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Hiroki Shirato - One of the best experts on this subject based on the ideXlab platform.
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impact of margin for target volume in low dose involved field radiotherapy after induction chemotherapy for intracranial Germinoma
International Journal of Radiation Oncology Biology Physics, 2004Co-Authors: Hiroki Shirato, Hidefumi Aoyama, Kazuo Miyasaka, Jun Ikeda, Yoshinobu Iwasaki, Kenji Fujieda, Norio Kato, Nobuaki Ishi, Yutaka SawamuraAbstract:Abstract Purpose We previously published a report stating that Germinomas with elevated serum beta human chorionic gonadotropin (HCG-β) had a poor relapse rate, but these findings have not been supported by a multi-institutional trial. The margin for initial gross tumor volume (GTV) before surgery and chemotherapy of the same materials was investigated by retrospective review. Methods and material The 27 patients reported on in the previous paper were analyzed. The two-dimensional margin from the initial GTV to 90% of the prescribed dose of 24 Gy was 2.0 cm for a solitary lesion in the protocol. This margin was measured retrospectively without knowledge of the serum HCG-β level. The whole ventricle field was used for patients with multifocal disease and whole central nervous system field was used for disseminated disease, respectively. Results Six relapses were seen in 18 patients with solitary tumors, and were treated with the minimum margin of 1.5 cm or less to the initial GTV. Five of the 6 had initially elevated serum HCG-β at the median of 7.4 mIU/mL, ranging from 0.7–233 mIU/mL. No relapses were seen in the 9 patients who were treated with whole ventricle or whole central nervous system field. Conclusions An inadequate margin and elevated serum HCG-β were equally determined to be candidates that caused the poor local control. The whole ventricle is recommended as the smallest target volume for Germinoma with or without elevated HCG-β after induction chemotherapy.
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pathologically proven intracranial Germinoma treated with radiation therapy
Radiotherapy and Oncology, 1998Co-Authors: Hidefumi Aoyama, Hiroki Shirato, Yoshihisa Kakuto, Hideki Inakoshi, Masamichi Nishio, Hiroshi Yoshida, Masato Hareyama, Touru Yanagisawa, Jirou Watarai, Kazuo MiyasakaAbstract:Abstract Background and purpose : A retrospective multi-institutional study was conducted to survey what percentage of intracranial Germinomas were treated with pathological confirmation before radiotherapy and to investigate the influence of field selection on outcome. Materials and methods : Thirty-seven percent of patients (41 of 110 patients) were pathologically confirmed before radiotherapy during the past 16 years at eight institutions in Northern Japanese prefectures. Pathological confirmation was obtained in 26, 37 and 53% of cases during 1978–1983, 1984–1989 and 1990–1994, respectively. All 110 patients were examined using computed tomography (CT) scans. Among the 41 patients with pathologically confirmed Germinoma, radiation fields were craniospinal in 23 patients, whole-brain in 10 patients and local without ventricle inclusion in eight patients. Results : For the 41 patients with pathologically confirmed Germinoma, the actuarial and cause-specific survival rates were 91/94% at 5 years and 87/90% at 10 years, respectively. The relapse-free survival rate at 10 years was 90, 76 and 22% for the craniospinal field, whole-brain field and local field without ventricle inclusion, respectively. Conclusion : Pathological confirmation was obtained in only 37% of CT-scan era cases, although the confirmations were more commonly carried out later in the study period. Limited local irradiation alone without ventricle inclusion cannot be recommended for localized tumors even with the help of CT scanning.
