Primary intracranial germ cell tumors in children: a report of eight cases and review of the literature

This study was conducted to evaluate the signs and symptoms on admission, diagnosis, localization, therapy, and survival of patients with primary intracranial germ cell tumors (PICGCT). Eight patients with surgically confirmed PICGCTs were treated and followed up at Hacettepe University'...

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Main Authors: C Akyüz, V Köseoğlu, V Bertan, F Söylemezoğlu, M T Kutluk, M Büyükpamukçu
Format: Article
Language:English
Published: Hacettepe University Institute of Child Health 1999-04-01
Series:The Turkish Journal of Pediatrics
Online Access:https://turkjpediatr.org/article/view/3216
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author C Akyüz
V Köseoğlu
V Bertan
F Söylemezoğlu
M T Kutluk
M Büyükpamukçu
author_facet C Akyüz
V Köseoğlu
V Bertan
F Söylemezoğlu
M T Kutluk
M Büyükpamukçu
author_sort C Akyüz
collection DOAJ
description This study was conducted to evaluate the signs and symptoms on admission, diagnosis, localization, therapy, and survival of patients with primary intracranial germ cell tumors (PICGCT). Eight patients with surgically confirmed PICGCTs were treated and followed up at Hacettepe University's Department of Pediatric Oncology between 1974 and 1995. While one patient was admitted with a second recurrence of her disease, the others were admitted or referred primarily to our institution. In this period, 357 germ cell tumor and 684 primary intracranial malignant tumors were diagnosed and treated at our institution. Thus, PICGCTs comprised 1.1 percent of the primary intracranial malignant tumors and 2.2 percent of the germ cell tumors. There were four females and four males and the median age was eight years (13 months to 12 years). On admission, the most common symptoms were diabetes insipidus (3/8) and vomiting (3/8). One patient also and Down's syndrome. Locations of the tumors were suprasellar in three, in the third ventricle in two, and in the cerebral parenchyma, and pineal and hypothalamic regions in the remainder. There were germinomas, three malignant teratomas, and two mixed germ cell tumors. Only two patients could be treated with appropriate and adequate chemotherapy and radiotherapy. Three patients died: one in the postsurgical period, one after the third surgical approach and one 11 months after the diagnosis of progressive disease; three were lost to follow-up. The remaining two patients (with second recurrence and disseminated disease) are alive and without disease. Our experience with these patients demonstrated that appropriate and adequate chemotherapy is as effective a treatment as radiotherapy, even with recurrence of the disease.
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spelling doaj.art-73052515755a4b99bd7c6b609ca9003e2025-03-07T19:44:07ZengHacettepe University Institute of Child HealthThe Turkish Journal of Pediatrics0041-43012791-64211999-04-01412Primary intracranial germ cell tumors in children: a report of eight cases and review of the literatureC Akyüz0V KöseoğluV BertanF SöylemezoğluM T KutlukM BüyükpamukçuDepartment of Pediatric Oncology, Hacettepe University Institute of Oncology, Ankara. This study was conducted to evaluate the signs and symptoms on admission, diagnosis, localization, therapy, and survival of patients with primary intracranial germ cell tumors (PICGCT). Eight patients with surgically confirmed PICGCTs were treated and followed up at Hacettepe University's Department of Pediatric Oncology between 1974 and 1995. While one patient was admitted with a second recurrence of her disease, the others were admitted or referred primarily to our institution. In this period, 357 germ cell tumor and 684 primary intracranial malignant tumors were diagnosed and treated at our institution. Thus, PICGCTs comprised 1.1 percent of the primary intracranial malignant tumors and 2.2 percent of the germ cell tumors. There were four females and four males and the median age was eight years (13 months to 12 years). On admission, the most common symptoms were diabetes insipidus (3/8) and vomiting (3/8). One patient also and Down's syndrome. Locations of the tumors were suprasellar in three, in the third ventricle in two, and in the cerebral parenchyma, and pineal and hypothalamic regions in the remainder. There were germinomas, three malignant teratomas, and two mixed germ cell tumors. Only two patients could be treated with appropriate and adequate chemotherapy and radiotherapy. Three patients died: one in the postsurgical period, one after the third surgical approach and one 11 months after the diagnosis of progressive disease; three were lost to follow-up. The remaining two patients (with second recurrence and disseminated disease) are alive and without disease. Our experience with these patients demonstrated that appropriate and adequate chemotherapy is as effective a treatment as radiotherapy, even with recurrence of the disease. https://turkjpediatr.org/article/view/3216
spellingShingle C Akyüz
V Köseoğlu
V Bertan
F Söylemezoğlu
M T Kutluk
M Büyükpamukçu
Primary intracranial germ cell tumors in children: a report of eight cases and review of the literature
The Turkish Journal of Pediatrics
title Primary intracranial germ cell tumors in children: a report of eight cases and review of the literature
title_full Primary intracranial germ cell tumors in children: a report of eight cases and review of the literature
title_fullStr Primary intracranial germ cell tumors in children: a report of eight cases and review of the literature
title_full_unstemmed Primary intracranial germ cell tumors in children: a report of eight cases and review of the literature
title_short Primary intracranial germ cell tumors in children: a report of eight cases and review of the literature
title_sort primary intracranial germ cell tumors in children a report of eight cases and review of the literature
url https://turkjpediatr.org/article/view/3216
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