and analyzed. Results: This study included 49 patients (37 boys and 12 girls). Their ages ranged from 7.5 to 17.9 years, and the mean age at diagnosis was 13.6 years. Initial symptoms included visual disturbance (n = 23, 47.9%), motor impairment (n = 20, 40.8%), polyuria (n = 20, 40.8%), headache (n = 17, 34.7%), dizziness or vertigo (n = 14, 28.6%), nausea/vomiting (n = 13, 26.5%), and short stature (n = 8, 18.2%). Laboratory data indicated growth hormone deficiency or low IGF-1 levels (n = 18, 85.7%), adrenal insufficiency (n = 21, 77.8%), central diabetes insipidus (n = 27, 55.1%), central hypothyroidism (n = 15, 48.4%), and hypogonadotropic hypogonadism (n = 4, 44.4%). Conclusion: Intracranial pure germinomas may initially manifest as neurological symptoms or endocrinological findings at diagnosis. As endocrinologic presentation is related to delayed diagnosis, clinicians should be aware of patients with such complaints. Laboratory data should be surveyed carefully, and neuroimaging must be considered if the result is abnormal.http://www.sciencedirect.com/science/article/pii/S1875957220301650brain neoplasmsgerminomahypopituitarismpediatrics
spellingShingle Hsin-Yuan Chang
Chiao-Fan Chiu
Shih-Ming Jung
Alex Mun-Ching Wong
Chieh-Tsai Wu
Fu-Sung Lo
Neurological and endocrinological manifestations of 49 children with intracranial pure germinoma at initial diagnosis in Taiwan
Pediatrics and Neonatology
brain neoplasms
germinoma
hypopituitarism
pediatrics
title Neurological and endocrinological manifestations of 49 children with intracranial pure germinoma at initial diagnosis in Taiwan
title_full Neurological and endocrinological manifestations of 49 children with intracranial pure germinoma at initial diagnosis in Taiwan
title_fullStr Neurological and endocrinological manifestations of 49 children with intracranial pure germinoma at initial diagnosis in Taiwan
title_full_unstemmed Neurological and endocrinological manifestations of 49 children with intracranial pure germinoma at initial diagnosis in Taiwan
title_short Neurological and endocrinological manifestations of 49 children with intracranial pure germinoma at initial diagnosis in Taiwan
title_sort neurological and endocrinological manifestations of 49 children with intracranial pure germinoma at initial diagnosis in taiwan
topic brain neoplasms
germinoma
hypopituitarism
pediatrics
url http://www.sciencedirect.com/science/article/pii/S1875957220301650
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