Bing-Neel Syndrome: A Case Report and Systematic Review of Clinical Manifestations,Diagnosis, and Treatment Options

BackgroundBing-Neel syndrome is an extremely rare neurologic complication of Waldenström macroglobulinemia (WM) that was first described in 1936. It is associated with central nervous system infiltration by neoplastic lymphoplasmacytoid and plasma cells with or without cerebrospinal fluid (CSF) hyperglobulinemia.Case ReportWe report a case of a 69-year-old white man with a 10-year history of WM. He was diagnosed with Bing-Neel syndrome based on magnetic resonance imaging and pathology studies of CSF. In addition, a comprehensive review of the reported cases of Bing-Neel syndrome in the up-to-date English-language literature was performed.ResultsOur patient underwent successful treatment with cranial radiation and intrathecal chemotherapy. He has been in clinical and pathologic remission for 3 years following the completion of his treatment. Based on our literature review, we also summarize and discuss clinical manifestations, diagnosis, and treatment options for Bing-Neel syndrome.ConclusionBing-Neel syndrome is a rare and potentially treatable complication of WM. Patients with a history of WM presenting with neurologic symptoms should be evaluated for possible Bing-Neel syndrome. Cranial radiation therapy alone or in combination with intrathecal chemotherapy is more likely to achieve sustainable remission than intrathecal chemotherapy alone.     

胃肠间质瘤诊断和治疗进展

近年来,随着新研究证据的出现,胃肠间质瘤(GISTs)领域的国内外指南随之作出更新,从GISTs诊断、生物学行为、手术治疗到靶向药物治疗,几乎涵盖了GISTs管理的每个环节。尤其自2020年以来,美国国立综合癌症网络(NCCN)首次将GISTs相关内容从《软组织肉瘤临床实践指南》中独立出来形成2021 V.1版本,中国临床肿瘤学会(CSCO)也将往年的《中国胃肠道间质瘤诊断治疗专家共识》升级为《胃肠间质瘤诊疗指南》2020版和2021版,由此开启了循证医学证据指导下更加规范、精准的GISTs诊疗新模式。新型靶向药物阿伐替尼和瑞派替尼的上市,有望摆脱转移性GISTs的耐药治疗困境,充实后线治疗阵营,为外科干预提供更多机会,进而为晚期GISTs患者带来生存获益。     

急性肿瘤溶解综合征诊断与治疗

急性肿瘤溶解综合征(Acute tumor lysis syn-drome ATLS)是肿瘤治疗过程中出现的一种具有潜在性的致命的严重并发症.发病率为1.1%～6%[1,2],死亡率有时高达36%[3],尽管采取一些预防和治疗措施,仍有25%的患者在化疗期间发生急性肾功能衰竭[4].急性肿瘤溶解综合征虽然是一种危险的肿瘤急症,但可以逆转,关键是积极预防、早期诊断和有效的治疗,特别是对化疗患者予以足够的重视[5].     

The 5q- Syndrome: a Scientific and Clinical Update

The 5q- syndrome is a subset of the myelodysplastic syndromes characterized by hypolob-ulated micromegakaryocytic hyperplasia and an interstitial deletion of the long arm of chromosome 5. In this concise review, we discuss the current understanding in regard to the genetic basis of the disorder and provide an updated clinical report on 43 patients with the disease who were seen at our institution.     

Chronic Lymphocytic Leukemia: An Update on Biology and Treatment

Combination chemoimmunotherapy with fludarabine, cyclophosphamide, and rituximab (FCR) has emerged as the current standard of care in the treatment of chronic lymphocytic leukemia (CLL). Despite very high response rates, this treatment is too toxic for many patients, and it remains unclear as how to manage patients who do not respond to these agents or who relapse early after treatment. An increase in our understanding of the biology of CLL has led to the development of a wide range of therapies aimed at specific defects in this disease. B-cell receptor signaling is aberrantly increased in CLL, and so many of these drugs target key steps in these pathways. Antitumor immunity is also impaired, and a number of strategies are being developed to repair this acquired immune dysfunction. This review highlights some of the emerging agents and describes the biological rationale for their use in CLL.     

