Agerbaek, Mads

Als, Anne-Birgitte

Cortnum, Soeren

Henriksen, Louise Tram

Kristensen, Ines Ackerl

Lassen-Ramshad, Yasmin

Mikkelsen, Torben Stamm

Rosthoej, Steen

Safwat, Akmal

Tuckuvienne, Ruta

von Oettingen, Gorm

English

Intracranial malignant germ cell tumours (iGCT) are rare brain tumours mainly diagnosed in children and younger adults.A retrospective analysis was performed by chart review of patients treated for iGCT in the northern and central region of Denmark. Teratoma only patients were not included in the study.20 patients with iGCT were diagnosed from 2008–2019 in Western Denmark. The cumulative incidence was 1.05 per 100.000. The yearly incidence was 0.1 per 100.000. Mean age at diagnosis was 18 years (range 8–36 years), 17 were males and 3 were females. 13 patients presented with germinoma and 7 patients with non germinomateous germ cell tumours (NGGCT). Three patients had disseminated disease, two with germinoma and one with NGGCT. All patients had received radiotherapy and 18 patients were treated with multidrug chemotherapy including platinum and etoposide before irradiation. Two patients experienced recurrent disease, both non disseminated at diagnosis, one patient with germinoma and one patient with NGGCT. Both received salvage treatment including high dose chemotherapy with stem cell transplantation and reirradiation. Two NGGCT patients died, one patient after development of an anaplastic astrocytoma in the radiation field five years after radiotherapy and one patient after intracranial hemorraghe 18 months after salvage treatment for recurrent disease. Overall survival was 90\ 100\1\% for NGGCT.The outcome of patients with iGCT in Western Denmark was comparable to the literature. A nationwide study of epidemiology and outcome of iGCT in Denmark is planned

