scholarly journals Intracranial Germ Cells Tumour: a single institution experience in Argentina

2021 ◽  
Author(s):  
Lorena Viviana Baroni ◽  
Agustina Oller ◽  
Candela Soledad Freytes ◽  
Claudia Vanesa Sampor ◽  
Nicolas Ponce Fernandez ◽  
...  
Keyword(s):  
Germ Cell ◽  
Western Europe ◽  
Germ Cell Tumors ◽  
Treatment Strategies ◽  
Cooperative Groups ◽  
Middle Income ◽  
Intracranial Germ Cell Tumor ◽  
Excellent Outcome ◽  
Low Middle Income Countries ◽  
Multiagent Chemotherapy

Abstract Background: Intracranial germ cell tumor (iGCT) represents a rare and heterogeneous group, with variable incidence and diverse treatment strategies. Although multiagent chemotherapy with reduced radiotherapy strategy has been applied by several cooperative groups in North America and Western Europe, there is a paucity of data to understand if this combined regimen issuitable in low-middle income countries (LMIC).Methods: We evaluate the outcome in a cohort of iGCT treated by SIOP-CNS-GCT-96 strategy at Hospital J.P Garrahan in Argentinaover the last 20 years. Radiation field and dose included focal radiotherapy (FRT) before 2009 or focal radiotherapy plus whole ventricular radiotherapy (WVRT) after 2009 for localized germinoma and FRT or FRT plus WVRT or CSI for non germinomatous germ cell tumors (NGGCT)Results: Sixty iGCT were identified; 39 germinoma and 21 NGGCT. Median follow-up was 6.57 years (range 0.13-20.5). Five-year PFS and OS were 83.5% (95% CI [165.53 - 223.2]) and 88.7% (95% CI [169.84 - 223.2]) for the germinoma group, while for the NGGCT group were 75% (95% CI [133.27 – 219.96]) and 64.2% (95% CI [107.38 – 201.81]) respectively. The localized germinoma group showed poor results between 2000-2009 with 5-year PFS and OS of 69% and 75% respectively, and an excellent outcome between 2010-2019 with a 5-years PFS and OS of 92.8% and 100%. A univariable analysis identified this difference in survival as related to the field of radiotherapy, specifically whole ventricular radiotherapy. FRT increased the risk of recurrence in localized germinoma, involving not only ventricular relapses; but spinal cord and disseminated disease as well. There were no relapses of localized NGGCT after FRT and FRT plus WVRT.Conclusion: Herein we demonstrate that intensive chemotherapy followed by FRT plus WVRT for germinoma is a feasible and effective strategy, warranting further study in the developing world.

Factors that impact the outcomes in testicular germ cell tumors in low–middle-income countries

2019 ◽  
Vol 36 (3) ◽  
Author(s):  
S. V. Saju ◽  
Venkatraman Radhakrishnan ◽  
Trivadi S. Ganesan ◽  
Manikandan Dhanushkodi ◽  
Anand Raja ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumors ◽  
Testicular Germ Cell Tumors ◽  
Testicular Germ Cell ◽  
Middle Income ◽  
Middle Income Countries ◽  
Low Middle Income Countries

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.

scholarly journals MPC-08 CLINICOPATHOLOGICAL ANALYSIS OF 12P GAIN IN INTRACRANIAL GERM CELL TUMORS

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii23-ii23
Author(s):  
Kaishi Satomi ◽  
Hirokazu Takami ◽  
Shintaro Fukushima ◽  
Yoichi Nakazato ◽  
Shota Tanaka ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumor ◽  
Copy Number ◽  
Mature Teratoma ◽  
Methylation Status ◽  
Germ Cell Tumors ◽  
Copy Number Variations ◽  
Cell Tumor ◽  
Testicular Germ Cell ◽  
Intracranial Germ Cell Tumor

