Sinonasal

鼻窦
  • 文章类型: Case Reports
    SMARCB1是一种已知在许多软组织肿瘤中引起癌变的基因,包括恶性横纹肌样瘤和上皮样肉瘤。自从2014年首次报道以SMARCB1基因缺陷为特征的鼻窦癌亚型以来,报告的病例不到200例。我们报告了一例SMARCB1缺陷的鼻窦癌,具有透明细胞形态。在我们的案例中,没有明显的基底细胞样或浆细胞样/横纹肌样瘤细胞,这是SMARCB1缺陷型鼻腔鼻窦癌的典型组织病理学特征。缺乏SMARCB1的鼻窦癌易于复发,预后极差。随着分子靶向药物的发展,治疗效果有望提高。同时,SMARCB1缺陷型鼻窦炎早期准确诊断的重要性将增加.活检标本提供的信息有限,有必要通过免疫组织化学确认SMARCB1表达的缺失,并通过分子遗传学研究SMARCB1基因缺失的存在,考虑到SMARCB1缺陷性鼻腔鼻窦癌的可能性,即使在没有基底细胞样或浆细胞样/横纹肌样细胞形态的非典型病例中,就像我们的情况一样。
    SMARCB1 is a gene known to cause carcinogenesis in many soft tissue tumors, including malignant rhabdoid tumors and epithelioid sarcoma. Since the first report of a subtype of sinonasal carcinoma characterized by a deficiency of the SMARCB1 gene in 2014 to date, fewer than 200 cases have been reported. We report a case of SMARCB1-deficient sinonasal carcinoma with clear cell morphology. In our case, there are no evident basaloid or plasmacytoid/rhabdoid tumor cells, which are typical histopathological features of SMARCB1-deficient sinonasal carcinoma. SMARCB1-deficient sinonasal carcinoma is prone to recurrence and has a very poor prognosis. As the development of molecularly targeted agents progresses, therapeutic efficacy is expected to improve. Simultaneously, the importance of early and accurate diagnosis of SMARCB1-deficient sinonasal carcinoma will increase. With the limited information provided by biopsy specimens, it is necessary to confirm the loss of SMARCB1 expression by immunohistochemistry and investigate the presence of SMARCB1 gene deletion by molecular genetics, considering the possibility of SMARCB1-deficient sinonasal carcinoma even in atypical cases without basaloid or plasmacytoid/rhabdoid cell morphology, as in our case.
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  • 文章类型: Journal Article
    提出了一个14岁的完整雌性美洲驼(Lamaglama),用于评估3个月的右上颌肿胀。临床上,动物有轻微的鼻涕,右眼反常的逆行,和中度牙龈疾病。上颌肿块的切开活检显示,多形性和有丝分裂活性的肿瘤纺锤体至星状细胞组织在嵌入丰富的软骨样基质中的任意腔隙中。鉴于预后不良,安乐死当选。尸检和头部切片暴露了一个大固体,白色,坚定的质量极大地扩大了正确的轨道下区域,延伸到上颌骨,清除右侧鼻甲和同侧颧骨。总的来说,尸检,细胞学,和原发性肿块的组织病理学支持诊断为鼻窦软骨肉瘤。据我们所知,该实体以前从未在该物种中报道过,应被视为新世界骆驼科动物面部畸形的差异。
    A 14-y-old intact female llama (Lama glama) was presented for evaluation of a right maxillary swelling of 3-mo duration. Clinically, the animal had mild nasal discharge, abnormal retropulsion of the right eye, and moderate gingival disease. An incisional biopsy of the maxillary mass revealed pleomorphic and mitotically active neoplastic spindle-to-stellate cells organized in haphazard lacunae embedded in abundant chondroid matrix. Given the poor prognosis, euthanasia was elected. Postmortem examination and sectioning of the head exposed a large solid, white, firm mass that vastly expanded the right infraorbital region, extending to the maxilla, effacing the right nasal conchae and ipsilateral zygomatic bone. Collectively, postmortem dissection, cytology, and histopathology of the primary mass supported a diagnosis of sinonasal chondrosarcoma. To our knowledge, this entity had not been reported previously in this species and should be considered a differential for facial deformities in New World camelids.
