MR imaging findings of germ cell tumors arising from the basal ganglia: focused on early imaging finding

Object Intracranial germ cell tumors (GCTs) originating in the basal ganglia are rare. The authors investigated factors related to the diagnosis of these lesions as well as outcome in order to help decrease the time to diagnosis and improve treatment efficacy. Methods The authors reviewed the clinical features of 142 cases of intracranial GCT in their institute. Fourteen cases of basal ganglia GCT were identified. The symptoms, neuroimaging findings, delay between symptom onset and diagnosis or treatment, initial and further treatment, and outcome were investigated. Results Major symptoms were motor weakness and precocious puberty. Gadolinium-enhanced T1-weighted MR images showed enhancement in 8 of 11 patients examined, but only slight hyperintensity without enhancement in 2 patients. Ipsilateral peduncle and hemispheric atrophy were found in 3 and 4 patients, respectively. Cases of basal ganglia GCT were characterized by a longer delay from the initial neuroimaging examination to diagnosis compared with GCT in other regions. Five patients had aggravated hemiparesis in the extremities due to the delay in diagnosis. Despite good response to the initial therapy, 5 patients experienced recurrence; 2 of these 5 had malignant GCTs, and 3 had been treated only with chemotherapy or radiochemotherapy with insufficient radiation dose and field. Finally, the 2 patients with malignant GCTs died of the disease, and 1 died of aspiration pneumonia due to dissemination around the brainstem. Conclusions Early diagnosis requires MR imaging with administration of contrast medium in young patients presenting with motor weakness and/or precocious puberty. Serial neuroimaging studies should be performed if any tiny lesion is detected in the basal ganglia. Since insufficient treatment resulted in early recurrence, radiation therapy with adequate dose and field is essential.

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

Neuro-Oncology ◽

10.1093/neuonc/noaa222.235 ◽

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.

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CTNI-63. PROGNOSTIC FACTORS IN PATIENTS WITH BASAL GANGLIA GERM-CELL TUMORS: A RETROSPECTIVE ANALYSIS OF THE SINGLE CHINESE INSTITUTE EXPERIENCE FROM 2009 TO 2019

Neuro-Oncology ◽

10.1093/neuonc/noaa215.229 ◽

2020 ◽

Vol 22 (Supplement_2) ◽

pp. ii57-ii57

Author(s):

Qingjun Hu ◽

Juan Li ◽

Mingyao Lai ◽

Cheng Zhou ◽

Zhaoming Zhou ◽

...

Keyword(s):

Survival Rate ◽

Basal Ganglia ◽

Germ Cell ◽

Retrospective Analysis ◽

Surgical Resection ◽

Germ Cell Tumors ◽

Progression Free Survival ◽

Course Of Disease ◽

Free Survival

Abstract OBJECTIVE To evaluate the clinical factors related to the prognosis of basal ganglia germ cell tumors. METHODS A retrospective analysis of 52 cases of the basal ganglia germ cell tumors treated from January 2009 to January 2019 in the department of oncology of Guangdong Sanjiu Brain Hospital. The median age: 12 years (range: 5–32), The median course of disease: 11.7 months (range: 1–54). Thirteen cases were diagnosed by biopsy and 39 cases were diagnosed by elevated tumor markers. There were 31 patients (59.6%) diagnosed with germinomas and 21 patients (40.4%) with non-germ germ cell tumors. Univariate and multivariate survival analysis was performed. RESULTS To October 15, 2019, the median follow-up time was 30.4 months (range 2–124 months). The 5-year survival rate was 85%, and the 5-year progression-free survival rate was 84%. Multivariate analysis found whether serum AFP was greater than 100mIU / ml, (with HR: 11.441,95% CI: 2.09–47.66, P = 0.005),the degree of surgical resection(with HR 5.323 (1.19–23.812), P = 0.029), PD as the effect of radiotherapy (HR: 16.53, (1.19–23.81), P = 0.001) were independent prognostic factor affecting survival. CONCLUSION The pathological type, degree of surgical resection, and response to initial treatment can all affect survival.

