sinonasal undifferentiated carcinoma

鼻窦未分化癌
  • 文章类型: Journal Article
    增强现实(AR)是增强图像引导手术的有前途的技术,代表了将精确的虚拟计划与手术室手术操作的计算机辅助执行相结合的完美桥梁。在颅面外科肿瘤学中,AR给外科医生带来了一个数字,解剖结构的三维表示,有助于确定肿瘤边界和最佳手术路径。术中,实时AR引导为外科医生提供准确的空间信息,确保准确的肿瘤切除和关键结构的保存。在本文中,作者回顾了目前在颅面外科中应用AR的证据,专注于真正的外科应用,并将现有文献与他们在AR和导航引导颅面切除术中的经验进行比较,随后分析哪些技术轨迹将代表AR的未来,并为这一革命性技术定义新的应用视角。
    Augmented reality (AR) is a promising technology to enhance image guided surgery and represents the perfect bridge to combine precise virtual planning with computer-aided execution of surgical maneuvers in the operating room. In craniofacial surgical oncology, AR brings to the surgeon\'s sight a digital, three-dimensional representation of the anatomy and helps to identify tumor boundaries and optimal surgical paths. Intraoperatively, real-time AR guidance provides surgeons with accurate spatial information, ensuring accurate tumor resection and preservation of critical structures. In this paper, the authors review current evidence of AR applications in craniofacial surgery, focusing on real surgical applications, and compare existing literature with their experience during an AR and navigation guided craniofacial resection, to subsequently analyze which technological trajectories will represent the future of AR and define new perspectives of application for this revolutionizing technology.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    鼻窦道中最具挑战性的诊断类别之一包括小蓝色圆形细胞肿瘤。这些是恶性肿瘤,显示出许多重叠的组织形态学和免疫组织化学(IHC)发现。有限,这些未完全切除的肿瘤的小活检增加了诊断混乱。
    在一家三级保健机构进行了为期2年(2018年1月至2020年12月)的横断面研究,其中包括70例肿瘤,其中49例为恶性肿瘤。对所有石蜡包埋的块进行苏木精和曙红染色和IHC,然后在需要时进行分子研究。
    在所有案件中,小蓝圆细胞肿瘤构成主要类别,包括20例罕见和有趣的病例,其中包括鼻窦未分化癌(4例),恶性淋巴瘤(2例弥漫性大B细胞淋巴瘤和2例结外自然杀伤/T细胞淋巴瘤),横纹肌肉瘤(2例),嗅神经母细胞瘤(2例),恶性黑色素瘤(2例),浆细胞瘤(2例),非典型尤因肉瘤(EWS)(1例),EWS(1例),睾丸核蛋白(NUT)癌(1例),小细胞神经内分泌癌1例。
    鼻窦肿瘤种类繁多,在存在未分化形态的小圆细胞肿瘤中更是如此。因此,准确的诊断需要临床放射学参数和特殊的辅助技术,如IHC和分子研究,以及早期诊断和治疗的组织病理学,以防止这些肿瘤的显著发病率和死亡率.
    UNASSIGNED: One of the most challenging diagnostic categories in the sinonasal tract includes small-blue-round-cell tumors. These are malignant tumors which show many overlapping histomorphology and immunohistochemistry (IHC) findings. Limited, small biopsy of these not completely excisable tumors adds to the diagnostic confusion.
    UNASSIGNED: A cross-sectional study was done for 2 years (January 2018-December 2020) in a tertiary care institute, which included 70 cases of tumors of which 49 cases were malignant. All paraffin-embedded blocks were subjected to hematoxylin and eosin stain and IHC followed by molecular study wherever needed.
    UNASSIGNED: Of the total cases, small-blue-round-cell tumor constituted the major category comprising 20 rare and interesting cases which included sinonasal undifferentiated carcinoma (4 cases), malignant lymphoma (2 cases of diffuse large B-cell lymphoma and 2 cases of extranodal natural killer/T-cell lymphoma), rhabdomyosarcoma (2 cases), olfactory neuroblastoma (2 cases), malignant melanoma (2 cases), plasmacytoma (2 cases), atypical Ewing\'s sarcoma (EWS) (1 case), EWS (1 case), nuclear protein in testis (NUT) carcinoma (1 case), and small-cell neuroendocrine carcinoma (1 case).