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  • 文章类型: Journal Article
    愈创甘油醚,一种广泛用于非处方咳嗽和感冒药的成分,关于其治疗呼吸系统疾病的功效一直是争论的话题。尽管它的历史用途和美国食品和药物管理局的批准,最近的研究质疑其声称的祛痰效果及其在控制症状方面的有效性。这篇文章研究了现有的证据,强调上呼吸道疾病与安慰剂相比缺乏显著的益处。研究了临床使用的基本原理,以及推荐愈创木醚作为治疗选择的潜在缺点和替代品。
    Guaifenesin, a widely used ingredient in over-the-counter cough and cold medications, has been a subject of debate regarding its efficacy in treating respiratory conditions. Despite its historical use and US Food and Drug Administration approval, recent studies have questioned its claimed expectorant effect and its effectiveness in managing symptoms. This article examines the available evidence, highlighting the lack of significant benefits over placebo for upper respiratory disease. The rationale for clinical use is examined alongside the potential downside and alternatives to recommending guaifenesin as a treatment option.
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  • 文章类型: Journal Article
    一个77岁的动脉高血压患者,在我们的诊所进行常规眼科检查的II型糖尿病。他抱怨间歇性复视。眼科检查显示III瘫痪(n。眼动肌)和VI(n.外展)上睑下垂的颅神经,左眼抬高和外展的缺陷。患者接受了脑/眼眶和鼻旁窦的紧急MRI成像,和紧急神经学评估。MRI显示有一个体积占据过程,从左上颌窦的后壁开始,伴有神经周围扩散和同侧三叉神经受累,颅内扩散在内侧颅窝和海绵体受累,左侧的蝶窦和眶尖。进行了活检,组织学导致鼻窦鳞状细胞癌颅内扩散。
    A 77-year-old-man with arterial hypertension, diabetes mellitus type II presented at our clinic for a routine ophthalmological exam. He complained of intermittent double vision. The ophthalmic examination revealed paralysis of III (n. oculomotorius) and VI (n. abducens) cranial nerves with ptosis, deficit in elevation and abduction of the left eye. The patient underwent urgent MRI imaging of the brain/orbits and paranasal sinuses, and urgent neurological assessment. MRI revealed a volume-occupying process, starting from the posterior wall of the left maxillary sinus with perineural diffusion and involvement of the homolateral trigeminal nerve, intracranial spread in the medial cranial fossa and involvement of the cavernous, sphenoidal sinuses and the orbital apex on the left side. Biopsy was performed, and the histology resulted in sinonasal squamous cell carcinoma with intracranial spread.
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  • 文章类型: Journal Article
    目的:介绍一例30多岁女性患者的鼻窦血管外皮细胞瘤(GPC)的病例报告,并强调即使在常规鼻窦手术中也要收集病理标本。
    方法:一个病例报告,详述了一名30多岁的女性的GPC诊断,包括她最初的演讲,治疗,和后续行动,以及对文献的简要回顾。
    结果:收集的标本病理显示鼻窦GPC和慢性鼻-鼻窦炎。免疫组织化学显示SMA阳性,β-catenin,和细胞周期蛋白D1;STAT6、ERG阴性,pankeratin,SOX10和S100。
    结论:该诊断扩展了GPC患者人口统计学特征的知识。鼻窦肿块的鉴别诊断应包括GPC,即使是年轻患者。该病例强调了在所有病例中收集整个病理标本的重要性,甚至那些看起来常规和良性的。
    OBJECTIVE: To present a case report of sinonasal glomangiopericytoma (GPC) in a female patient in her thirties and to highlight the importance of collecting pathology specimens even in routine sinus surgery cases.
    METHODS: A case report detailing the diagnosis of GPC in a female in her thirties, including her initial presentation, treatment, and follow-up, along with a brief review of the literature.