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induction chemotherapy followed by reduced volume radiation therapy for newly diagnosed central nervous system Germinoma
Journal of Neurosurgery, 1998Co-Authors: Yutaka Sawamura, Hiroki Shirato, Nobuaki Ishii, Jun Ikeda, Hiroshi Abe, Mitsuhiro Tada, Tsutomu Kato, Kenji FujiedaAbstract:Object. Although curative, radiation, which is conventionally administered for Germinomas, causes significant neurological sequelae. This study aimed at reducing the volume and dose of radiation to a localized level of 24 Gy by pretreating the patient with chemotherapy. Methods. Seventeen patients were divided into two risk groups based on serological findings and the extent of tumor. They were treated with chemotherapy prior to receiving localized radiation therapy. Six patients with solitary pure Germinomas were treated with three or four cycles of cisplatin and etoposide (EP regimen) followed by 24-Gy local radiation therapy. Eleven patients with human chorionic gonadotropin (HCG)—secreting, multifocal, or disseminated Germinomas received four to five cycles of ifosfamide, cisplatin, and etoposide (ICE regimen) followed by 24-Gy local radiation therapy. Craniospinal ports were used only in three cases of Germinomas with dissemination. Gross-total resection was performed in three patients. Fourteen pati...
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analysis of long term treatment of intracranial Germinoma
International Journal of Radiation Oncology Biology Physics, 1997Co-Authors: Hiroki Shirato, Masamichi Nishio, Yutaka Sawamura, Miyako Myohjin, Toshihiro Kitahara, Takeshi Nishioka, Yoshihide Mizutani, Hiroshi Abe, Kazuo MiyasakaAbstract:Abstract Purpose: To determine an appropriate treatment policy for intracranial Germinoma. Methods and Materials: From 1976 to 1992, 51 patients with Germinoma (18 with pathologically verified Germinoma and 33 diagnosed as having Germinoma by neuroimagings) were treated by radiation therapy. Various radiation doses and treatment fields were used. Results:The 10-year cause-specific survival rate for pathologically verified and unverified Germinoma was 100% and 96%, respectively. Relapse were noted in four patients, three of whom died from the disease. Two of the four patients with a tumor larger than 4 cm in gross diameter experienced relapse. Two relapse occurred in a nonirradiated spinal canal and two occurred in the irradiation area treated by 25 Gy in 10 fractions and 30 Gy in 20 fractions. No relapse was noted in patients in whom the whole ventricle field was determined precisely using three-dimensional treatment planning. Five patients who were followed at an outpatient clinic experienced significant late neurocognitive dysfunction, which set in after radiotherapy. Conclusion: After pathological confirmation, 40 Gy whole-ventricle irradiation using precise three-dimensional treatment planning is appropriate as a standard treatment for most intracranial Germinoma.
Kazuo Miyasaka - One of the best experts on this subject based on the ideXlab platform.
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impact of margin for target volume in low dose involved field radiotherapy after induction chemotherapy for intracranial Germinoma
International Journal of Radiation Oncology Biology Physics, 2004Co-Authors: Hiroki Shirato, Hidefumi Aoyama, Kazuo Miyasaka, Jun Ikeda, Yoshinobu Iwasaki, Kenji Fujieda, Norio Kato, Nobuaki Ishi, Yutaka SawamuraAbstract:Abstract Purpose We previously published a report stating that Germinomas with elevated serum beta human chorionic gonadotropin (HCG-β) had a poor relapse rate, but these findings have not been supported by a multi-institutional trial. The margin for initial gross tumor volume (GTV) before surgery and chemotherapy of the same materials was investigated by retrospective review. Methods and material The 27 patients reported on in the previous paper were analyzed. The two-dimensional margin from the initial GTV to 90% of the prescribed dose of 24 Gy was 2.0 cm for a solitary lesion in the protocol. This margin was measured retrospectively without knowledge of the serum HCG-β level. The whole ventricle field was used for patients with multifocal disease and whole central nervous system field was used for disseminated disease, respectively. Results Six relapses were seen in 18 patients with solitary tumors, and were treated with the minimum margin of 1.5 cm or less to the initial GTV. Five of the 6 had initially elevated serum HCG-β at the median of 7.4 mIU/mL, ranging from 0.7–233 mIU/mL. No relapses were seen in the 9 patients who were treated with whole ventricle or whole central nervous system field. Conclusions An inadequate margin and elevated serum HCG-β were equally determined to be candidates that caused the poor local control. The whole ventricle is recommended as the smallest target volume for Germinoma with or without elevated HCG-β after induction chemotherapy.
