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Purpose@#This multicenter retrospective study aimed to investigate clinical, radiologic, and treatment-related factors affecting survival in patients with newly diagnosed diffuse intrinsic pontine glioma (DIPG) treated with radiotherapy. @*Materials and Methods@#Patients aged <30 years who underwent radiotherapy as an initial treatment for DIPG between 2000 and 2018 were included; patients who did not undergo magnetic resonance imaging at diagnosis and those with pathologically diagnosed grade I glioma were excluded. We examined medical records of 162 patients collected from 10 participating centers in Korea. The patients’ clinical, radiological, molecular, and histopathologic characteristics, and treatment responses were evaluated to identify the prognosticators for DIPG and estimate survival outcomes. @*Results@#The median follow-up period was 10.8 months (interquartile range, 7.5 to 18.1). The 1- and 2-year overall survival (OS) rates were 53.5% and 19.0%, respectively, with a median OS of 13.1 months. Long-term survival rate (≥ 2 years) was 16.7%, and median OS was 43.6 months. Age (< 10 years), poor performance status, treatment before 2010, and post-radiotherapy necrosis were independently associated with poor OS in multivariate analysis. In patients with increased post-radiotherapy necrosis, the median OS estimates were 13.3 months and 11.4 months with and without bevacizumab, respectively (p=0.138). @*Conclusion@#Therapeutic strategy for DIPG has remained unchanged over time, and the associated prognosis remains poor. Our findings suggest that appropriate efforts are needed to reduce the occurrence of post-radiotherapy necrosis. Further well-designed clinical trials are recommended to improve the poor prognosis observed in DIPG patients.
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Purpose@#This study aimed to evaluate the role of postoperative radiotherapy (PORT) in intracranial solitary fibrous tumor/hemangiopericytoma (SFT/HPC). @*Materials and Methods@#A total of 133 patients with histologically confirmed HPC were included from eight institutions. Gross total resection (GTR) and subtotal resection (STR) were performed in 86 and 47 patients, respectively. PORT was performed in 85 patients (64%). The prognostic effects of sex, age, performance, World Health Organization (WHO) grade, location, size, Ki-67, surgical extent, and PORT on local control (LC), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS) were estimated by univariate and multivariate analyses. @*Results@#The 10-year PFS, and OS rates were 45%, and 71%, respectively. The multivariate analysis suggested that PORT significantly improved LC (p < 0.001) and PFS (p < 0.001). The PFS benefit of PORT was maintained in the subgroup of GTR (p=0.001), WHO grade II (p=0.001), or STR (p < 0.001). In the favorable subgroup of GTR and WHO grade II, PORT was also significantly related to better PFS (p=0.028). WHO grade III was significantly associated with poor DMFS (p=0.029). In the PORT subgroup, the 0-0.5 cm margin of the target volume showed an inferior LC to a large margin with 1.0-2.0 cm (p=0.021). Time-dependent Cox proportion analysis showed that distant failures were significantly associated with poor OS (p=0.003). @*Conclusion@#This multicenter study supports the role of PORT in disease control of intracranial SFT/HPC, irrespective of the surgical extent and grade. For LC, PORT should enclose the tumor bed with sufficient margin.