Abstract BACKGROUND Gain of short arm of chromosome 12 (12p) is commonly observed in testicular germ cell tumors (tGCTs). 12p gain is also frequently seen in intracranial GCTs (iGCTs). However, little is known about the clinical significance of 12p gain in iGCTs. MATERIALS AND METHODS We have collected over 200 fresh frozen tissue samples of iGCTs through the Intracranial Germ Cell Tumor Genome Analysis Consortium in Japan. Firstly, we analyzed DNA methylation status in 83 iGCTs, 3 seminomas and 6 normal control samples using Infinium Human Methylation 450K BeadChip array (Illumina, CA). Idat files were processed using R (Version 3.5.3) and minfi package (1.30.0) to generate copy number variations. Compared with average genome-wide copy number level, 12p gain was determined. Then, 58 iGCTs with clinicopathological information were analyzed for progression-free survival (PFS) and overall survival (OS). Those tumors that consist of only either germinoma and/or mature teratoma components were classified as Favorable Histology (FH) and all the others that contains malignant histological components were classified as Unfavorable Histology (UFH). RESULT 12p gain was observed in 100% (3/3) of seminoma, 13.6% (3/22) of germinoma, 16.7% (1/6) of mature teratoma, 25% (1/4) of immature teratoma, 55% (11/20) of mixed germ cell tumor, 100% (4/4) of yolk sac tumor, 100% (1/1) of embryonal carcinoma, and 100% (1/1) of choriocarcinoma. In total, 44.6% (37/83) of iGCT showed 12p gain. Regarding histological classification, the 12p gain rate in UFH (72%, 18/25) was significantly higher than that in FH (12.1%, 4/33, P<0.01). Both PFS and OS were significantly worse in iGCTs with 12p gain (PFS: P=0.027, OS: P=0.0012). DISCUSSION 12p gain can be a molecular marker to predict prognosis and histological malignancy in iGCTs.

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.

Gynecologic Cancer Intergroup (GCIG) Consensus Review for Ovarian Germ Cell Tumors

2014 ◽  
Vol 24 (Supp 3) ◽  
pp. S48-S54 ◽  
Author(s):  
Jubilee Brown ◽  
Michael Friedlander ◽  
Floor J. Backes ◽  
Philipp Harter ◽  
Dennis M. O’Connor ◽  
...  
Keyword(s):  
Germ Cell ◽  
Early Stage ◽  
Gynecologic Cancer ◽  
Tumor Biology ◽  
Germ Cell Tumors ◽  
Clinical Trial Design ◽  
Cooperative Groups ◽  
Stage Disease ◽  
Fertility Sparing ◽  
Collaborative Efforts

AbstractMost women diagnosed with malignant ovarian germ cell tumors have curable disease and experience excellent survival with manageable treatment-associated morbidity, related both to tumor biology and improvements in treatment over the last 4 decades. Malignant ovarian germ cell tumors occur predominantly in girls, adolescents, and young women and are often unilateral tumors of early stage, although advanced-stage disease occurs in approximately 30% of patients. Tumors are usually chemosensitive, thereby allowing fertility-sparing surgery in most women with high chance of cure. Differences in practice do exist among providers in various subspecialties and geographic areas. In most settings, collaborative efforts among specialties allow the optimal treatment of women with these rare tumors, and implementation of standard guidelines at an international level should translate to effective clinical trial design, rapid accrual to clinical trials, and universally improved patient outcomes.This consensus guideline represents a summary of recommendations for diagnosis and management that has been agreed upon by cooperative groups worldwide. It builds upon individual publications including previously published summary documents and provides the most current practice standards validated worldwide.

scholarly journals Clinical interpretation of residual uptake in 11C-methionine positron emission tomography after treatment of basal ganglia germ cell tumors: report of 3 cases

2015 ◽  
Vol 16 (4) ◽  
pp. 367-371
Author(s):  
Kohei Fukuoka ◽  
Takaaki Yanagisawa ◽  
Yuko Watanabe ◽  
Tomonari Suzuki ◽  
Masao Matsutani ◽  
...  
Keyword(s):  
Basal Ganglia ◽  
Germ Cell ◽  
Germ Cell Tumors ◽  
Treatment Strategies ◽  
Standardized Uptake Value ◽  
Progression Free Survival ◽  
Additional Treatment ◽  
Free Survival ◽  
Posterior Limb ◽  
Met Pet

Although 11C-methionine (MET)-PET has been used to diagnose intracranial germ cell tumors (GCTs) arising in the basal ganglia, whether this imaging technique is useful in assessing treatment response and residual tumor is still unclear. The authors report 3 cases of basal ganglia GCTs in which the residual MET uptake at the end of treatment did not develop into a relapse, even without additional treatment. Case 1 is a 22-year-old man who had a second relapse of a left basal ganglia germinoma with diffuse dissemination on the walls of both of his lateral ventricles. MET-PET revealed high MET accumulation around tumors and their surroundings (maximum standardized uptake value [SUVmax] 3.3). After all treatments, MET-PET demonstrated mild tracer accumulation in both basal ganglia (SUVmax 2.2). Progression-free survival was 56 months from the second relapse without any further treatment. Case 2 is a 17-year-old boy with a left basal ganglia germinoma that showed increased MET uptake (SUVmax 4.2). After treatment, MET-PET revealed residual MET uptake (SUVmax 2.4) along the left posterior limb of the internal capsule. Progression-free survival was 52 months from the start of treatment. Case 3 is a 7-year-old boy with a left basal ganglia choriocarcinoma with increased tumor MET uptake (SUVmax 2.5). A minor enhanced mass remained on MRI after treatment with residual MET accumulation (SUVmax 1.4). Progression-free survival was 44 months. Treatment strategies based on MET uptake on PET should be carefully designed in patients with basal ganglia GCTs to avoid overtreatment and complications.