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pathologically proven intracranial Germinoma treated with radiation therapy
Radiotherapy and Oncology, 1998Co-Authors: Hidefumi Aoyama, Hiroki Shirato, Yoshihisa Kakuto, Hideki Inakoshi, Masamichi Nishio, Hiroshi Yoshida, Masato Hareyama, Touru Yanagisawa, Jirou Watarai, Kazuo MiyasakaAbstract:Abstract Background and purpose : A retrospective multi-institutional study was conducted to survey what percentage of intracranial Germinomas were treated with pathological confirmation before radiotherapy and to investigate the influence of field selection on outcome. Materials and methods : Thirty-seven percent of patients (41 of 110 patients) were pathologically confirmed before radiotherapy during the past 16 years at eight institutions in Northern Japanese prefectures. Pathological confirmation was obtained in 26, 37 and 53% of cases during 1978–1983, 1984–1989 and 1990–1994, respectively. All 110 patients were examined using computed tomography (CT) scans. Among the 41 patients with pathologically confirmed Germinoma, radiation fields were craniospinal in 23 patients, whole-brain in 10 patients and local without ventricle inclusion in eight patients. Results : For the 41 patients with pathologically confirmed Germinoma, the actuarial and cause-specific survival rates were 91/94% at 5 years and 87/90% at 10 years, respectively. The relapse-free survival rate at 10 years was 90, 76 and 22% for the craniospinal field, whole-brain field and local field without ventricle inclusion, respectively. Conclusion : Pathological confirmation was obtained in only 37% of CT-scan era cases, although the confirmations were more commonly carried out later in the study period. Limited local irradiation alone without ventricle inclusion cannot be recommended for localized tumors even with the help of CT scanning.
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analysis of long term treatment of intracranial Germinoma
International Journal of Radiation Oncology Biology Physics, 1997Co-Authors: Hiroki Shirato, Masamichi Nishio, Yutaka Sawamura, Miyako Myohjin, Toshihiro Kitahara, Takeshi Nishioka, Yoshihide Mizutani, Hiroshi Abe, Kazuo MiyasakaAbstract:Abstract Purpose: To determine an appropriate treatment policy for intracranial Germinoma. Methods and Materials: From 1976 to 1992, 51 patients with Germinoma (18 with pathologically verified Germinoma and 33 diagnosed as having Germinoma by neuroimagings) were treated by radiation therapy. Various radiation doses and treatment fields were used. Results:The 10-year cause-specific survival rate for pathologically verified and unverified Germinoma was 100% and 96%, respectively. Relapse were noted in four patients, three of whom died from the disease. Two of the four patients with a tumor larger than 4 cm in gross diameter experienced relapse. Two relapse occurred in a nonirradiated spinal canal and two occurred in the irradiation area treated by 25 Gy in 10 fractions and 30 Gy in 20 fractions. No relapse was noted in patients in whom the whole ventricle field was determined precisely using three-dimensional treatment planning. Five patients who were followed at an outpatient clinic experienced significant late neurocognitive dysfunction, which set in after radiotherapy. Conclusion: After pathological confirmation, 40 Gy whole-ventricle irradiation using precise three-dimensional treatment planning is appropriate as a standard treatment for most intracranial Germinoma.
Chang Ok Suh - One of the best experts on this subject based on the ideXlab platform.
