Pub Date : 2024-12-31Epub Date: 2024-07-23DOI: 10.1080/20450907.2024.2375960
Jackson Griffith-Linsley, William Robert Bell, Aaron Cohen-Gadol, Diane Donegan, Angela Richardson, Michael Robertson, Kevin Shiue, Kathryn Nevel
Aim: Atypical teratoid rhabdoid tumor (ATRT) is a rare and highly aggressive primary CNS neoplasm, predominantly observed in children. The use of autologous stem cell transplantation (ASCT) in pediatric ATRT has shown promise; however, its utility in adult ATRT remains unclear. Patients & methods: This study presents the case of an adult patient with ATRT who is in remission after ASCT and reviews the literature on ASCT in adults with ATRT. Four cases of ATRT in adults who underwent ASCT were identified, with pertinent data summarized. Results: All five patients survived longer than the historical average survival rate, four of whom had no clinical or radiographic evidence of disease at the final follow-up. Conclusion: Based on limited data, there may be a role for ASCT in the treatment of adults with ATRT.
{"title":"Autologous stem cell transplantation in adults with atypical teratoid rhabdoid tumor: a case report and review.","authors":"Jackson Griffith-Linsley, William Robert Bell, Aaron Cohen-Gadol, Diane Donegan, Angela Richardson, Michael Robertson, Kevin Shiue, Kathryn Nevel","doi":"10.1080/20450907.2024.2375960","DOIUrl":"10.1080/20450907.2024.2375960","url":null,"abstract":"<p><p><b>Aim:</b> Atypical teratoid rhabdoid tumor (ATRT) is a rare and highly aggressive primary CNS neoplasm, predominantly observed in children. The use of autologous stem cell transplantation (ASCT) in pediatric ATRT has shown promise; however, its utility in adult ATRT remains unclear. <b>Patients & methods:</b> This study presents the case of an adult patient with ATRT who is in remission after ASCT and reviews the literature on ASCT in adults with ATRT. Four cases of ATRT in adults who underwent ASCT were identified, with pertinent data summarized. <b>Results:</b> All five patients survived longer than the historical average survival rate, four of whom had no clinical or radiographic evidence of disease at the final follow-up. <b>Conclusion:</b> Based on limited data, there may be a role for ASCT in the treatment of adults with ATRT.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11285279/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141747640","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-12-31Epub Date: 2024-06-14DOI: 10.1080/20450907.2024.2357532
Vincenzo Di Nunno, Marta Aprile, Lidia Gatto, Alicia Tosoni, Lucia Ranieri, Stefania Bartolini, Enrico Franceschi
Aim: Glioneuronal and neuronal tumors are rare primary central nervous system malignancies with heterogeneous features. Due to the rarity of these malignancies diagnosis and treatment remains a clinical challenge. Methods: Here we performed a narrative review aimed to investigate the principal issues concerning the diagnosis, pathology, and clinical management of glioneuronal tumors. Results: Diagnostic criteria have been recently overturned thanks to a better characterization on a histological and molecular biology level. The study of genomic alterations occurring within these tumors has allowed us to identify potential therapeutic targets including BRAF, FGFR, and PDGFRA. Conclusion: Techniques allowing molecular sequencing DNA methylation assessment of the disease are essential diagnostic tools. Targeting agents should be included in the therapeutic armamentarium after loco-regional treatment failure.