    RESULTS: Pathology of the collected specimen revealed sinonasal GPC along with chronic rhinosinusitis. Immunohistochemistry was positive for SMA, beta-catenin, and cyclin D1; and negative for STAT6, ERG, pankeratin, SOX10, and S100.
    CONCLUSIONS: This diagnosis expands the knowledge around the demographic profile of GPC patients. GPC should be included in the differential diagnosis of sinonasal masses, even in younger patients. The case highlights the importance of collecting the entire pathology specimen in all cases, even of ones that seem routine and benign.
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  • 文章类型: Journal Article
    近几十年来,头颈部肿瘤的分类已经发展,包括分子检测在鼻窦肿瘤中的广泛应用。唾液腺,和头部和颈部的软组织。新分子技术的可用性允许定义头颈部部位特有的多种新型肿瘤类型。此外,遗传改变特异性的免疫组织化学标记物的扩展谱有助于快速鉴定诊断性分子异常.因此,目前,头颈部病理学家可以从分子定义的肿瘤分类中获益,同时做出主要基于组织病理学和免疫组织化学的诊断。这篇综述涵盖了鼻窦恶性肿瘤的主要分子改变。比如DEK的改动,AFF2,NUTM1,IDH1-2,特别是SWI/SNF基因,从诊断的实际角度来看,这很重要,预后,和对治疗反应的预测。
    Classification of tumors of the head and neck has evolved in recent decades including a widespread application of molecular testing in tumors of the sinonasal tract, salivary glands, and soft tissues with a predilection for the head and neck. The availability of new molecular techniques has allowed for the definition of multiple novel tumor types unique to head and neck sites. Moreover, an expanding spectrum of immunohistochemical markers specific to genetic alterations facilitates rapid identification of diagnostic molecular abnormalities. As such, it is currently possible for head and neck pathologists to benefit from a molecularly defined tumor classification while making diagnoses that are still based largely on histopathology and immunohistochemistry. This review covers the principal molecular alterations in sinonasal malignancies, such as alterations in DEK, AFF2, NUTM1, IDH1-2, and SWI/SNF genes in particular, that are important from a practical standpoint for diagnosis, prognosis, and prediction of response to treatment.
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  • 文章类型: Systematic Review
    目的:嗅觉功能障碍(OD)常见于鼻窦功能障碍患者,但青少年囊性纤维化(AwCF)患者嗅觉问题的患病率和严重程度尚不清楚.OD可能导致饮食不足并加剧营养挑战。我们试图回顾有关AwCF鼻窦症状的药物和手术治疗的有效性以及对嗅觉功能的相关影响的文献。
    方法:我们对PubMed进行了系统的文献检索,Embase,WebofScience,和EbscoCINAHL从1980年到2022年根据PRISMA-ScR协议进行范围审查,以努力编制研究设计数据,患者人口统计学,临床特征和结果,还有偏见的风险。
    结果:在368篇摘要中,3篇文章专门评估了总共34名患者的AwCF。两项研究评估了内窥镜鼻窦手术(ESS)和dornasealfa。另外6篇文章纳入了儿童和成人CF混合人群,共313例患者。干预措施包括ESS,elexacaftor-tezacaftor-ivacaftor(ETI),ivacaftor,盐水,dornasealfa,透明质酸,和透明质酸-妥布霉素组合。结果指标包括使用未经验证(4/9)和经过验证(4/9)的调查对OD进行主观评估,和心理物理(1/9)气味测试。评估ESS的研究,FESS,dornasealfa,ivacaftor,高渗和等渗盐水都报告了OD的统计学显着改善,尽管其他生活质量指标有所改善,但ETI未能改善OD。
    结论:关于药物和手术干预对AwCF嗅觉影响的数据有限。嗅觉评估通常仅限于主观和定性的自我报告。我们建议在有饮食挑战和体重管理问题的人群中,通过心理物理测试跟踪嗅觉结果至关重要。
    OBJECTIVE: Olfactory dysfunction (OD) commonly occurs in patients with sinonasal dysfunction, but the prevalence and severity of olfactory issues in adolescents with cystic fibrosis (AwCF) is unclear. OD may contribute to dietary deficiencies and exacerbate nutritional challenges. We sought to review literature on the effectiveness of medical and surgical management of sinonasal symptoms in AwCF and the associated impact on olfactory function.