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treatment outcomes based on radiation therapy fields for bifocal Germinoma synchronous or disseminated disease
PLOS ONE, 2019Co-Authors: Seung Yeun Chung, Jung Woo Han, Dongseok Kim, Hong In Yoon, Chang Ok SuhAbstract:Intracranial Germinoma sometimes present as bifocal Germinoma, and whether bifocal Germinoma should be treated as a synchronous or disseminated disease remains unclear. This study aimed to determine the optimal treatment modality for bifocal Germinoma. Patients with bifocal Germinoma who received radiotherapy (RT) from March 1990 to August 2017 were included for analysis. A total of 21 patients were included. The median follow-up period was 76.2 months (range, 6.2–305.4 months). There were 17 patients who received cranio-spinal irradiation (CSI) with local RT; 3, whole ventricular RT (WVRT) with local RT; and 1, local RT only. Three recurrences occurred (1 patient each among those who underwent CSI, WVRT, and local RT). Recurrence in the patient who received CSI and who received WVRT occurred in the right thalamus and right frontal convexity, respectively. Meanwhile, the patient who received local RT showed not only a recurred lesion in the hypothalamus, but also cerebrospinal fluid seeding. For this patient, salvage CSI was performed and complete response was achieved after treatment. However, after 9 years and 6 months, he was diagnosed with glioblastoma and expired. As for toxicity, although 17 patients showed decrease in complete blood count levels during treatment, all patients recovered soon after treatment completion. Our findings suggest that bifocal Germinoma may be considered as a disseminated disease when considering the patterns of failure according to RT fields. In addition, patients who received CSI showed low acute toxicity rates. However, further studies are necessary to confirm these findings.
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upfront chemotherapy followed by response adaptive radiotherapy for intracranial Germinoma prospective multicenter cohort study
Radiotherapy and Oncology, 2019Co-Authors: Dong Soo Lee, Keon Hee Yoo, Do Hoon Lim, Il Han Kim, Jooyoung Kim, Jung Woo Han, Kyung Duk Park, Hyeon Jin Park, Nack Gyun Chung, Chang Ok SuhAbstract:Abstract Purpose To assess the efficacy of upfront chemotherapy followed by response-adapted reduced-dose/reduced-volume radiotherapy (RT) for intracranial Germinoma. Materials and methods Ninety-one patients from five institutions were registered in the KSPNO G051/G081 Protocol. Germinomas were classified as solitary or multiple/disseminated diseases, and upfront chemotherapy was administered. For all patients with multiple or disseminated disease, and patients with partial response after chemotherapy, 19.5–24 Gy of craniospinal irradiation plus 10.8–19.8 Gy of tumor bed boost were planned. For patients with complete response (CR), reduced-dose RT (30.6 Gy) was planned, along with a reduced field for solitary lesions. Results The median patient age was 14 (range, 3–30) years. Sixty-five patients (71.4%) had a solitary lesion. The median follow-up duration was 67.9 (range, 6.6–119.3) months. Recurrence was not observed in 32 patients in the protocol compliant group. Four patients (4.4%) in the protocol non-compliant group experienced relapse after CR and one patient died of the disease. The 5-year and 7-year overall survival rates were 98.8% and 98.8%, while the corresponding event-free survival rates were 96.6% and 93.8%, respectively. All three patients with basal ganglia Germinomas who were treated with local RT experienced recurrence outside the RT field. Among the 23 patients with pineal or suprasellar lesions who received whole-ventricle RT, there was no recurrence. Conclusions Currently used upfront chemotherapy followed by reduced-dose, reduced-volume RT appears acceptable, when whole-ventricle RT for pineal or suprasellar tumors and, at minimum, whole-brain RT for basal ganglia/thalamus lesions are applied.