目的:神经胶质细胞瘤和神经元肿瘤是罕见的原发性中枢神经系统恶性肿瘤,具有不同的特征。由于这些恶性肿瘤的罕见性,其诊断和治疗仍是一项临床挑战。方法:在此,我们进行了一项叙述性综述,旨在研究有关神经胶质细胞瘤的诊断、病理和临床治疗的主要问题。结果由于组织学和分子生物学层面的特征得到了更好的描述,诊断标准最近已被推翻。对这些肿瘤内发生的基因组改变的研究使我们能够确定潜在的治疗靶点,包括BRAF、FGFR和PDGFRA。结论对疾病进行分子测序 DNA 甲基化评估的技术是必不可少的诊断工具。在局部区域治疗失败后,应将靶向药物纳入治疗范围。
{"title":"Novel insights toward diagnosis and treatment of glioneuronal and neuronal tumors in young adults.","authors":"Vincenzo Di Nunno, Marta Aprile, Lidia Gatto, Alicia Tosoni, Lucia Ranieri, Stefania Bartolini, Enrico Franceschi","doi":"10.1080/20450907.2024.2357532","DOIUrl":"10.1080/20450907.2024.2357532","url":null,"abstract":"<p><p><b>Aim:</b> Glioneuronal and neuronal tumors are rare primary central nervous system malignancies with heterogeneous features. Due to the rarity of these malignancies diagnosis and treatment remains a clinical challenge. <b>Methods:</b> Here we performed a narrative review aimed to investigate the principal issues concerning the diagnosis, pathology, and clinical management of glioneuronal tumors. <b>Results:</b> Diagnostic criteria have been recently overturned thanks to a better characterization on a histological and molecular biology level. The study of genomic alterations occurring within these tumors has allowed us to identify potential therapeutic targets including BRAF, FGFR, and PDGFRA. <b>Conclusion:</b> Techniques allowing molecular sequencing DNA methylation assessment of the disease are essential diagnostic tools. Targeting agents should be included in the therapeutic armamentarium after loco-regional treatment failure.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11181933/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141316900","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-06Epub Date: 2024-05-09DOI: 10.1080/20450907.2024.2345579
Steven Du, Dan Beverly Fu, Daniela A Bota, Xiao-Tang Kong
Background: Treatment for refractory or relapsed primary CNS lymphoma (r/r PCNSL) is challenging. Salvage whole-brain radiation therapy (WBRT) is an option but has a short duration of disease control, so additional treatment modalities are warranted. Case: A 75-year-old female with r/r PCNSL who had multiple progressions after multiple lines of treatment underwent salvage WBRT. The patient received ibrutinib, a Bruton's tyrosine kinase inhibitor, as maintenance therapy for 18 months following WBRT with the intention of increasing survival duration after salvage WBRT. She survived 81 months from diagnosis, including 57 months after completion of WBRT. Conclusion: This case presentation describes the experience of using ibrutinib as maintenance therapy in treating r/r PCNSL after salvage WBRT.
{"title":"Prolonged remission with ibrutinib maintenance therapy following radiation in a patient with relapsed primary CNS lymphoma.","authors":"Steven Du, Dan Beverly Fu, Daniela A Bota, Xiao-Tang Kong","doi":"10.1080/20450907.2024.2345579","DOIUrl":"10.1080/20450907.2024.2345579","url":null,"abstract":"<p><p><b>Background:</b> Treatment for refractory or relapsed primary CNS lymphoma (r/r PCNSL) is challenging. Salvage whole-brain radiation therapy (WBRT) is an option but has a short duration of disease control, so additional treatment modalities are warranted. <b>Case:</b> A 75-year-old female with r/r PCNSL who had multiple progressions after multiple lines of treatment underwent salvage WBRT. The patient received ibrutinib, a Bruton's tyrosine kinase inhibitor, as maintenance therapy for 18 months following WBRT with the intention of increasing survival duration after salvage WBRT. She survived 81 months from diagnosis, including 57 months after completion of WBRT. <b>Conclusion:</b> This case presentation describes the experience of using ibrutinib as maintenance therapy in treating r/r PCNSL after salvage WBRT.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11137789/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140897590","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-06Epub Date: 2024-05-28DOI: 10.1080/20450907.2024.2352414
Keng Lam, Cheuk Hong Leung, Zaid Soomro, Chetna Wathoo, Shiao-Pei Weathers
Aim: To evaluate the neuro-oncology providers' experience with patient online access to electronic records. Methods: Cross-sectional survey for physicians and advanced care providers within the field of neuro-oncology in the USA. Results: 65 providers completed the survey, from all major regions of the USA. 58% reported that at least once per month, patients contacted them outside of an office visit about provider notes or a laboratory or imaging finding accessed online. 54% of providers did not think that all laboratory results should be released automatically, and only 25% of providers thought that all radiology reads should be released immediately. 97% thought that some patients suffered substantial distress viewing test results prior to appointments. Qualitative responses aligned with the quantitative results. Conclusion: Most neuro-oncology providers are concerned about the immediate release of laboratory and imaging findings to patients without guidance.