scholarly journals GC-03 * INTRACRANIAL NONGERMINOMATOUS GERM CELL TUMORS: TREATMENT STRATEGIES AND OUTCOMES

2015 ◽  
Vol 17 (suppl 3) ◽  
pp. iii10-iii10 ◽  
Author(s):  
B. Imber ◽  
S. Braunstein ◽  
A. Banerjee ◽  
T. Tihan ◽  
A. Bollen ◽  
...  
Keyword(s):  
Germ Cell ◽  
Germ Cell Tumors ◽  
Treatment Strategies

Combination chemotherapy with cisplatin and etoposide for malignant intracranial germ-cell tumors

1989 ◽  
Vol 70 (5) ◽  
pp. 676-681 ◽  
Author(s):  
Tatsuya Kobayashi ◽  
Jun Yoshida ◽  
Junzo Ishiyama ◽  
Satoshi Noda ◽  
Akira Kito ◽  
...  
Keyword(s):  
Germ Cell ◽  
Combination Chemotherapy ◽  
Germ Cell Tumors ◽  
Intracranial Germ Cell Tumor ◽  
Content Type ◽  
Total Response Rate ◽  
Intracranial Germ Cell Tumors ◽  
Dna Histograms

✓ Antitumor activity against intracranial malignant teratoma by combination chemotherapy with cisplatin and etoposide was evaluated in experimental and clinical studies. A human teratoma cell line (Tera 2) was exposed in vitro to cisplatin and/or etoposide, after which cell growth inhibition and alterations of deoxyribonucleic acid (DNA) histograms were observed. The results indicated that a synergistic cytotoxic effect was achieved by use of both agents in combination. Four cases of recurrent intracranial germ-cell tumor (three malignant teratomas and one germinoma) were treated with cisplatin and etoposide. With this combinationtherapy, regression of the tumor was observed in all four cases (three complete and one partial), for a total response rate of 100%. During a follow-up period of 9 to 22 months, no recurrence or progression has been noted in three of these cases.

scholarly journals NI-11 Clinical significance of intracystic diffusion hyperintensity lesions remaining after treatment of intracranial germ cell tumor

2020 ◽  
Vol 2 (Supplement_3) ◽  
pp. ii14-ii14
Author(s):  
Motoki Takano ◽  
Takeshi Takayasu ◽  
Ushio Yonezawa ◽  
Akira Taguchi ◽  
Kazuhiko Sugiyama ◽  
...  
Keyword(s):  
Germ Cell ◽  
Clinical Significance ◽  
Cyst Wall ◽  
High Intensity ◽  
Mature Teratoma ◽  
Germ Cell Tumors ◽  
Residual Tumor ◽  
Cystic Tumor ◽  
Intracranial Germ Cell Tumor ◽  
Intensity Lesion

Abstract Background and purpose: About 30% of intracranial germ cell tumors are mixed germ cell tumors and teratomas are often found as those components. Intense chemoradiotherapy is performed according to the malignancy of the histopathology, but high-intensity lesion inside the cystic tumor on diffusion weighted imaging (DWI) sometimes remains after completion of the chemoradiotherapy. In this study, we examined the clinical significance of the DWI high-intensity lesion remaining in the cyst. METHODS: Five patients after initial chemoradiotherapy were resected residual tumor by craniotomy at our hospital from 2009 to 2019. Preoperative gadolinium-enhanced MRI defined the non-contrast-enhanced part of the tumor as intracystic, and DWI intensity was classified by its look as low-intensity, equal-intensity, and high-intensity compared to the cortex of the same slice. DWI signals in the solid area, cyst wall, and cyst were evaluated. Results: All cases were mature teratoma in histopathology, and no other tumor components were observed. On DWI, the cyst wall and solid part were visualized with low signal. High-intensity lesions and equal-intensity lesions in the cyst cavity were found in 3 and 1 cases, respectively. In these cases, pathological findings revealed a keratin-like substance in the cyst. Discussion: The intracystic high and equal intensity lesions on DWI removed after completion of chemoradiotherapy are considered to reflect the keratin-like component of mature teratoma. If DWI- high intensity and equal intensity lesions remain in the cyst of the tumor after the completion of chemoradiotherapy, tumor shrinkage cannot be expected even if the chemotherapy is strengthened. In such cases, we should consider to removing them by surgery. Conclusion: When DWI high and equal intensity lesions are found in the cysts of tumors remaining after chemoradiotherapy for intracranial germ tumors, it is possible that mature teratoma remains.

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