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treatment strategy for intracranial primary pure Germinoma
Childs Nervous System, 2013Co-Authors: Kyu Won Shim, Eun Kyung Park, Yoonho Lee, Chang Ok Suh, Jaeho Cho, J U Choi, Dongseok KimAbstract:This prospective randomized clinical study will address the efficacy of radiation (RT)-alone and combined with pre-RT chemotherapy (CTX) treatments and propose the novel standard treatment strategy for intracranial primary pure Germinoma. Between 2005 and 2008, there were 54 patients diagnosed with intracranial primary pure Germinomas in a single institute. Twenty-eight patients were enrolled. The mean age of the patients was 16.2 years (range 6–31 years). There were 19 men and 9 women (men/women ratio = 2.1:1). There were 21 patients with solitary tumors and 7 with multiple tumors. These patients were randomized as RT-only treatment group (11 solitary and 3 multiple tumors) and combined (10 solitary and 4 multiple tumors, neo-adjuvant CTX followed by response-adapted RT) treatment group. The follow-up period for RT only group has a median of 58 months (mean 58.2 months, range 41–82 months), and for combine therapy group, the median was 68.5 months (mean 67.8 months, range 41–88 months). All 14 patients in the RT-only group showed complete response (CR) and no recurrence. Eleven patients in the combined group had CR and three patients had partial response after neo-adjuvant CTX. All patients responded to RT as CR without recurrence. At the time of analysis, all 28 patients were alive without evidence of disease. Neo-adjuvant CTX for localized Germinomas seems to be unnecessary as a method to reduce radiation dose in our RT protocol. However, the effective control of multifocal or disseminated Germinoma can be achieved by neo-adjuvant CTX followed by response-adapted reduced dose RT.
Eric Bouffet - One of the best experts on this subject based on the ideXlab platform.
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redefining ventricular target volume in Germinoma is inclusion of temporal horns necessary
International Journal of Radiation Oncology Biology Physics, 2019Co-Authors: Eric Bouffet, Ute Bartels, Normand Laperriere, Michael Yan, Michael Velec, Vijay Ramaswamy, Derek S TsangAbstract:Purpose We reviewed the outcomes of a retrospective Germinoma cohort and analyzed radiation therapy plans to determine dosimetric differences for critical structures. Methods and Materials Data from pediatric patients treated with photon radiation for intracranial Germinoma were analyzed for clinical outcomes and dosimetry to critical structures, with particular interest in the temporal ventricular horns (TVHs). A consensus contour was generated for TVH-sparing ventricular clinical target volumes (CTVs) via deformable image registration. Results Twelve and 10 patients had their TVHs included or excluded in their ventricular CTVs, respectively. All patients were living at the time of analysis. One patient relapsed in the fourth ventricle, which had been omitted from the radiation therapy field. Mean dose was significantly lower to the hippocampi (Δ = –578 cGy, P = .0016) and temporal lobes (Δ = –599 cGy, P = .0007) in the TVH-excluded cohort compared with those with TVHs included in the treatment field. Conclusions Exclusion of the TVHs from the CTV results in significant dose sparing to the hippocampi and temporal lobes. Clinical outcomes remain excellent with no deaths and no TVH failures. Exclusion of TVHs from the ventricular CTV in Germinoma requires prospective study.
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outcomes of children with central nervous system Germinoma treated with multi agent chemotherapy followed by reduced radiation
Journal of Neuro-oncology, 2016Co-Authors: Eric Bouffet, James M Drake, Johnpaul Kilday, Sylvia Cheng, Normand Laperriere, Laura Janzen, Ute BartelsAbstract:CNS Germinomas have an excellent prognosis with radiation therapy alone. However, in children, volume and dose of CNS radiation are associated with neurocognitive and neuroendocrine sequelae. Our objective was to determine long-term outcomes of our cohort who received chemotherapy and reduced radiation. This retrospective cohort study analyzed treatment and outcome of intracranial Germinoma patients consecutively treated at Sick Kids, Toronto, Canada, from January 2000 to December 2013. 24 children (13 male, 11 female; median age 13.36 years) were identified. Median follow up was 61 months (range 1–144 months). Tumor location was suprasellar (n = 9), bifocal (8), pineal (6), and basal ganglia (1). Three children showed dissemination on imaging. 2/24 had only elevated serum human chorionic gonadotropin, 3/24 only elevated lumbar cerebrospinal fluid (CSF) hCG, and 2/24 had both elevated serum and lumbar CSF hCG. 23/24 children completed treatment and received multi-agent chemotherapy followed by either ventricular radiation (2340–2400 cGy) (n = 9), ventricular radiation + boost (1600 cGy) (n = 8), whole brain (2340 cGy) (n = 3), focal (4000 cGy) (n = 2) or craniospinal radiation (2340 cGy) (n = 1). Five-year progression free and overall survival was 96 and 100 % respectively. 8/24 patients with ventricular radiation ± boost (2340/4000 cGy) displayed stable full scale intelligence quotient over a mean interval of 3 years following radiation, but showed declined processing speed. In this limited experience, excellent 5-year overall survival rates were achieved with chemotherapy followed by reduced whole ventricular radiation even if ventricular radiation was delivered without boost.