    Aalborg UniversitetIncidence and outcome of intracranial malignant germ cell tumours diagnosed inwestern Denmark in the last decadeLassen-Ramshad, Yasmin; Mikkelsen, Torben Stamm; Rosthoej, Steen; Henriksen, LouiseTram; Tuckuvienne, Ruta; Kristensen, Ines Ackerl; von Oettingen, Gorm; Cortnum, Soeren;Als, Anne-Birgitte; Safwat, Akmal; Agerbaek, MadsPublished in:Neuro-OncologyDOI (link to publication from Publisher):10.1093/neuonc/noaa222.282Creative Commons LicenseCC BY 4.0Publication date:2020Document VersionPublisher's PDF, also known as Version of recordLink to publication from Aalborg UniversityCitation for published version (APA):Lassen-Ramshad, Y., Mikkelsen, T. S., Rosthoej, S., Henriksen, L. T., Tuckuvienne, R., Kristensen, I. A., vonOettingen, G., Cortnum, S., Als, A-B., Safwat, A., & Agerbaek, M. (2020). Incidence and outcome of intracranialmalignant germ cell tumours diagnosed in western Denmark in the last decade. Neuro-Oncology, 22(Suppl. 3),iii341. [GCT-65]. https://doi.org/10.1093/neuonc/noaa222.282General rightsCopyright and moral rights for the publications made accessible in the public portal are retained by the authors and/or other copyright ownersand it is a condition of accessing publications that users recognise and abide by the legal requirements associated with these rights.            ? Users may download and print one copy of any publication from the public portal for the purpose of private study or research.            ? You may not further distribute the material or use it for any profit-making activity or commercial gain            ? You may freely distribute the URL identifying the publication in the public portal ?Abstractsiii341NEURO-ONCOLOGY •  December 202011Department of Neurosurgery, Faculty of Medicine, University of Tsukuba, Tsukuba, Ibaraki, Japan, 12Department of Neurosurgery, Graduate School of Medicine, Yokohama City University, Yokohama, Kanagawa, Japan, 13Department of Pathology, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Hyogo, Japan, 14Department of Neurosurgery, Hyogo Prefectural Kobe Children’s Hospital, Kobe, Hyogo, Japan, 15Department of Hematology/Oncology, Saitama Children’s Medical Center, Saitama, Japan, 16Department of Neurosurgery, Saitama Children’s Medical Center, Saitama, Japan, 17Department of Neurosurgery, Kyorin University Faculty of Medicine, Mitaka, Tokyo, Japan, 18Department of Neurosurgery, Faculty of Medicine, The University of Tokyo, Bunkyo-ku, Tokyo, JapanBACKGROUND: Central nervous system germ cell tumor (CNSGCT) is a rare pediatric brain tumor. However, they are found at a relatively high incidence in East Asia. Germinoma is sensitive toward radiotherapy and chemotherapy; however, non-germinoma GCTs (NGGCT) often show poor response. Some cases are a mixture of germinoma and NGGCT (mixed GCT), and they sometimes change histological subtypes at recurrence. Previous report demonstrated that a germinoma and NGGCT component within the same mixed GCT tissue shared the same gene mutation, whereas the genome-wide methylation profiles were distinct from each other. The methylation profiles of germinoma was similar to the primordial germ cells (PGC) at the migration phase, supporting a model that PGC is the cell of origin for CNSGCT. However, tumor heterogeneity hinder informa-tion of the mixed bulk RNA-sequence data, causing difficulty in elucidating the mechanism of tumor development. The purpose of this study was to investigate the tumor cells subpopulations at the resolution of individual cells by single-cell RNA-seq.  RESULTS: Fresh surgical tumor tissue was immediately dissociated mechanically and enzymatically. Tumor cells are separated from CD45-labelled lymphocytes by FACS, and libraries were generated by Chromium Single cell 3’ Reagent Kit. Total of 11 tumor sam-ples were collected and sequenced. Unsupervised Clustering showed indi-vidual clusters. One of the clusters had high expression of Oct-4, which is a marker of germinoma. The other clusters showed different subtypes of cells representing the heterogeneity of CNSGCT. Further analysis including a pseudo-time course analysis is underway to identify the lineage of tumor cell development.GCT-63. STEREOTACTIC RADIOSURGERY FOR RESIDUAL LESIONS OF PINEAL NON-GERMINOMATOUS GERM CELL TUMORS AFTER CONVENTIONAL RADIOTHERAPY: A RETROSPECTIVE STUDYMingyao Lai, Juan Li, Qingjun Hu, Zhaoming Zhou, Lei Wen, Cheng Zhou, Changguo Shan, and Linbo Cai; Guangdong Sanjiu Brain Hospital, Guangzhou, Guangdong, ChinaOBJECTIVE: To explore the efficacy and safety of SRS for residual le-sions of NGGCTs after conventional RT. METHODS: The clinical data of patients with iGCT who were admitted to Department of Oncology, Guang-dong Sanjiu Brain Hospital between January 1, 2008 and December 30, 2019 were gathered. Those who were pathologically or clinically diagnosed with NGGCTs, with lesions located at pineal region, limited stage and re-sidual lesions (with a maximum diameter>10mm) of pineal NGGCTs after RT with a total dose of 50-54Gy/25-30f, were eligible for the study. Several indexes such as local control rate, PFS, OS and treatment-related toxicity were analyzed.  RESULTS: A total of 27 patients were included; all were male, with a median age of 16 years (range 8–31 years). The patients were followed-up to December 30, 2019, but there were 2 cases lost to follow-up. The median follow-up time was 34  months (range 8–142  months). After a month of treatment with SRS, the ORR and DCR were 71.4% and 95.2%, respectively. During follow-up, 5 cases had radiographic progres-sions, including 3 cases combined with increased AFP which were diagnosed with local recurrence and 2 cases diagnosed with GTS；The 3y-PFS and OS were 85.2% and 88.0%.no acute radiation response was found after treat-ment with SRS, and only one patient had brain neurotoxicity. CONCLU-SION: SRS for residual lesions of NGGCTs after RT is proved to be safe and feasible, with well tolerance, which is beneficial for the improvement of local control and the prolongation of survival.GCT-64. TREATMENT RESULTS IN CHILDREN WITH LOCALIZED