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Background@#There have been no guidelines for the management of adult patients with diffuse midline glioma (DMG), H3K27M-mutant in Korea since the 2016 revised WHO classification newly defined this disease entity. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, had begun preparing guidelines for DMG since 2019. @*Methods@#The Working Group was composed of 27 multidisciplinary medical experts in Korea.References were identified through searches of PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL using specific and sensitive keywords as well as combinations of keywords. As ‘diffuse midline glioma’ was recently defined, and there was no international guideline, trials and guidelines of ‘diffuse intrinsic pontine glioma’ or ‘brain stem glioma’ were thoroughly reviewed first. @*Results@#The core contents are as follows. The DMG can be diagnosed when all of the following three criteria are satisfied: the presence of the H3K27M mutation, midline location, and infiltrating feature. Without identification of H3K27M mutation by diagnostic biopsy, DMG cannot be diagnosed. For the primary treatment, maximal safe resection should be considered for tumors when feasible. Radiotherapy is the primary option for tumors in case the total resection is not possible. A total dose of 54 Gy to 60 Gy with conventional fractionation prescribed at 1-2 cm plus gross tumor volume is recommended. Although no chemotherapy has proven to be effective in DMG, concurrent chemoradiotherapy (± maintenance chemotherapy) with temozolomide following WHO grade IV glioblastoma’s protocol is recommended. @*Conclusion@#The detection of H3K27M mutation is the most important diagnostic criteria for DMG. Combination of surgery (if amenable to surgery), radiotherapy, and chemotherapy based on comprehensive multidisciplinary discussion can be considered as the treatment options for DMG.
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Background@#To date, there has been no practical guidelines for the prescription of antiepileptic drugs (AEDs) in brain tumor patients in Korea. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, had begun preparing guidelines for AED usage in brain tumors since 2019. @*Methods@#The Working Group was composed of 27 multidisciplinary medical experts in Korea.References were identified through searches of PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL using specific and sensitive keywords as well as combinations of the keywords. @*Results@#The core contents are as follows. Prophylactic AED administration is not recommended in newly diagnosed brain tumor patients without previous seizure history. When AEDs are administered during peri/postoperative period, it may be tapered off according to the following recommendations. In seizure-naïve patients with no postoperative seizure, it is recommended to stop or reduce AED 1 week after surgery. In seizure-naïve patients with one early postoperative seizure (<1 week after surgery), it is advisable to maintain AED for at least 3 months before tapering. In seizure-naïve patients with ≥2 postoperative seizures or in patients with preoperative seizure history, it is recommended to maintain AEDs for more than 1 year. The possibility of drug interactions should be considered when selecting AEDs in brain tumor patients. Driving can be allowed in brain tumor patients when proven to be seizure-free for more than 1 year. @*Conclusion@#The KSNO suggests prescribing AEDs in patients with brain tumor based on the current guideline. This guideline will contribute to spreading evidence-based prescription of AEDs in brain tumor patients in Korea.
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Background@#There have been no guidelines for the management of adult patients with diffuse midline glioma (DMG), H3K27M-mutant in Korea since the 2016 revised WHO classification newly defined this disease entity. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, had begun preparing guidelines for DMG since 2019. @*Methods@#The Working Group was composed of 27 multidisciplinary medical experts in Korea.References were identified through searches of PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL using specific and sensitive keywords as well as combinations of keywords. As ‘diffuse midline glioma’ was recently defined, and there was no international guideline, trials and guidelines of ‘diffuse intrinsic pontine glioma’ or ‘brain stem glioma’ were thoroughly reviewed first. @*Results@#The core contents are as follows. The DMG can be diagnosed when all of the following three criteria are satisfied: the presence of the H3K27M mutation, midline location, and infiltrating feature. Without identification of H3K27M mutation by diagnostic biopsy, DMG cannot be diagnosed. For the primary treatment, maximal safe resection should be considered for tumors when feasible. Radiotherapy is the primary option for tumors in case the total resection is not possible. A total dose of 54 Gy to 60 Gy with conventional fractionation prescribed at 1-2 cm plus gross tumor volume is recommended. Although no chemotherapy has proven to be effective in DMG, concurrent chemoradiotherapy (± maintenance chemotherapy) with temozolomide following WHO grade IV glioblastoma’s protocol is recommended. @*Conclusion@#The detection of H3K27M mutation is the most important diagnostic criteria for DMG. Combination of surgery (if amenable to surgery), radiotherapy, and chemotherapy based on comprehensive multidisciplinary discussion can be considered as the treatment options for DMG.