    UNASSIGNED: Tumors of the sinonasal tract are very diverse, more so in small-round-cell tumor which present with a undifferentiated morphology. Thus, accurate diagnosis needs clinicoradiological parameters and special ancillary techniques such as IHC and molecular study in addition to histopathology for early diagnosis and therapy to prevent significant morbidity and mortality caused in these tumors.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    目的:鼻窦未分化癌(SNUC)是一种罕见的,侵袭性鼻窦腔恶性肿瘤,预后不良,治疗选择有限。为了研究SNUC对联合免疫疗法的敏感性,我们使用SNUC细胞系进行了体外研究,并使用多光谱免疫荧光来表征体内患者SNUC肿瘤免疫微环境(TIME)。
    方法:将人源SNUC细胞系用于肿瘤细胞对基于自然杀伤(NK)细胞的免疫治疗策略的敏感性的体外研究。通过多光谱免疫荧光和临床相关性评估检查来自14名治疗初治SNUC患者的肿瘤样品。
    结果:抗PD-L1阻断可增强SNUC细胞系的NK细胞裂解约5.4倍(P≤0.0001)。通过抗体依赖性细胞毒性(ADCC)介导的途径,证明活性的CD16中和抗体阻断了这种作用。通过外源性干扰素-γ(IFN-γ)施用或白介素-15(IL-15)刺激的IFN-γ从NK细胞释放,肿瘤细胞上的PD-L1的上调,进一步增强了SNUC细胞的ADCC依赖性裂解。抗PD-L1阻断和IL-15超激动作用的联合治疗可使NK细胞对SNUC细胞的杀伤增强9.6倍(P≤0.0001)。未处理的SNUC患者肿瘤样品被发现具有NK细胞浸润和PD-L1+肿瘤细胞的中值为5.4个细胞/mm2。在治疗后无疾病复发的患者中,CKlow肿瘤细胞/NK细胞相互作用显着增加了55.7倍(P=0.022)。间质中CD3+CD8+较高的患者5年总生存率显著提高(P=0.0029),长期存活者CKlow肿瘤细胞/CD8+细胞毒性T细胞相互作用显著增加(P=0.0225)。
    结论:这些数据为正在进行的SNUC联合免疫治疗方法研究提供了临床前理论基础。
    OBJECTIVE: Sinonasal undifferentiated carcinoma (SNUC) is a rare, aggressive malignancy of the sinonasal cavity with poor prognosis and limited treatment options. To investigate the potential for SNUC sensitivity to combinatory immunotherapy, we performed in vitro studies with SNUC cell lines and used multi-spectral immunofluorescence to characterize the in vivo patient SNUC tumor immune microenvironment (TIME).
    METHODS: Human-derived SNUC cell lines were used for in vitro studies of tumor cell susceptibility to natural killer (NK) cell-based immunotherapeutic strategies. Tumor samples from 14 treatment naïve SNUC patients were examined via multi-spectral immunofluorescence and clinical correlations assessed.
    RESULTS: Anti-PD-L1 blockade enhanced NK cell lysis of SNUC cell lines ∼5.4 fold (P ≤ 0.0001). This effect was blocked by a CD16 neutralizing antibody demonstrating activity through an antibody-dependent cellular cytotoxicity (ADCC) mediated pathway. ADCC-dependent lysis of SNUC cells was further enhanced by upregulation of PD-L1 on tumor cells by exogenous interferon-gamma (IFN-γ) administration or interleukin-15 (IL-15) stimulated IFN-γ release from NK cells. Combination treatment with anti-PD-L1 blockade and IL-15 superagonism enhanced NK-cell killing of SNUC cells 9.6-fold (P ≤ 0.0001). Untreated SNUC patient tumor samples were found to have an NK cell infiltrate and PD-L1+ tumor cells at a median of 5.4 cells per mm2. A striking 55.7-fold increase in CKlow tumor cell/NK cell interactions was observed in patients without disease recurrence after treatment (P = 0.022). Patients with higher CD3+CD8+ in the stroma had a significantly improved 5-year overall survival (P = 0.0029) and a significant increase in CKlow tumor cell/CD8+ cytotoxic T cell interactions was noted in long-term survivors (P = 0.0225).