Malignant Pleural Mesothelioma: Medical Treatment Update

Malignant pleural mesothelioma (MPM) is a disease usually unaffected by current therapeutic strategies, but for the majority of patients, the use of systemic chemotherapeutic drugs remains the only therapeutic option available. During the past 15-20 years, many phase II and a few phase III clinical trials have studied a large variety of drugs such as anthracyclines, alkylating agents, platinum compounds, taxanes, vinka alkaloids, and antifolates as single agents and in combination, with the aim to increase responses and survival. The combination of pemetrexed and cisplatin tested in the largest phase III randomized trial of malignant pleural mesothelioma ever conducted has become the current standard of care. New targeted therapeutic approaches with a variety of anti–growth factor drugs are currently undergoing investigation worldwide.     

Update on the Treatment of Anaplastic Large Cell Lymphoma

Purpose of Review

Given the rarity of anaplastic large cell lymphoma (ALCL), studies evaluating new therapies have typically grouped ALCL together with other peripheral T cell lymphomas (PTCL). Thus, the treatment paradigm for ALCL largely mirrors that of PTCL in general. In this review, we discuss the current standard of care as well as emerging therapies, including antibody-based drugs, in systemic ALCL as well as primary cutaneous and breast implant-associated ALCL.

Recent Findings

High CD30 expression in ALCL has allowed the use of brentuximab vedotin, an anti-CD30 antibody-drug conjugate, in both systemic and primary cutaneous ALCL. Recent clinical trials with brentuximab have shown substantial activity in the relapsed/refractory setting. A randomized phase III study is ongoing comparing brentuximab plus CHP (cyclophosphamide, doxorubicin, prednisone) with standard CHOP in the front-line setting.

Summary

The use of targeted therapies and other novel agents have improved outcomes for ALCL patients and in the future can complement or even replace the current standard of care and front-line treatment options.

     

甲状腺微小癌的早期诊断和治疗(附34例临床分析)

目的:探讨B超、FNA、术中冰冻切片在甲状腺微小癌TMC的早期诊断和治疗中的意义.方法:分析1992年～1998年经手术和病理证实的甲状腺微小癌34例的临床资料.结果:术前拟诊32例,B超诊断阳性率94.4%(32/34),FNA诊断阳性率81.1%,冰冻切片诊断阳性率90%,术后病理检查确诊微小癌34例,其中术中冰冻切片未发现3例,4例作患侧甲状腺叶加对侧甲状腺次全切除和双侧甲状腺次全切除术,28例行患侧甲状腺叶切除术:2例患侧甲状腺叶切除加改良性颈淋巴结清扫术,随访率88%(30/34),随访时间5～10年;1例对侧复发,无一例死亡.结论:B超结合FNA、术中冰冻检查能提高甲状腺微小癌的临床诊断率,有临床价值.甲状腺叶或次全切除治疗微小癌具有较好的疗效.     

Update on Anti-angiogenic Treatment for Malignant Gliomas

Malignant gliomas are the most common primary brain tumor found in adults. Unfortunately, the prognosis for these type of tumors remains dismal despite aggressive treatment with surgical resection, radiation and chemotherapy. Therefore, therapeutics aimed at disrupting the angiogenesis of these tumors is being utilized in to improve survival outcomes and quality of life. This paper reviews the history of antiangiogenic agents in malignant gliomas, discusses the FDA approval of bevacizumab as monotherapy in recurrent glioblastoma and the subsequent controversy, and analyzes the most recent newly diagnosed trials of RTOG 0825 and AVAglio. Additionally, the results of the latest trials with antiangiogenic agents and possible biomarkers are reviewed. Multiple questions remain regarding the potential benefit of antiangiogenic treatments in patients with glioblastoma. Future clinical trials should be designed to learn more about these drugs, to optimize their future use.