{"title":"Perspectives from neuro-oncology providers on patient access to electronic records: a survey study.","authors":"Keng Lam, Cheuk Hong Leung, Zaid Soomro, Chetna Wathoo, Shiao-Pei Weathers","doi":"10.1080/20450907.2024.2352414","DOIUrl":"10.1080/20450907.2024.2352414","url":null,"abstract":"<p><p><b>Aim:</b> To evaluate the neuro-oncology providers' experience with patient online access to electronic records. <b>Methods:</b> Cross-sectional survey for physicians and advanced care providers within the field of neuro-oncology in the USA. <b>Results:</b> 65 providers completed the survey, from all major regions of the USA. 58% reported that at least once per month, patients contacted them outside of an office visit about provider notes or a laboratory or imaging finding accessed online. 54% of providers did not think that all laboratory results should be released automatically, and only 25% of providers thought that all radiology reads should be released immediately. 97% thought that some patients suffered substantial distress viewing test results prior to appointments. Qualitative responses aligned with the quantitative results. <b>Conclusion:</b> Most neuro-oncology providers are concerned about the immediate release of laboratory and imaging findings to patients without guidance.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11152585/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141310228","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Primary effusion lymphoma (PEL) is an uncommon B-cell lymphoma associated with human herpesvirus 8 and comprises 3-4% of all HIV-related lymphomas. It traditionally presents as a pleural, pericardial, and/or peritoneal effusion, though it can occasionally manifest as an extracavitary or solid mass in the absence of an effusion. The extracavitary or solid variant of primary effusion lymphoma has been reported in the skin, gastrointestinal tract, lung, and lymph nodes. However, very few cases have been reported in the central nervous system. We describe a case of extracavitary or solid variant of primary effusion lymphoma presenting as a brain mass in an HIV-positive man, highlighting the clinicopathologic and immunophenotypic findings of a rare entity.
原发性渗出淋巴瘤(PEL)是一种不常见的与人类疱疹病毒 8 相关的 B 细胞淋巴瘤,占所有 HIV 相关淋巴瘤的 3-4%。它传统上表现为胸膜、心包和/或腹腔积液,但偶尔也会表现为无积液的腔外或实性肿块。据报道,原发性渗出淋巴瘤的腔外或实性变异表现在皮肤、胃肠道、肺部和淋巴结。然而,中枢神经系统淋巴瘤的病例却鲜有报道。我们描述了一例表现为脑部肿块的原发性腔外或实性变异性渗出淋巴瘤(HIV 阳性男性),重点介绍了这一罕见病例的临床病理和免疫分型结果。
{"title":"Extracavitary primary effusion lymphoma presenting as a solitary brain mass.","authors":"Tiffany Javadi, Bryan Morales, Jeffery J Olson, Shawn Kothari, Linsheng Zhang, Malak Abedalthagafi","doi":"10.1080/20450907.2024.2357535","DOIUrl":"10.1080/20450907.2024.2357535","url":null,"abstract":"<p><p>Primary effusion lymphoma (PEL) is an uncommon B-cell lymphoma associated with human herpesvirus 8 and comprises 3-4% of all HIV-related lymphomas. It traditionally presents as a pleural, pericardial, and/or peritoneal effusion, though it can occasionally manifest as an extracavitary or solid mass in the absence of an effusion. The extracavitary or solid variant of primary effusion lymphoma has been reported in the skin, gastrointestinal tract, lung, and lymph nodes. However, very few cases have been reported in the central nervous system. We describe a case of extracavitary or solid variant of primary effusion lymphoma presenting as a brain mass in an HIV-positive man, highlighting the clinicopathologic and immunophenotypic findings of a rare entity.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11172242/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141305639","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-06Epub Date: 2024-03-08DOI: 10.2217/cns-2023-0029
Edina Komlodi-Pasztor, Vasu Munjapara, Charles G Eberhart, Danielle A Bazer, Mohamed R Sherief, David Olayinka Kamson, Xiaobu Ye, Byram Hirsch Ozer, Matthias Holdhoff
Aim: Adult medulloblastomas (MB) are rare, and optimal post-craniospinal irradiation (CSI) chemotherapy is not yet defined. We investigated hematological toxicity in patients treated with platinum-etoposide (EP) post-CSI. Methods: Retrospective, single-institution study to determine hematological toxicity in adult MB patients treated with EP (1995-2022). Results: Thirteen patients with a median follow-up of 50 months (range, 10-233) were analyzed. Four discontinued treatment due to toxicity, one after 1, 3 after 3 cycles. Hematological toxicities included grade 3 (5 patients) and grade 4 (6 patients). Two patients experienced post-treatment progression and died 16 and 37 months from diagnosis. Conclusion: Post-CSI EP demonstrates acceptable hematological toxicity in adult MB. However, the small cohort precludes definitive survival outcome conclusions. Prospective studies for comprehensive comparisons with other regimens are needed in this context.