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Diffusion-weighted imaging and the apparent diffusion coefficient on 3T MR imaging in the differentiation of craniopharyngiomas and germ cell tumors

Neurosurgical Review ◽

10.1007/s10143-015-0660-0 ◽

2015 ◽

Vol 39 (2) ◽

pp. 207-213 ◽

Cited By ~ 8

Author(s):

Yasuyuki Kinoshita ◽

Fumiyuki Yamasaki ◽

Atsushi Tominaga ◽

Megu Ohtaki ◽

Satoshi Usui ◽

...

Keyword(s):

Diffusion Coefficient ◽

Mr Imaging ◽

Apparent Diffusion Coefficient ◽

Germ Cell ◽

Diffusion Weighted Imaging ◽

Germ Cell Tumors ◽

Diffusion Weighted ◽

Apparent Diffusion ◽

3T Mr

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

Neuro-Oncology ◽

10.1093/neuonc/noz139 ◽

2019 ◽

Vol 21 (12) ◽

pp. 1565-1577 ◽

Cited By ~ 1

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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Unusual Imaging Findings Associated with Germ Cell Tumors

RadioGraphics ◽

10.1148/rg.2019180050.podcast ◽

2019 ◽

Keyword(s):

Germ Cell ◽

Germ Cell Tumors ◽

Imaging Findings

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Germ cell tumors in male patients without gonadal involvement: computed tomography/magnetic resonance imaging findings and diagnostic workflow

Quantitative Imaging in Medicine and Surgery ◽

10.21037/qims.2019.11.01 ◽

2019 ◽

Vol 9 (12) ◽

pp. 2000-2007 ◽

Cited By ~ 1

Author(s):

Serena Dell’Aversana ◽

Milena Coppola ◽

Valeria Romeo ◽

Lorenzo Ugga ◽

Luisa Piccin ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Computed Tomography ◽

Magnetic Resonance ◽

Germ Cell ◽

Germ Cell Tumors ◽

Imaging Findings ◽

Resonance Imaging ◽

Male Patients

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RARE-14. STRUCTURAL BRAIN PLASTICITY OF THE GRAY MATTER IN PATIENTS WITH BASAL GANGLIA GERM CELL TUMORS: A VBM STUDY

Neuro-Oncology ◽

10.1093/neuonc/noz175.937 ◽

2019 ◽

Vol 21 (Supplement_6) ◽

pp. vi224-vi224

Author(s):

Yanong Li

Keyword(s):

Basal Ganglia ◽

Germ Cell ◽

Gray Matter ◽

Gray Matter Volume ◽

Germ Cell Tumors ◽

T Test ◽

Whole Brain ◽

Volume Increase ◽

Matter Volume ◽

Significant Difference

Abstract OBJECTIVE To assess the whole brain structural plasticity in case of unilateral basal ganglia germ cell tumors (BGGCTs). METHODS To detect changes in gray matter volume of the whole brain from structural Magnetic Resonance Imaging (MRI), we used voxel-based morphometry (VBM) in a sample of 41 patients with BGGCTs invading the left basal ganglia (BasalG_L group; n = 22) or the right basal ganglia (BasalG_R group; n = 19) and a sample of 16 patients with GCTs arising in pineal or suprasellar regions, comparing these groups with 16 age-matched normal controls (NCs) by two-sample t test after that. RESULTS To left BGGCTs patients, the regions of whole brain VBM analysis emphasized a large cluster of voxels with gray matter volume increase in left para hippocampal (k = 529 voxels, T=4.18, p< 0.01) and decrease in left thalamus (k = 527 voxels, T=-4.88, p< 0.01). At the same time, the cluster of voxels with gray matter volume increase in right middle cingulate cortex (rMCC) (k = 172 voxels, T=3.96, p< 0.01), and decrease in right inferior frontal gyrus (rIFG), pars opercular (k = 495 voxels, T= -4.29, p< 0.01) in right BGGCTs patients. Furthermore, gray matter volume showed no significant difference between groups of patients with GCTs arising in pineal or suprasellar regions and NCs by two-sample t test. And the results were corrected by family-wise-error correction. CONCLUSIONS The revealed results demonstrate that slow-growing but destructive lesion of the BGGCTs markedly and asymmetrically atrophies the gray matter volume in specific brain regions and shows compensatory plasticity in each side of cerebral hemisphere. Our findings direct focus on the whole cerebral adaptation that perhaps be a physiologic basis for the high level of functional compensation and partially explain the relationships between gray matter remodeling and cognitive disturbances observed in patients with BGGCTs.

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