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Background@#To date, there has been no practical guidelines for the prescription of antiepileptic drugs (AEDs) in brain tumor patients in Korea. Thus, the Korean Society for Neuro-Oncology (KSNO), a multidisciplinary academic society, had begun preparing guidelines for AED usage in brain tumors since 2019. @*Methods@#The Working Group was composed of 27 multidisciplinary medical experts in Korea.References were identified through searches of PubMed, MEDLINE, EMBASE, and Cochrane CENTRAL using specific and sensitive keywords as well as combinations of the keywords. @*Results@#The core contents are as follows. Prophylactic AED administration is not recommended in newly diagnosed brain tumor patients without previous seizure history. When AEDs are administered during peri/postoperative period, it may be tapered off according to the following recommendations. In seizure-naïve patients with no postoperative seizure, it is recommended to stop or reduce AED 1 week after surgery. In seizure-naïve patients with one early postoperative seizure (<1 week after surgery), it is advisable to maintain AED for at least 3 months before tapering. In seizure-naïve patients with ≥2 postoperative seizures or in patients with preoperative seizure history, it is recommended to maintain AEDs for more than 1 year. The possibility of drug interactions should be considered when selecting AEDs in brain tumor patients. Driving can be allowed in brain tumor patients when proven to be seizure-free for more than 1 year. @*Conclusion@#The KSNO suggests prescribing AEDs in patients with brain tumor based on the current guideline. This guideline will contribute to spreading evidence-based prescription of AEDs in brain tumor patients in Korea.
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Background@#: The Guideline Working Group of the Korean Society for Neuro-Oncology (KSNO)conducted a nationwide questionnaire survey for diverse queries faced in the treatment of brain tumors.As part I of the survey, the aim of this study is to evaluate national patterns of clinical practiceabout antiepileptic drug (AED) and steroid usage for management of brain tumors. @*Methods@#: A web-based survey was sent to all members of the KSNO by email. The survey included9 questions of AED usage and 5 questions of steroid usage for brain tumor patients. All questionswere developed by consensus of the Guideline Working Group. @*Results@#: The overall response rate was 12.8% (54/423). Regarding AED usage, the majority ofrespondents (95.2%) routinely prescribed prophylactic AEDs for patients with seizure at the peri/postoperativeperiod. However, as many as 72.8% of respondents prescribed AED routinely for seizure-naïvepatients, and others prescribed AED as the case may be. The duration of AED prophylaxis showedwide variance according to the epilepsy status and the location of tumor. Levetiracetam (82.9%) wasthe most preferred AED for epilepsy prophylaxis. Regarding steroid usage, 90.5% of respondents usesteroids in perioperative period, including 34.2% of them as a routine manner. Presence of peritumoraledema (90.9%) was considered as the most important factor determining steroid usage followed bydegree of clinical symptoms (60.6%). More than half of respondents (51.2%) replied to discontinue thesteroids within a week after surgery if there are no specific medical conditions, while 7.3% preferredslow tapering up to a month after surgery. @*Conclusion@#: The survey demonstrated the prevailing practice patterns on AED and steroid usagein neuro-oncologic field among members of the KSNO. This information provides a point of referencefor establishing a practical guideline in the management of brain tumor patients.