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diabetes insipidus in pediatric Germinomas of the suprasellar region characteristic features and significance of the pituitary bright spot
Journal of Neuro-oncology, 2015Co-Authors: Johnpaul Kilday, Eric Bouffet, Suzanne Laughlin, Stacey Urbach, Ute BartelsAbstract:The pituitary bright spot is acknowledged to indicate functional integrity of the posterior pituitary gland, whilst its absence supports a diagnosis of central diabetes insipidus (DI). This feature was evaluated, together with the incidence and clinical characteristics of DI in children with suprasellar/neurohypophyseal Germinomas. We performed a review of all suprasellar (SS) or bifocal (BF) Germinoma pediatric patients treated in Toronto since 2000. Demographics, symptomatology, treatment outcome and imaging were evaluated. Nineteen patients fulfilled inclusion criteria (10 SS, 9 BF; median age 12.5 years (6.2–16.8 years)). All remained alive at 6.4 years median follow-up (1.2–13.7 years) after receiving chemotherapy and radiotherapy (13 focal/ventricular, four whole brain, two neuraxis), with only one progression. All had symptoms of DI at presentation with a symptom interval above one year in eight cases (42 %). Desmopressin was commenced and maintained in 16 patients (84 %). The pituitary bright spot was lost in most diagnostic interpretable cases, but was appreciated in three patients (18 %) who had normal serum sodium values compared to ‘absent’ cases (p = 0.013). For two such cases, spots remained visible until last follow-up (range 0.4–3.3 years), with one still receiving desmopressin. No case of bright spot recovery was observed following therapy. Protracted symptom intervals for Germinoma-induced central DI may reflect poor clinical awareness. Explanations for persistence of the pituitary bright spot in symptomatic patients remain elusive. Desmopressin seldom reverses the clinical features of Germinoma-induced DI to allow discontinuation, nor does treatment cause bright spot recovery.
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Inherent diagnostic and treatment challenges in Germinoma of the basal ganglia: a case report and review of the literature
Journal of Neuro-Oncology, 2008Co-Authors: Anita Villani, Eric Bouffet, Susan Blaser, Barbara-ann Millar, Cynthia Hawkins, Ute BartelsAbstract:Among intracranial Germinomas, basal ganglia Germinomas represent a specific clinical and anatomical entity. Based on an unusual case of a basal ganglia Germinoma in a 13-year-old Caucasian male, we highlight the diagnostic challenges and discuss treatment considerations in this disease.
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limited field radiation for bifocal Germinoma
International Journal of Radiation Oncology Biology Physics, 2006Co-Authors: Lucie Lafaycousin, Barbara-ann Millar, Ute Bartels, Donald J Mabbott, Brenda J Spiegler, Jim Drake, Annie Huang, Eric BouffetAbstract:Purpose: To report the incidence, characteristics, treatment, and outcomes of bifocal Germinomas treated with chemotherapy followed by focal radiation. Methods and Materials: This was a retrospective review. Inclusion criteria included radiologic diagnosis of bifocal Germinoma involving the pineal and neurohypophyseal region, no evidence of dissemination on spinal MRI, negative results from cerebrospinal fluid cytologic evaluation, and negative tumor markers. Results: Between 1995 and 2004, 6 patients (5 male, 1 female; median age, 12.8 years) fulfilled the inclusion criteria. All had symptoms of diabetes insipidus at presentation. On MRI, 4 patients had a pineal and suprasellar mass, and 2 had a pineal mass associated with abnormal neurohypophyseal enhancement. All patients received chemotherapy followed by limited-field radiation and achieved complete remission after chemotherapy. The radiation field involved the whole ventricular system (range, 2,400-4,000 cGy) with or without a boost to the primary lesions. All patients remain in complete remission at a median follow-up of 48.1 months (range, 9-73.4 months). Conclusions: This experience suggests that bifocal Germinoma can be considered a locoregional rather than a metastatic disease. Chemotherapy and focal radiotherapy might be sufficient to provide excellent outcomes. Staging refinement with new diagnostic tools will likely increase the incidence of the entity.