    METHODS: We performed a systematic literature search of PubMed, Embase, Web of Science, and Ebsco CINAHL from 1980 to 2022 per PRISMA-ScR protocols to conduct a scoping review in an effort to compile data on study design, patient demographics, clinical characteristics and outcomes, along with risk of bias.
    RESULTS: Of 368 abstracts, 3 articles exclusively evaluated AwCF for a total of 34 patients. Two studies evaluated endoscopic sinus surgery (ESS) and dornase alfa. An additional 6 articles were included for mixed pediatric and adult CF populations totaling 313 patients. Interventions included ESS, elexacaftor-tezacaftor-ivacaftor (ETI), ivacaftor, saline, dornase alfa, hyaluronic acid, and hyaluronic acid-tobramycin combination. Outcome measures included subjective assessment of OD using non-validated (4/9) and validated (4/9) surveys, and psychophysical (1/9) smell testing. Studies evaluating ESS, FESS, dornase alfa, ivacaftor, and both hypertonic and isotonic saline reported statistically significant improvement in OD, whereas ETI failed to improve OD despite improvement in other quality of life measures.
    CONCLUSIONS: There is limited data regarding the impact of medical and surgical interventions on olfaction for AwCF. Assessment of olfaction was often limited to subjective and qualitative self-report. We suggest that tracking of olfactory outcomes with psychophysical testing is critical in this population with dietary challenges and weight management issues.
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  • 文章类型: Journal Article
    鼻窦错构瘤,根据世界卫生组织第5版的头颈部肿瘤分类分为呼吸道上皮腺瘤样错构瘤(REAH),浆膜粘质错构瘤和软骨间充质错构瘤。浆膜粘质错构瘤是由纤维基质和立方细胞包围的小嗜酸性腺体的良性增殖。鼻腔和鼻旁窦错构瘤是罕见的实体,临床表现为鼻窦息肉。
    一名79岁女性患者因严重呼吸困难被转诊至急诊室。前鼻镜检查显示单侧灰色息肉样肿块阻塞中部,下鼻道和普通鼻道。观察全身皮质类固醇和氧疗。鼻旁窦的计算机断层扫描成像在所有三个平面上都进行了对比,显示所有鼻道和上颌都有混浊的息肉样肿块,筛前和蝶窦向后延伸至choanae。在冠状平面上,描述了嗅裂的加宽约12毫米。FESS观察到息肉样肿块起源于后隔膜,并向前延伸到所有鼻道,向后延伸到choanae。息肉样病变经内镜完全切除。组织病理学分析显示为浆膜粘质错构瘤。
    浆膜黏液性错构瘤是一种罕见的具有潜在恶性改变的鼻腔鼻窦区良性肿瘤。不幸的是,它们与鼻窦区域的其他良性疾病具有共同的症状和临床表现。因此,将它们视为鉴别诊断更为重要。
    UNASSIGNED: Sinonasal hamartomas, according to the 5th edition of the World Health Organisation classification of head and neck tumours are divided into respiratory epithelial adenomatoid hamartoma (REAH), seromucinous hamartoma and chondromesenchymal hamartoma. Seromucinous hamartoma are benign proliferations of small eosinophilic glands surrounded by fibrous stroma and cuboidal cells. Hamartomas of the nasal cavity and paranasal sinuses are rare entities, clinically presenting as sinonasal polyposis.
    UNASSIGNED: A 79- year-old female patient was referred to our emergency room due to severe dyspnea. Anterior rhinoscopy revealed unilateral greyish polypoid mass obstructing the middle, inferior and common nasal meatus. Systemic corticosteroids and oxygen therapy were administered under observation. Computerized tomographic imaging of the paranasal sinuses with contrast on all three planes showed an opacified polypoid mass in all meatus and the maxillary, anterior ethmoidal and sphenoidal sinus posteriorly extending to the choanae. On the coronal plane a widening of the olfactory clefts about 12 mm was described. FESS visualized that the polypoid mass originated from the posterior septum and extended to all meatus anteriorly and to the choanae posteriorly. The polypoid lesion was endoscopically completely excised. Histopathological analysis revealed a seromucinous hamartoma.