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Background@#: The Guideline Working Group of the Korean Society for Neuro-Oncology (KSNO)conducted a nationwide questionnaire survey for diverse queries faced in the treatment of brain tumors.As part II of the survey, the aim of this study is to evaluate the national patterns of clinical practicefor patients with diffuse midline glioma and meningioma. @*Methods@#: A web-based survey was sent to all members of the KSNO by email. The survey included4 questions of diffuse midline glioma and 6 questions of meningioma (including 2 case scenarios).All questions were developed by consensus of the Guideline Working Group. @*Results@#: In the survey about diffuse midline glioma, 76% respondents performed histologicconfirmation to identify H3K27M mutation on immunohistochemical staining or sequencing methods.For treatment of diffuse midline glioma, respondents preferred concurrent chemoradiotherapy withtemozolomide (TMZ) and adjuvant TMZ (63.8%) than radiotherapy alone (34.0%). In the surveyabout meningioma, respondents prefer wait-and-see policy for the asymptomatic small meningiomawithout peritumoral edema. However, a greater number of respondents had chosen surgical resectionas the first choice for all large size meningiomas without exception, and small size meningiomaswith either peritumoral edema or eloquent location. There was no single opinion with major consensuson long-term follow-up plans for asymptomatic meningioma with observation policy. As many as68.1% of respondents answered that they would not add any adjuvant therapies for World Health Organizationgrade II meningiomas if the tumor was totally resected including dura. @*Conclusion@#: The survey demonstrates the prevailing clinical practice patterns for patients with diffusemidline glioma and meningioma among members of the KSNO. This information provides a pointof reference for establishing a practical guideline in the management of diffuse midline glioma andmeningioma.
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Background@#: The Guideline Working Group of the Korean Society for Neuro-Oncology (KSNO)conducted the nationwide questionnaire survey for diverse queries facing to treat patients with braintumor. As part III of the survey, the aim of this study is to evaluate the national patterns of clinical practicefor patients with brain metastasis and primary central nervous system lymphoma (PCNSL). @*Methods@#: A web-based survey was sent to all members of the KSNO by email. The survey included7 questions of brain metastasis and 5 questions of PCNSL, focused on the management strategiesin specific situations. All questions were developed by consensus of the Guideline WorkingGroup. @*Results@#" In the survey about brain metastasis, respondents preferred surgical resection withadjuvant treatment for patients with a surgically accessible single brain metastatic lesion less than 3cm in size without extracranial systemic lesions. However, most respondents considered radiosurgeryfor surgically inaccessible lesions. As the preferred treatment of multiple brain metastases according tothe number of brain lesions, respondents tended to choose radiotherapy with increasing number of lesions.Radiosurgery was mostly chosen for the brain metastases of less than or equal to 4. In the surveyabout PCNSL, a half of respondents choose high-dose methotrexate-based polychemotherapy asthe first-line induction therapy for PCNSL. The consolidation and salvage therapy showed a little variationamong respondents. For PCNSL patients with cerebrospinal fluid dissemination, intrathecal chemotherapywas most preferred. @*Conclusion@#: The survey demonstrates the prevailing clinical practice patterns for patients withbrain metastasis and PCNSL among members of the KSNO. This information provides a point of referencefor establishing a practical guideline in the management of brain metastasis and PCNSL.
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PURPOSE: This study analyzed the outcomes of patients with resected extrahepatic bile duct cancer (EHBDC) in order to clarify the role of adjuvant treatments in these patients. MATERIALS AND METHODS: A total of 336 patients with EHBDC who underwent curative resection between 2001 and 2010 were analyzed retrospectively. The treatment types were as follows: surgery alone (n=168), surgery with chemotherapy (CTx, n=90), surgery with radiotherapy (RT) alone (n=29), and surgery with chemoradiotherapy (CRT, n=49). RESULTS: The median follow-up period was 63 months. The 5-year rates of locoregional failure-free survival (LRFFS), distant metastasis-free survival (DMFS), progression-free survival (PFS), and overall survival (OS) for all patients were 56.5%, 59.7%, 36.6%, and 42.0%, respectively. In multivariate analysis, surgery with RT and CRT was a significant prognostic factor for LRFFS, and surgery with CTx was a significant prognostic factor for DMFS, and surgery with CTx, RT, and CRT was a significant prognostic factor for PFS (p < 0.05). Surgery with CTx and CRT showed association with superior OS (p < 0.05), and surgery with RT had marginal significance (p=0.078). In multivariate analysis of the R1 resection patients, surgery with CRT showed significant association with OS (p < 0.05). CONCLUSION: Adjuvant RT and CTx may be helpful in improving clinical outcomes of patients with resected EHBDC who have a high risk of disease recurrence, particularly R1 resection patients. Conduct of additional prospective, larger-scale studies will be required in order to confirm the benefit of adjuvant RT and CTx in these patients.
