scholarly journals Prognostic Nomogram in Children and Adolescents with Central Nervous System Germ Cell Tumors

2021 ◽  
Author(s):  
Zetian Jia ◽  
Yaqi Yan ◽  
Jiuxin Wang ◽  
He Yang ◽  
Shuo Liu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Germ Cell ◽  
Children And Adolescents ◽  
Survival Probability ◽  
Survival Curve ◽  
Characteristic Curve ◽  
Germ Cell Tumors ◽  
Calibration Plot ◽  
Development Group

Abstract Background: The prognostic risk factors for primary central nervous system (CNS) germ cell tumors (GCTs) in children and adolescents remain controversial. This study aimed to develop and validate a nomogram that predicts 5-and 10-year survival rates for CNS GCTs in children and adolescents.Methods: Pediatric with intracranial GCTs in the Surveillance, Epidemiology, and End Results (SEER) database from 2000 to 2018 were analyzed. All GCTs children were randomly divided into the development group (70%) and the validation group (30%). The least absolute shrinkage and selection operator (LASSO) was used to screen the characteristic variables, and the 5- and 10-year survival probability nomogram was constructed. The accuracy of the nomogram was evaluated by consistency index (C-index), calibration plot, receiver operating characteristic curve (ROC), decision curve analysis (DCA) and survival curve.Results: We identified 819 cases of CNS GCTs in children. Three variables (histology, site and size) were selected by lasso regression to construct the nomogram. The C-index of the development group and verification group is 0.744 and 0.759. According to the time points of 5 and 10 years, the area under the curve of the development group is 0.753 and 0.696, and the verification set is 0.787 and 0.718. The results show that the prognostic nomogram developed by us has good accuracy.Conclusions: This is the first prognostic nomogram of CNS GCTs in children and adolescents. It can effectively predict the 5- and 10-year survival probability of children. It provides a useful prediction tool for clinicians.

scholarly journals Prognostic Nomogram in Children and Adolescents With Central Nervous System Germ Cell Tumors

2021 ◽  
Author(s):  
Zetian Jia ◽  
Yaqi Yan ◽  
Jiuxin Wang ◽  
He Yang ◽  
Shuo Liu ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Germ Cell ◽  
Children And Adolescents ◽  
Survival Probability ◽  
Survival Curve ◽  
Characteristic Curve ◽  
Germ Cell Tumors ◽  
Calibration Plot ◽  
Development Group

Abstract Background: The prognostic risk factors for primary central nervous system (CNS) germ cell tumors (GCTs) in children and adolescents remain controversial. This study aimed to develop and validate a nomogram that predicts 5-and 10-year survival rates for CNS GCTs in children and adolescents.Methods: Pediatric with intracranial GCTs in the Surveillance, Epidemiology, and End Results (SEER) database from 2000 to 2018 were analyzed. All GCTs children were randomly divided into the development group (70%) and the validation group (30%). The least absolute shrinkage and selection operator (LASSO) was used to screen the characteristic variables, and the 5- and 10-year survival probability nomogram was constructed. The accuracy of the nomogram was evaluated by consistency index (C-index), calibration plot, receiver operating characteristic curve (ROC), decision curve analysis (DCA) and survival curve.Results: We identified 819 cases of CNS GCTs in children. Three variables (histology, site and size) were selected by lasso regression to construct the nomogram. The C-index of the development group and verification group is 0.744 and 0.759. According to the time points of 5 and 10 years, the area under the curve of the development group is 0.753 and 0.696, and the verification set is 0.787 and 0.718. The results show that the prognostic nomogram developed by us has good accuracy.Conclusions: This is the first prognostic nomogram of CNS GCTs in children and adolescents. It can effectively predict the 5- and 10-year survival probability of children. It provides a useful prediction tool for clinicians.

scholarly journals GCT-15. INTEGRATED CLINICAL, HISTOPATHOLOGICAL, AND MOLECULAR DATA ANALYSIS OF 190 CENTRAL NERVOUS SYSTEM GERM CELL TUMORS FROM THE IGCT CONSORTIUM

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii330-iii331
Author(s):  
Hirokazu Takami ◽  
Koichi Ichimura ◽  
Kohei Fukuoka ◽  
Akitake Mukasa ◽  
Nobuhito Saito ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Basal Ganglia ◽  
Pineal Gland ◽  
Germ Cell ◽  
Prognostic Significance ◽  
Germ Cell Tumors ◽  
Molecular Data ◽  
Molecular Heterogeneity ◽  
Intracranial Germ Cell Tumor