    CONCLUSIONS: These data provide the pre-clinical rationale for ongoing investigation into combinatory immunotherapy approaches for SNUC.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    鼻窦未分化癌(SNUC)是一种罕见的肿瘤,具有侵袭性,与鼻窦癌相比,临床病程不同,预后较差。这里,我们报告了首例孤立性额叶SNUC病例,该病例通过前期手术治疗,然后进行辅助治疗.我们想强调在这种高度侵袭性的肿瘤中,早期表现和及时以保守手术形式进行干预的重要性。
    Sinonasal undifferentiated carcinoma (SNUC) is a rare tumor which is aggressive in nature, has a different clinical course in contrast to sinonasal carcinoma and poor prognosis. Here, we are reporting first case of isolated frontal SNUC which was managed by upfront surgery followed by adjuvant treatment. We want to emphasize the importance of early presentation and timely intervention in form of conservative surgery in this highly aggressive tumor.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    鼻窦未分化癌(SNUC)是一种极其罕见且高度侵袭性的鼻腔和/或鼻旁窦恶性肿瘤。SNUC在临床病理上与其他肿瘤不同,但由于其低发病率而难以研究。关于SNUC的病因也很少有共识,包括其与爱泼斯坦-巴尔病毒(EBV)的关联。治疗方式包括手术,化疗,和辐射取决于阶段和等级。在这里,我们讨论了一个病人,他出现了慢性鼻漏和各种眼科症状,如闪光,漂浮物,和复视。除了先前的膀胱癌病史外,该患者后来在EBV血清学检测阴性的情况下被诊断为SNUC。本病例报告的目的是为SNUC的更广泛的文献和围绕所使用的诊断方式的细节做出贡献,管理,非典型症状患者的非EBV鼻窦未分化癌的预后。
    Sinonasal undifferentiated carcinoma (SNUC) is an extremely rare and highly aggressive malignant neoplasm of the nasal cavity and/or paranasal sinuses. SNUC is clinicopathologically distinctive from other tumors but is difficult to study due to its low incidence. There is also very little consensus about the etiology of SNUC, including its association with Epstein-Barr virus (EBV). Treatment modalities include surgery, chemotherapy, and radiation depending on the stage and grading. Herein, we discuss a patient who presented to the emergency department with chronic rhinorrhea and various ophthalmologic symptoms such as flashes, floaters, and diplopia. The patient was later diagnosed with SNUC in the setting of negative serological testing for EBV in addition to his previously concomitant history of bladder cancer. The purpose of this case report is to contribute to the broader literature of SNUC and the specifics surrounding the diagnostic modalities utilized, management, and outcome of non-EBV sinonasal undifferentiated carcinoma in a patient with atypical symptomatology.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    背景:鼻窦未分化癌(SNUC)是一种极为罕见的头颈部恶性肿瘤。对于转移性疾病的治疗尚无共识。
    方法:一名56岁女性在鼻窦充血后被诊断为SNUC,复视,和右眼眶疼痛。最初接受手术和放射治疗,她后来发展为严重的转移性疾病。在pembrolizumab治疗下,她的肝转移进展。然而,加用ipilimumab和COX-2抑制剂可显著改善患者的病变,并持续缓解.她的治疗方案因使用类固醇治疗成功的免疫相关不良事件而复杂化。
    结论:治疗转移性SNUC时,双重检查点抑制值得考虑,尤其是单药治疗失败后。这种治疗的积极作用可以通过IDO1抑制来增强。
    Sinonasal undifferentiated carcinoma (SNUC) is an exceedingly rare head and neck malignancy. No consensus exists on treatment for metastatic disease.