Ute Bartels - One of the best experts on this subject based on the ideXlab platform.
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redefining ventricular target volume in Germinoma is inclusion of temporal horns necessary
International Journal of Radiation Oncology Biology Physics, 2019Co-Authors: Eric Bouffet, Ute Bartels, Normand Laperriere, Michael Yan, Michael Velec, Vijay Ramaswamy, Derek S TsangAbstract:Purpose We reviewed the outcomes of a retrospective Germinoma cohort and analyzed radiation therapy plans to determine dosimetric differences for critical structures. Methods and Materials Data from pediatric patients treated with photon radiation for intracranial Germinoma were analyzed for clinical outcomes and dosimetry to critical structures, with particular interest in the temporal ventricular horns (TVHs). A consensus contour was generated for TVH-sparing ventricular clinical target volumes (CTVs) via deformable image registration. Results Twelve and 10 patients had their TVHs included or excluded in their ventricular CTVs, respectively. All patients were living at the time of analysis. One patient relapsed in the fourth ventricle, which had been omitted from the radiation therapy field. Mean dose was significantly lower to the hippocampi (Δ = –578 cGy, P = .0016) and temporal lobes (Δ = –599 cGy, P = .0007) in the TVH-excluded cohort compared with those with TVHs included in the treatment field. Conclusions Exclusion of the TVHs from the CTV results in significant dose sparing to the hippocampi and temporal lobes. Clinical outcomes remain excellent with no deaths and no TVH failures. Exclusion of TVHs from the ventricular CTV in Germinoma requires prospective study.
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outcomes of children with central nervous system Germinoma treated with multi agent chemotherapy followed by reduced radiation
Journal of Neuro-oncology, 2016Co-Authors: Eric Bouffet, James M Drake, Johnpaul Kilday, Sylvia Cheng, Normand Laperriere, Laura Janzen, Ute BartelsAbstract:CNS Germinomas have an excellent prognosis with radiation therapy alone. However, in children, volume and dose of CNS radiation are associated with neurocognitive and neuroendocrine sequelae. Our objective was to determine long-term outcomes of our cohort who received chemotherapy and reduced radiation. This retrospective cohort study analyzed treatment and outcome of intracranial Germinoma patients consecutively treated at Sick Kids, Toronto, Canada, from January 2000 to December 2013. 24 children (13 male, 11 female; median age 13.36 years) were identified. Median follow up was 61 months (range 1–144 months). Tumor location was suprasellar (n = 9), bifocal (8), pineal (6), and basal ganglia (1). Three children showed dissemination on imaging. 2/24 had only elevated serum human chorionic gonadotropin, 3/24 only elevated lumbar cerebrospinal fluid (CSF) hCG, and 2/24 had both elevated serum and lumbar CSF hCG. 23/24 children completed treatment and received multi-agent chemotherapy followed by either ventricular radiation (2340–2400 cGy) (n = 9), ventricular radiation + boost (1600 cGy) (n = 8), whole brain (2340 cGy) (n = 3), focal (4000 cGy) (n = 2) or craniospinal radiation (2340 cGy) (n = 1). Five-year progression free and overall survival was 96 and 100 % respectively. 8/24 patients with ventricular radiation ± boost (2340/4000 cGy) displayed stable full scale intelligence quotient over a mean interval of 3 years following radiation, but showed declined processing speed. In this limited experience, excellent 5-year overall survival rates were achieved with chemotherapy followed by reduced whole ventricular radiation even if ventricular radiation was delivered without boost.