Abstract BACKGROUND We integrated clinical, histopathological, and molecular data of central nervous system germ cell tumors to provide insights into their management. METHODS Data from the Intracranial Germ Cell Tumor Genome Analysis Consortium were reviewed. A total of 190 cases were classified as primary GCTs based on central pathological reviews. RESULTS All but one of the cases that were bifocal (neurohypophysis and pineal glands) and cases with multiple lesions including neurohypophysis or pineal gland were germinomas (34 of 35). Age was significantly higher in patients with germinoma than other histologies. Comparison between tumor marker and histopathological diagnoses showed that 18.2% of histopathologically diagnosed germinomas were marker-positive and 6.1% of non-germinomatous GCTs were marker-negative, suggesting a limitation in the utility of markers or histopathology alone using small specimens for diagnosis. Comparison between local and central histopathological diagnoses revealed a discordance of 12.7%. Discordance was significantly less frequent in biopsy cases, implying difficulty in detecting all histopathological components of heterogeneous GCTs. Germinomas at the typical sites (neurohypophysis or pineal gland) showed a better PFS than those at atypical sites (p=0.03). A molecular-clinical association study revealed frequent MAPK pathway mutations in males (51.4 vs 14.3 %, p=0.007), and PI3K/mTOR pathway mutations in basal ganglia cases (p=0.004). Basal ganglia cases also had frequent chromosomal losses. Some chromosomal aberrations (2q, 8q gain, 5q, 9p/q, 13q, 15q loss) showed potential prognostic significance. CONCLUSIONS These in-depth findings of this study regarding the clinical and molecular heterogeneity will increase our understanding of the pathogenesis of this enigmatic tumor.

Endocrine manifestations of central nervous system germ cell tumors in children

2020 ◽  
Vol 67 (8) ◽  
pp. 540-544
Author(s):  
Emilio García García ◽  
Ana Lucía Gómez Gila ◽  
Elena Merchante ◽  
Mónica Rivero Garvia ◽  
Eva Venegas Moreno ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Germ Cell ◽  
Germ Cell Tumors

scholarly journals Longitudinal evaluation of neurocognitive function after treatment for central nervous system germ cell tumors in childhood

Cancer ◽  
2011 ◽  
Vol 117 (23) ◽  
pp. 5402-5411 ◽  
Author(s):  
Donald J. Mabbott ◽  
Eric Monsalves ◽  
Brenda J. Spiegler ◽  
Ute Bartels ◽  
Laura Janzen ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Germ Cell ◽  
Germ Cell Tumors ◽  
Neurocognitive Function ◽  
Longitudinal Evaluation

Germ Cell Tumors of the Central Nervous System

2020 ◽  
pp. 270-285
Author(s):  
Matthew J. Murray ◽  
Ute Bartels ◽  
James C. Nicholson
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Germ Cell ◽  
Germ Cell Tumors ◽  
The Central Nervous System

scholarly journals Integrated clinical, histopathological, and molecular data analysis of 190 central nervous system germ cell tumors from the iGCT Consortium

2019 ◽  
Vol 21 (12) ◽  
pp. 1565-1577 ◽  
Author(s):  
Hirokazu Takami ◽  
Kohei Fukuoka ◽  
Shintaro Fukushima ◽  
Taishi Nakamura ◽  
Akitake Mukasa ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Basal Ganglia ◽  
Pineal Gland ◽  
Germ Cell ◽  
Prognostic Significance ◽  
Germ Cell Tumors ◽  
Molecular Data ◽  
Molecular Heterogeneity ◽  
Intracranial Germ Cell Tumor

Abstract Background We integrated clinical, histopathological, and molecular data of central nervous system germ cell tumors to provide insights into their management. Methods Data from the Intracranial Germ Cell Tumor Genome Analysis (iGCT) Consortium were reviewed. A total of 190 cases were classified as primary germ cell tumors (GCTs) based on central pathological reviews. Results All but one of the cases that were bifocal (neurohypophysis and pineal glands) and cases with multiple lesions including neurohypophysis or pineal gland were germinomas (34 of 35). Age was significantly higher in patients with germinoma than other histologies. Comparison between tumor marker and histopathological diagnoses showed that 18.2% of histopathologically diagnosed germinomas were marker positive and 6.1% of non-germinomatous GCTs were marker negative, suggesting a limitation in the utility of markers or histopathology alone using small specimens for diagnosis. Comparison between local and central histopathological diagnoses revealed a discordance of 12.7%. Discordance was significantly less frequent in biopsy cases, implying difficulty in detecting all histopathological components of heterogeneous GCTs. Germinomas at the typical sites (neurohypophysis or pineal gland) showed a better progression-free survival than those at atypical sites (P = 0.03). A molecular clinical association study revealed frequent mitogen-activated protein kinase (MAPK) pathway mutations in males (51.4% vs 14.3%, P = 0.007), and phosphatidylinositol-3 kinase/mammalian target of rapamycin (PI3K/mTOR) pathway mutations in basal ganglia cases (P = 0.004). Basal ganglia cases also had frequent chromosomal losses. Some chromosomal aberrations (2q, 8q gain, 5q, 9p/q, 13q, 15q loss) showed potential prognostic significance. Conclusions The in-depth findings of this study regarding clinical and molecular heterogeneity will increase our understanding of the pathogenesis of this enigmatic tumor.

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