    A 56-year-old female was diagnosed with SNUC after endorsing sinus congestion, diplopia, and right orbital pain. Initially treated with surgery and radiation, she later developed significant metastatic disease. She demonstrated progression of her hepatic metastases under pembrolizumab therapy. However, the addition of ipilimumab and a COX-2 inhibitor resulted in significant improvement in her lesions as well as an ongoing durable response. Her regimen was complicated by immune-related adverse events successfully treated with steroids.
    Dual checkpoint inhibition deserves consideration when treating metastatic SNUC, especially after single agent therapy has failed. The positive effect of this treatment may be augmented by IDO1 inhibition.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Systematic Review
    目的:鼻窦未分化癌(SNUC)是一种罕见但侵袭性肿瘤,预后极差。目前没有公认的临床试验来指导治疗,各种治疗方式对生存的影响也没有很好的定义。我们的目标是提供关于生存结果的当前治疗方式的最新系统评价。
    方法:提取个体患者数据,和生存数据汇集在一个阶段的荟萃分析中。使用Kaplan-Meier方法分析描述性统计。按治疗方式分层的患者水平比较,根据人口统计进行调整,使用共享脆弱Cox回归进行。
    方法:参与者包括根据组织学证据诊断为SNUC的所有患者。我们观察了不同治疗方式的总体累积生存结果,以及低阶段和高阶段SNUC患者的治疗方式的总体生存。
    结论:确定了17项研究,包括1993年至2020年的208名患者。低阶段患者与高阶段患者的累积总生存率没有显着差异,不同治疗方式的结局无显著差异。在95个月时,SNUC的总累积生存率为30%。在接受各种治疗方式组合治疗的患者中,接受放化疗的患者在40个月时的累积生存率最高,为42%.明确放化疗与提高疾病生存率相关。不管肿瘤分期,患者应及早积极治疗,一种治疗方案没有另一种治疗方案的优越性。与双模治疗相比,三模治疗并不具有生存优势。
    OBJECTIVE: Sinonasal undifferentiated carcinoma (SNUC) is a rare but aggressive tumour with very poor prognosis. There are currently no well-established clinical trials to guide therapy and the impact of various treatment modalities on survival is not well defined. We aim to provide an updated systematic review on current treatment modalities on survival outcomes.
    METHODS: Individual patient data were extracted, and survival data pooled in a one-stage meta-analysis. Descriptive statistics were analysed using the Kaplan-Meier method. Patient-level comparisons stratified by treatment modalities, adjusted for demographics, were conducted using shared-frailty Cox regression.
    METHODS: Participants include all patients diagnosed with SNUC based on histological evidence. We looked at the overall cumulative survival outcome for different treatment modalities and overall survival by treatment modality in low versus high stage SNUC patients.
    CONCLUSIONS: Seventeen studies were identified, comprising 208 patients from 1993 to 2020. There was no significant difference in cumulative overall survival in low versus high stage patients, and no significant difference in outcomes by treatment modality. The overall cumulative survival of SNUC is 30% at 95 months. Among patients treated with various combinations of treatment modalities, patients with chemoradiotherapy had the highest cumulative survival of 42% at 40 months. Definitive chemoradiotherapy was associated with improved disease survival rate. Regardless of tumour stage, patients should be treated early and aggressively, with no superiority of one treatment regimen over another. Trimodality treatment does not confer survival advantage over bimodality treatment.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    背景:鼻窦肿瘤,无论是良性还是恶性,对临床医生构成了重大挑战,并代表了多学科合作的典范领域,以优化患者护理。关于过敏和鼻窦肿瘤的国际共识声明(ICSNT)旨在总结现有的最佳证据,并提出48个主题和组织病理学主题。
    方法:根据以前的ICAR文件,ICSNT将每个主题分配为带有建议的循证审查,循证审查,和基于证据水平的文献综述。使用系统评论和荟萃分析格式的首选报告项目,组建了一个多学科作者团队的国际小组进行主题评论。完成的部分经历了一个彻底和迭代的建立共识过程。最终文件在出版之前经过了严格的综合和审查。
    结果:ICNST文件包括4个主要部分:一般原则,良性肿瘤和病变,恶性肿瘤,以及生活质量和监测。它涵盖了48个与鼻窦肿瘤和肿块相关的概念和/或组织病理学主题。具有高水平证据的主题提供了具体建议,而其他领域则总结了目前的证据状况。最后一节强调研究机会和未来方向,促进知识和社区干预。
    结论:作为鼻腔鼻窦肿瘤和肿块的多学科和协作护理模式的体现,ICSNT被设计为一个全面的,国际,和多学科协作努力。其主要目的是总结鼻窦肿瘤和肿块领域的现有证据。本文受版权保护。保留所有权利。
    BACKGROUND: Sinonasal neoplasms, whether benign and malignant, pose a significant challenge to clinicians and represent a model area for multidisciplinary collaboration in order to optimize patient care. The International Consensus Statement on Allergy and Rhinology: Sinonasal Tumors (ICSNT) aims to summarize the best available evidence and presents 48 thematic and histopathology-based topics spanning the field.