CNS NGGCTIrina Vilesova1, Ekaterina Tarasova2, Andge Valiakhmetova3, Ekaterina Salnikova1, Alexey Pshonkin1, Galina Novichkova1, Alexander Karachunsky1, and Ludmila Papusha1; 1Dmitry Rogachev National Medical Research Center Of Pediatric Hematology, Oncology and Immunology, Moscow, Russian Federation, 2Russian Children’s Clinical Hospital, Moscow, Russian Federation, 3N. N. Burdenko National Medical Research Center of Neurosurgery, Moscow, Russian FederationBACKGROUND/OBJECTIVES: Treatment of children with CNS NGGCT remains challenge: 5y OS is 60 – 80%; relapses are very aggres-sive. DESIGN/METHODS: Between 2003 and 2019, 14 children (median age 10.5, range 4  – 16  years) with localized intracranial NGGCT were treated with RT after induction chemotherapy (focal – 4, WVI+boost – 6, WBI+boost – 3, CSI+boost – 1). Tumor markers were elevated in 13 pa-tients: 6 – AFP, 5 – HCG, 2 – both. One patient with level of HCG 72049 IU/l in serum and 121451 IU/l in CSF received 4 cycles of PEI + CSI 30 Gy with boost 54Gy. RESULTS: At a median follow-up of 4,7 years (range 1 – 16,25 years), 12 patients are alive. 5-year PFS and OS are 77,1% and 85,7%, respectively. Two patients (both AFP and HCG) progressed during RT (1 – focal, 1 – WBI+boost), both died. Two patients with high level of HCG recurred after therapy (WVI+boost – 1, focal – 1), both are alive. The first of them at recurrence (mts of lateral ventricle) received 4 cycles of PEI and RT (WBI+boost). The second patient had level of HCG 620IU/l and ini-tially received focal irradiation 54Gy. At recurrence with distant spinal mts he received HD-CHT with auto-SCT, surgical resection of residual tumor and CSI with boost. CONCLUSIONS: Good results of treatment of local-ized CNS NGGCT with CSI, WBI or WVI in compare with focal RT show advantages of extended irradiation field. CSI should be considered for pa-tients with extremely high levels of tumor markers and respectively poor prognostic histology.GCT-65. INCIDENCE AND OUTCOME OF INTRACRANIAL MALIGNANT GERM CELL TUMOURS DIAGNOSED IN WESTERN DENMARK IN THE LAST DECADEYasmin Lassen-Ramshad1, Torben Stamm Mikkelsen2, Steen Rosthoej3, Louise Tram Henriksen2, Ruta Tuckuvienne3, Ines Ackerl Kristensen2, Gorm von Oettingen4, Soeren Cortnum4, Anne-Birgitte Als5, Akmal Safwat1,5, and Mads Agerbaek5; 1Danish Centre For Particle Therapy, Aarhus University Hospital, Aarhus, Denmark, 2Pediatric Department, Aarhus University Hospital, Aarhus, Denmark, 3Pediatric Department, Aalborg University Hospital, Aalborg, Denmark, 4Department of Neurosurgery, Aarhus University Hospital, Aarhus, Denmark, 5Department of Oncology, Aarhus University Hospital, Aarhus, DenmarkINTRODUCTION: Intracranial malignant germ cell tumours (iGCT) are rare brain tumours mainly diagnosed in children and younger adults. MATERIAL AND METHODS: A retrospective analysis was per-formed by chart review of patients treated for iGCT in the northern and central region of Denmark. Teratoma only patients were not included in the study.  RESULTS: 20 patients with iGCT were diagnosed from 2008–2019 in Western Denmark. The cumulative incidence was 1.05 per 100.000. The yearly incidence was 0.1 per 100.000. Mean age at diag-nosis was 18  years (range 8–36  years), 17 were males and 3 were fe-males. 13 patients presented with germinoma and 7 patients with non germinomateous germ cell tumours (NGGCT). Three patients had dissem-inated disease, two with germinoma and one with NGGCT. All patients had received radiotherapy and 18 patients were treated with multidrug chemotherapy including platinum and etoposide before irradiation. Two patients experienced recurrent disease, both non disseminated at diag-nosis, one patient with germinoma and one patient with NGGCT. Both re-ceived salvage treatment including high dose chemotherapy with stem cell transplantation and reirradiation. Two NGGCT patients died, one patient after development of an anaplastic astrocytoma in the radiation field five years after radiotherapy and one patient after intracranial hemorraghe 18 months after salvage treatment for recurrent disease. Overall survival was 90%, 100% for GCT and 71% for NGGCT. CONCLUSION: The outcome of patients with iGCT in Western Denmark was comparable to the literature. A nationwide study of epidemiology and outcome of iGCT in Denmark is planned.GCT-66. FINAL REPORT OF THE PROSPECTIVE NEXT/CNS-GCT-4 CONSORTIUM TRIAL (GEMPOX FOLLOWED BY MARROW-ABLATIVE CHEMOTHERAPY) IN PATIENTS WITH REFRACTORY/RECURRENT CNS GERM CELL TUMORSMargaret Shatara1, Megan Blue1, Joseph R. Stanek1, Allison Y. Liu2, Diana S. Osorio1, Mohamed S. AbdelBaki1, Daniel Prevedello3, Vinay Puduvalli3, Pierre Giglio3, Sharon Gardner4, Jeffrey Allen4, Kenneth E. Wong5, Girish Dhall6,7, and Jonathan L Finlay1; 1The Division of Hematology, Oncology, Blood and Marrow Transplant, Nationwide Children’s Hospital and The Ohio State University, Columbus, OH, USA, 2University of California-Davis Health, Sacramento, CA, USA, 3The Ohio State University, Columbus, OH, USA, 4New York University School of Medicine, New York, New York, USA, 5Children’s Hospital of Los Angeles, Sacramento, CA, USA, 6The University of Alabama at Birmingham, Birmingham, AL, USA, 7Children’s Hospital of Alabama, Birmingham, AL, USABACKGROUND: We report the responses, toxicities and long-term outcomes of gemcitabine, paclitaxel and oxaliplatin (GemPOx) regimen administered, in responsive patients, prior to single cycle marrow-ablative chemotherapy (thiotepa, etoposide and carboplatin) with autologous hem-atopoietic progenitor cell rescue (HDCx+AuHPCR). METHODS: Since De-Downloaded from https://academic.oup.com/neuro-oncology/article/22/Supplement_3/iii341/6018668 by Aalborg University Library user on 15 February 2021

Incidence and outcome of intracranial malignant germ cell tumours diagnosed in western Denmark in the last decade

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Incidence and outcome of intracranial malignant germ cell tumours diagnosed in western Denmark in the last decade

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