Subject(s)
Humans , Bile Ducts, Extrahepatic , Biliary Tract Neoplasms , Chemoradiotherapy , Cholangiocarcinoma , Disease-Free Survival , Drug Therapy , Follow-Up Studies , Multivariate Analysis , Prospective Studies , Radiotherapy , Radiotherapy, Adjuvant , Recurrence , Retrospective Studies , Treatment OutcomeABSTRACT
PURPOSE: The aim of this study was to evaluate the role of radiotherapy (RT) in the management of Ewing sarcoma family tumors (ESFT). MATERIALS AND METHODS: Retrospective analysiswas performed on 91 patientswith localized ESFT treated from 1988 to 2012. Primary tumor size was > or = 8 cm in 33 patients. Surgery, RT, and combined surgery with RT were applied in 37, 15, and 33 patients, respectively. RESULTS: Median follow-up was 43.8 months. Forty-three patients (47.3%) showed recurrence or progressive disease. Twelve patients (13.2%) showed local failure after initial treatment. Thirty-nine patients (42.9%) experienced distant metastases. The 5-year overall survival (OS), progression-free survival, and local control (LC) were 60.5%, 58.2%, and 85.1%, respectively. According to treatment, 5-year LCwas 64.8% with RT and 90.2% with combined surgery and RT (p=0.052). Prognostic factors for OS were tumor size (> or = 8 cm, p or = 8 cm), combined surgery and RT produced better LC compared to RT (p=0.033). However, in smaller tumors (< 8 cm), RT without surgery resulted in a similar LC rate as RT with surgery (p=0.374). CONCLUSION: RT used for patients with unfavorable risk factors resulted in worse outcome than for patientswho received surgery. Smallertumors could be controlled locallywith chemotherapy and RT. For large tumors, combined surgery and RT is needed. Proper selection of local treatment modality, RT, surgery, or both is crucial in the management of ESFT.
Subject(s)
Humans , Combined Modality Therapy , Disease-Free Survival , Drug Therapy , Follow-Up Studies , Neoplasm Metastasis , Radiotherapy , Recurrence , Retrospective Studies , Risk Factors , Sarcoma, Ewing , Tumor BurdenABSTRACT
PURPOSE: To evaluate the results of postoperative radiotherapy in patients with extra-hepatic bile duct cancer (EHBDC) and identify the prognostic factors for local control and survival. MATERIALS AND METHODS: Between January 2001 and December 2010, we retrospectively reviewed the cases of 70 patients with EHBDC who had undergone curative resection and received postoperative radiotherapy. The median radiation dose was 50.4 Gy (range, 41.4 to 54 Gy). The resection margin status was R0 in 30 patients (42.9%), R1 in 25 patients (35.7%), and R2 in 15 patients (21.4%). RESULTS: The 5-year rates of overall survival (OS), event-free survival (EFS), and locoregional control (LRC) for all patients were 42.9%, 38.3%, and 61.2%, respectively. The major pattern of failure was distant relapses (33 patients, 47.1%). A multivariate analysis showed that the postradiotherapy CA19-9 level, radiation dose (> or =50 Gy), R2 resection margins, perineural invasion, and T stage were the significant prognostic factors for OS, EFS, and LRC. OS was not significantly different between the patients receiving R0 and R1 resections, but was significantly lower among those receiving R2 resection (54.6%, 56.1%, and 7.1% for R0, R1, and R2 resections, respectively). CONCLUSION: In patients with EHBDC who had undergone curative resection, a postoperative radiotherapy dose less than 50 Gy was suboptimal for OS and LRC. Higher radiation doses may be needed to obtain better LRC. Further investigation of novel therapy or palliative treatment should be considered for patients receiving R2 resection.