    METHODS: In accordance with prior International Consensus Statement on Allergy and Rhinology documents, ICSNT assigned each topic as an Evidence-Based Review with Recommendations, Evidence-Based Review, and Literature Review based on the level of evidence. An international group of multidisciplinary author teams were assembled for the topic reviews using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses format, and completed sections underwent a thorough and iterative consensus-building process. The final document underwent rigorous synthesis and review prior to publication.
    RESULTS: The ICSNT document consists of four major sections: general principles, benign neoplasms and lesions, malignant neoplasms, and quality of life and surveillance. It covers 48 conceptual and/or histopathology-based topics relevant to sinonasal neoplasms and masses. Topics with a high level of evidence provided specific recommendations, while other areas summarized the current state of evidence. A final section highlights research opportunities and future directions, contributing to advancing knowledge and community intervention.
    CONCLUSIONS: As an embodiment of the multidisciplinary and collaborative model of care in sinonasal neoplasms and masses, ICSNT was designed as a comprehensive, international, and multidisciplinary collaborative endeavor. Its primary objective is to summarize the existing evidence in the field of sinonasal neoplasms and masses.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    目的:本研究的主要目的是了解鼻窦小圆形蓝细胞肿瘤的特征和治疗方法,并强调免疫组织化学在这些患者的诊断和内镜/开放切除术后的结果中的作用。方法:这是一项在印度三级护理转诊中心进行的回顾性研究,该研究包括38例鼻窦患者,为期5年。所有患者均进行了临床和放射学评估。所有病例均在内镜或开放手术切除后通过组织病理学检查和免疫组织化学进行诊断。一些病例接受了术后放疗。结果:在我们的研究中,在176例诊断为鼻中恶性肿瘤的病例中,38例(21.6%)被诊断为鼻腔鼻窦小圆蓝细胞肿瘤,男女比例为1.4:1。出现在我们面前的所有鼻窦小圆蓝色细胞肿瘤中最常见的组织病理学类型是美学神经母细胞瘤,即,8例(21%)患者其次是垂体大腺瘤7例(8.4%)患者。其他类型是未分化鳞状细胞癌10(13.1%),颅咽管瘤8(10.5%),淋巴瘤3(7.9%),滑膜/梭形细胞肉瘤,恶性黑色素瘤和腺癌各1例(2.6%)。神经鞘瘤,横纹肌肉瘤,神经内分泌癌和神经纤维瘤各2例(5.2%)。结论:鼻窦小圆蓝细胞瘤是极其罕见的肿瘤。免疫组化的组织病理学诊断是各种肿瘤的特征,是诊断的决定性因素。对这些肿瘤实体的了解至关重要,因为早期诊断有助于进一步管理,以防止扩散到重要结构并改善预后。大多数肿瘤都有多模式治疗方法,包括手术切除,放疗和化疗。
    Purpose: The main purpose of this study is to understand the characteristics and management of sinonasal small round blue cell tumors and also to emphasise the role of immunohistochemistry in their diagnosis and on the outcomes after endoscopic/open excision in these patients. Methods: This is a retrospective study conducted at a tertiary care referral centre in India which included 38 patients with sino nasal for a period of 5 years. All the patients were evaluated clinically and radiologically. All cases were confirmed diagnostically with histopathological examination and immunohistochemistry following surgical excision either by endoscopic or open approach. Some of the cases underwent post operative radiotherapy. Results: In our study, among 176 cases diagnosed with Sino nasal malignancies, 38 (21.6%) cases were diagnosed with sinonasal small round blue cell tumors with male to female ratio 