目的:成人髓母细胞瘤(MB)非常罕见,目前尚未确定最佳的颅椎照射(CSI)后化疗方案。我们研究了铂类-依托泊苷(EP)在CSI后治疗患者的血液毒性。研究方法回顾性单机构研究,确定接受 EP 治疗的成年 MB 患者的血液毒性(1995-2022 年)。研究结果对中位随访期为 50 个月(10-233 个月)的 13 例患者进行了分析。四名患者因毒性停止治疗,其中一人在治疗一个周期后停止治疗,三人在治疗三个周期后停止治疗。血液学毒性包括3级(5例)和4级(6例)。两名患者在治疗后病情恶化,分别在确诊后 16 个月和 37 个月死亡。结论在成人 MB 中,CSI 后 EP 显示出可接受的血液学毒性。然而,由于队列较小,无法得出明确的生存结果结论。在这种情况下,需要进行前瞻性研究,以便与其他治疗方案进行全面比较。
{"title":"Postradiation platinum-etoposide in adult medulloblastomas: retrospective analysis of hematological toxicity.","authors":"Edina Komlodi-Pasztor, Vasu Munjapara, Charles G Eberhart, Danielle A Bazer, Mohamed R Sherief, David Olayinka Kamson, Xiaobu Ye, Byram Hirsch Ozer, Matthias Holdhoff","doi":"10.2217/cns-2023-0029","DOIUrl":"10.2217/cns-2023-0029","url":null,"abstract":"<p><p><b>Aim:</b> Adult medulloblastomas (MB) are rare, and optimal post-craniospinal irradiation (CSI) chemotherapy is not yet defined. We investigated hematological toxicity in patients treated with platinum-etoposide (EP) post-CSI. <b>Methods:</b> Retrospective, single-institution study to determine hematological toxicity in adult MB patients treated with EP (1995-2022). <b>Results:</b> Thirteen patients with a median follow-up of 50 months (range, 10-233) were analyzed. Four discontinued treatment due to toxicity, one after 1, 3 after 3 cycles. Hematological toxicities included grade 3 (5 patients) and grade 4 (6 patients). Two patients experienced post-treatment progression and died 16 and 37 months from diagnosis. <b>Conclusion:</b> Post-CSI EP demonstrates acceptable hematological toxicity in adult MB. However, the small cohort precludes definitive survival outcome conclusions. Prospective studies for comprehensive comparisons with other regimens are needed in this context.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11137761/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140058866","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-06-06Epub Date: 2024-06-12DOI: 10.1080/20450907.2024.2351789
Carlen A Yuen, Melike Pekmezci, Silin Bao, Xiao-Tang Kong
Glioblastoma is the most common malignant primary brain tumor. Despite its infiltrative nature, extra-cranial glioblastoma metastases are rare. We present a case of a 63-year-old woman with metastatic glioblastoma in the lungs. Sarcomatous histology, a reported risk factor for disseminated disease, was found. Genomic alterations of TP53 mutation, TERT mutation, PTEN mutation, and +7/-10 were also uncovered. Early evidence suggests these molecular aberrations are common in metastatic glioblastoma. Treatment with third-line lenvatinib resulted in a mixed response. This case contributes to the growing body of evidence for the role of genomic alterations in predictive risk in metastatic glioblastoma. There remains an unmet need for treatment of metastatic glioblastoma.
{"title":"Metastatic glioblastoma to the lungs: a case report and literature review.","authors":"Carlen A Yuen, Melike Pekmezci, Silin Bao, Xiao-Tang Kong","doi":"10.1080/20450907.2024.2351789","DOIUrl":"10.1080/20450907.2024.2351789","url":null,"abstract":"<p><p>Glioblastoma is the most common malignant primary brain tumor. Despite its infiltrative nature, extra-cranial glioblastoma metastases are rare. We present a case of a 63-year-old woman with metastatic glioblastoma in the lungs. Sarcomatous histology, a reported risk factor for disseminated disease, was found. Genomic alterations of <i>TP53</i> mutation, <i>TERT</i> mutation, <i>PTEN</i> mutation, and +7/-10 were also uncovered. Early evidence suggests these molecular aberrations are common in metastatic glioblastoma. Treatment with third-line lenvatinib resulted in a mixed response. This case contributes to the growing body of evidence for the role of genomic alterations in predictive risk in metastatic glioblastoma. There remains an unmet need for treatment of metastatic glioblastoma.</p>","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11172249/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141305640","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2024-05-20DOI: 10.1080/20450907.2024.2347824
Toru Imai, H. Shoji, H. Hirano, Kunihito Matsuguma, Takahito Awatsu, T. Hirose, N. Okita, A. Takashima, Ken Kato
{"title":"BRAF\u0000 V600E-mutant colorectal cancer with CNS metastases treated successfully with encorafenib, binimetinib and cetuximab","authors":"Toru Imai, H. Shoji, H. Hirano, Kunihito Matsuguma, Takahito Awatsu, T. Hirose, N. Okita, A. Takashima, Ken Kato","doi":"10.1080/20450907.2024.2347824","DOIUrl":"https://doi.org/10.1080/20450907.2024.2347824","url":null,"abstract":"","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2024-05-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141118996","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}