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Secreting Germ Cell Tumors of the Central Nervous System: A Long-Term Follow-up Experience.

ABSTRACT: INTRODUCTION:Due to the rarity of nongerminomatous germ cell tumors (NGGCT) with non-standard treatment as yet, we report retrospectively our 30 year experience with chemotherapy followed by craniospinal irradiation (CSI), plus a boost of whole ventricular irradiation (WVI)/tumor bed (TB), tailored to pre-radiation chemotherapy response. METHODS:Between 1988 and 2016, 28 patients received four cycles of PEB (cisplatin/etoposide/bleomycin), then CSI, and two further PEB cycles. Between 1988 and1994, CSI was 25.5 Gy for patients in complete remission (CR), 30 Gy if in partial remission (PR) or metastatic, with a boost to TB up to 45-54 Gy. In the period of 1995-2010, the boost included WVI and any extra-ventricular tumor sites up to 45 Gy. After 2010, CSI was reduced to 25.5 Gy for all non-metastatic patients, and a boost was given only to TB up to 40.5/45.5 Gy, depending on patients' CR/PR status. After 2003, patients with alfafetoprotein (?FP) > 1000 ng/mL received intensified treatment, also including autologous stem cell transplantation. RESULTS:Among 28 patients (23 males; median age 12 years, 6 metastatic), 25 responded to PEB, and three progressed (PD) after one to four cycles; 26 received radiotherapy obtaining 13 CR, 7 PR and 5 stable disease (SD), 1 PD; 6 (21%) died (5 for disease, 1 for pneumonia while in CR). Five-year overall survival (OS) and progression-free survival (PFS) were both 81%; 10 year OS and PFS 81% and 76%, respectively (median follow-up 11 years). CONCLUSIONS:Survival for children with NGGCT, independently from disease extent, was encouraging. Further studies should elucidate which patients could benefit from reduced volume and dose irradiation.

SUBMITTER: Biassoni V

PROVIDER: S-EPMC7565315 | biostudies-literature | 2020 Sep

REPOSITORIES: biostudies-literature

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Secreting Germ Cell Tumors of the Central Nervous System: A Long-Term Follow-up Experience.

Biassoni Veronica V Schiavello Elisabetta E Gandola Lorenza L Pecori Emilia E Poggi Geraldina G Spreafico Filippo F Terenziani Monica M Meazza Cristina C Podda Marta M Ferrari Andrea A Luksch Roberto R Casanova Michela M Puma Nadia N Chiaravalli Stefano S Bergamaschi Luca L Cefalo Graziella G Simonetti Fabio F Gattuso Giovanna G Seregni Ettore Cesare EC Pallotti Federica F Gianno Francesca F Diletto Barbara B Barretta Francesco F Massimino Maura M

Cancers 20200921 9

<h4>Introduction</h4>Due to the rarity of nongerminomatous germ cell tumors (NGGCT) with non-standard treatment as yet, we report retrospectively our 30 year experience with chemotherapy followed by craniospinal irradiation (CSI), plus a boost of whole ventricular irradiation (WVI)/tumor bed (TB), tailored to pre-radiation chemotherapy response.<h4>Methods</h4>Between 1988 and 2016, 28 patients received four cycles of PEB (cisplatin/etoposide/bleomycin), then CSI, and two further PEB cycles. Bet ...[more]

PMID: 32967085

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Project description:Background: The wearable cardioverter-defibrillator (WCD) is available for patients at high risk for sudden cardiac death (SCD) when immediate implantable cardioverter-defibrillator (ICD) implantation is not possible or indicated. Patient selection remains challenging especially in primary prevention. Long-term data on these patients is still lacking.Methods153 patients were included in this study. They were prescribed the WCD between April 2012 and March 2019 at the University Medical Center, Mannheim, Germany. The mean follow-up period was 36.2 ± 15.6 months. Outcome data, including all-cause mortality, were analyzed by disease etiology and ICD implantation following WCD use.ResultsWe analyzed 56 patients with ischemic cardiomyopathy, 70 patients with non-ischemic cardiomyopathy, 16 patients with prior need for ICD/CRT-D (device for cardiac resynchronization therapy with defibrillator) explanation, 8 patients with acute myocarditis and 3 patients with congenital diseases. 58% of the patients did not need ICD/CRT-D implantation after WCD use. 4% of all patients suffered from appropriate WCD shocks. 2 of these patients (33%) experienced appropriate ICD shocks after implantation due to ventricular tachyarrhythmias. Long-term follow-up shows a good overall survival. All-cause mortality was 10%. There was no significant difference between patients with or without subsequent ICD implantation (p = 0.48). Patients with ischemic cardiomyopathy numerically showed a higher long-term mortality than patients with non-ischemic cardiomyopathy (14% vs. 6%, p = 0.13) and received significantly more ICD shocks after implantation (10% of ischemic cardiomyopathy (ICM) patients versus 3% of non-ischemic cardiomyopathy (NICM) patients, p = 0.04). All patients with ventricular tachyarrhythmias during WCD use or after ICD implantation survived the follow-up period.ConclusionFollowing WCD use, ICD implantation could be avoided in 58% of patients. Long-term follow-up shows good overall survival. The majority of all patients did not suffer from WCD shocks nor did receive ICD shocks after subsequent implantation. Patient selection regarding predictive conditions on long-term risk of ventricular tachyarrhythmias needs further risk stratification.

Dataset Information

Secreting Germ Cell Tumors of the Central Nervous System: A Long-Term Follow-up Experience.

Publications

Secreting Germ Cell Tumors of the Central Nervous System: A Long-Term Follow-up Experience.

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