1.4:1. Most common histopathological type among all the sinonasal small round blue cell tumors that presented to us was esthesioneuroblastoma i.e., 8 (21%) patients followed by pituitary macroadenoma in 7(8.4%) patients. Other types are undifferentiated squamous cell carcinoma 10(13.1%), craniopharyngioma 8(10.5%), lymphoma 3(7.9%), synovial/spindle cell sarcoma, malignant melanoma and adenocarcinoma 1(2.6%) each. Schwannoma, rhabdomyosarcoma, neuroendocrine carcinoma and neurofibroma 2 (5.2%) each. Conclusion: Sinonasal small round blue cell tumors are extremely rare tumours. Histopathological diagnosis with immunohistochemistry is characteristic of various tumors and is conclusive for diagnosis. Knowledge of these tumor entity is essential as early diagnosis helps in further management in preventing spread to vital structures and improving outcome. Most of the tumors have a multimodality treatment approach which includes surgical excision, radiotherapy and chemotherapy.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    新出现的鼻窦癌表现出多种形态和特定的分子重排,以及与常规对应物不同的临床行为。我们的目标是提出一种诊断算法,该算法基于每种鼻窦癌的分子发现,并考虑了新的实体。这种诊断算法应有助于诊断病理学家建立具有挑战性的鼻窦蓝细胞癌的诊断,并帮助研究人员对档案病例进行回顾性分析。除了咨询我们的档案案例,进行文献挖掘以检索有关新出现的实体的免疫组织化学和分子发现。我们提出的算法区分了低分化(非)角化SNSCC,间变性肌上皮癌,NUT中线癌,SMARCB1/SMARCA4缺陷型畸胎癌肉瘤,SMARCB1/SMARCA4缺陷性癌肉瘤,嗅觉神经母细胞瘤,鼻窦未分化癌,HPV相关的多表型鼻腔鼻窦癌和其他腺癌。通过结合形态学特征,免疫组织化学标记,和分子研究,该算法提高了诊断的准确性,特别是在全面的分子检测不容易获得的情况下。该算法是病理学家的宝贵资源,促进鼻窦恶性肿瘤的正确诊断并指导适当的患者管理。
    The newly emerging sinonasal carcinomas have demonstrated diverse morphologies and specific molecular rearrangements along with deviant clinical behavior from conventional counterparts. We aim to propose a diagnostic algorithm that is based on molecular findings of each sinonasal cancer and is considering the new entities has been called upon. Such a diagnostic algorithm should help diagnostic pathologists establish a diagnosis of a challenging sinonasal blue cell carcinomas and researchers performing retrospective analysis of archival cases. Along with consulting our archival cases, literature mining was conducted to retrieve the immunohistochemical and molecular findings regarding the newly emerging entities. Our proposed algorithm distinguishes poorly differentiated (non) keratinizing SNSCC, from anaplastic myoepithelial carcinoma, NUT midline carcinoma, SMARCB1/SMARCA4-deficient teratocarcinosarcoma, SMARCB1/SMARCA4-deficient carcinosarcoma, olfactory neuroblastoma, sinonasal undifferentiated carcinoma, HPV-related multiphenotypic sinonasal carcinoma and other adenocarcinomas. By incorporating morphologic features, immunohistochemical markers, and molecular investigations, the algorithm enhances the accuracy of diagnosis, particularly in cases where comprehensive molecular testing is not readily available. This algorithm serves as a valuable resource for pathologists, facilitating the proper diagnosis of sinonasal malignancies and guiding appropriate patient management.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

公众号