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diabetes insipidus in pediatric Germinomas of the suprasellar region characteristic features and significance of the pituitary bright spot
Journal of Neuro-oncology, 2015Co-Authors: Johnpaul Kilday, Eric Bouffet, Suzanne Laughlin, Stacey Urbach, Ute BartelsAbstract:The pituitary bright spot is acknowledged to indicate functional integrity of the posterior pituitary gland, whilst its absence supports a diagnosis of central diabetes insipidus (DI). This feature was evaluated, together with the incidence and clinical characteristics of DI in children with suprasellar/neurohypophyseal Germinomas. We performed a review of all suprasellar (SS) or bifocal (BF) Germinoma pediatric patients treated in Toronto since 2000. Demographics, symptomatology, treatment outcome and imaging were evaluated. Nineteen patients fulfilled inclusion criteria (10 SS, 9 BF; median age 12.5 years (6.2–16.8 years)). All remained alive at 6.4 years median follow-up (1.2–13.7 years) after receiving chemotherapy and radiotherapy (13 focal/ventricular, four whole brain, two neuraxis), with only one progression. All had symptoms of DI at presentation with a symptom interval above one year in eight cases (42 %). Desmopressin was commenced and maintained in 16 patients (84 %). The pituitary bright spot was lost in most diagnostic interpretable cases, but was appreciated in three patients (18 %) who had normal serum sodium values compared to ‘absent’ cases (p = 0.013). For two such cases, spots remained visible until last follow-up (range 0.4–3.3 years), with one still receiving desmopressin. No case of bright spot recovery was observed following therapy. Protracted symptom intervals for Germinoma-induced central DI may reflect poor clinical awareness. Explanations for persistence of the pituitary bright spot in symptomatic patients remain elusive. Desmopressin seldom reverses the clinical features of Germinoma-induced DI to allow discontinuation, nor does treatment cause bright spot recovery.
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Inherent diagnostic and treatment challenges in Germinoma of the basal ganglia: a case report and review of the literature
Journal of Neuro-Oncology, 2008Co-Authors: Anita Villani, Eric Bouffet, Susan Blaser, Barbara-ann Millar, Cynthia Hawkins, Ute BartelsAbstract:Among intracranial Germinomas, basal ganglia Germinomas represent a specific clinical and anatomical entity. Based on an unusual case of a basal ganglia Germinoma in a 13-year-old Caucasian male, we highlight the diagnostic challenges and discuss treatment considerations in this disease.
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limited field radiation for bifocal Germinoma
International Journal of Radiation Oncology Biology Physics, 2006Co-Authors: Lucie Lafaycousin, Barbara-ann Millar, Ute Bartels, Donald J Mabbott, Brenda J Spiegler, Jim Drake, Annie Huang, Eric BouffetAbstract:Purpose: To report the incidence, characteristics, treatment, and outcomes of bifocal Germinomas treated with chemotherapy followed by focal radiation. Methods and Materials: This was a retrospective review. Inclusion criteria included radiologic diagnosis of bifocal Germinoma involving the pineal and neurohypophyseal region, no evidence of dissemination on spinal MRI, negative results from cerebrospinal fluid cytologic evaluation, and negative tumor markers. Results: Between 1995 and 2004, 6 patients (5 male, 1 female; median age, 12.8 years) fulfilled the inclusion criteria. All had symptoms of diabetes insipidus at presentation. On MRI, 4 patients had a pineal and suprasellar mass, and 2 had a pineal mass associated with abnormal neurohypophyseal enhancement. All patients received chemotherapy followed by limited-field radiation and achieved complete remission after chemotherapy. The radiation field involved the whole ventricular system (range, 2,400-4,000 cGy) with or without a boost to the primary lesions. All patients remain in complete remission at a median follow-up of 48.1 months (range, 9-73.4 months). Conclusions: This experience suggests that bifocal Germinoma can be considered a locoregional rather than a metastatic disease. Chemotherapy and focal radiotherapy might be sufficient to provide excellent outcomes. Staging refinement with new diagnostic tools will likely increase the incidence of the entity.