    UNASSIGNED: Seromucinous hamartoma are rare benign tumors of the sinonasal region with potential of malignant alteration. Unfortunately, they share symptoms and clinical appearance with other benign conditions of the sinonasal region. Therefore, it is even more important to consider them as a differential diagnose.
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  • 文章类型: Journal Article
    血管外皮细胞瘤(GPC)是一种罕见的良性鼻窦肿瘤,起源于Zimmerman毛细血管周围的周细胞,占所有鼻窦肿瘤的0.05%以下。血管外皮细胞瘤具有较低的恶性可能性(5-10%),并且主要在6岁或7岁时被诊断出,女性略有优势。我们在这里介绍了5例鼻窦GPC患者的病例系列。这项研究是在印度北部的三级医疗保健中心进行的。在我们的案例系列中,所有患者均接受评估并接受内镜手术切除.一切患者均行数字减影血管造影(DSA)及术前栓塞。用于止血的消融术被证明非常有效且节省时间。所有患者均显示细胞质SMA阳性(GPC的标志物)和CD34阴性,而一名患者表现出较高的Ki-67指数(>10%),这是侵袭性肿瘤行为的预测指标。随访中无一例患者出现复发。我们建议进行完整的内窥镜手术切除以防止复发。DSA的使用,术前栓塞,术中使用消融术技术提供了一个更清洁的手术领域和减少的手术时间。
    Glomangiopericytoma (GPC) is a rare benign sinonasal tumor originating from Zimmerman\'s Pericytes surrounding capillaries and accounting for less than 0.05% of all sinonasal tumors. Glomangiopericytoma has low malignant potential (5-10%) and is mostly diagnosed in the 6th or 7th decade of age with slight female preponderance. We presented here a case series of 5 patients with sinonasal GPC. This research was conducted at a tertiary healthcare centre in North India. In our case series, all the patients were evaluated and underwent endoscopic surgical resection. All patients underwent digital subtraction angiography (DSA) and preoperative embolization. The coblation technique used for haemostasis proved very effective and time-saving. All patients exhibited cytoplasmic SMA positivity (a marker of GPC) and CD34 negativity, while one patient exhibited a high Ki-67 index (> 10%), which is a predictor of aggressive tumor behavior. None of the patients showed any recurrence in follow-up. We recommend performing complete endoscopic surgical excision to prevent recurrence. The use of DSA, preoperative embolization, and intraoperative use of the coblation technique provides a cleaner surgical field and reduced operating time.
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  • 文章类型: Review
    背景:肠腺癌占该地区所有恶性肿瘤的不到0.1-4%。这在木工和皮革工人中很常见。鼻窦腺癌通常起源于筛窦(40%)或鼻腔(25%)。延伸到附近的结构是常见的,但是颅内扩散非常罕见。这些肿瘤通常用手术治疗,报告的5年生存率为59%至80%。
    方法:这是一名60岁的黑人非洲男性患者,他出现了全球性头痛,睡眠时鼻塞伴有打鼾,嗅觉缺失,心理变化,有时躁动和左侧视力丧失持续一年,并在过去一个月内恶化上述症状。他不能闻到肥皂两侧;在他的左眼,他只能看到手的运动在近30厘米。在脑磁共振成像中,有一个T1低和T2高强度的前颅窝肿块,由左筛窦和蝶窦引起,并压迫了左光学结构,脑计算机断层扫描显示出异质的低密度到等密度的肿块。完成肿瘤切除并出院,并有显着改善,并与肿瘤单位进行放射治疗有关。
    结论:这些患者的管理是多学科的,涉及神经外科医生,耳鼻喉科医师,肿瘤学家,还有颌面外科医生.手术切除是主要的治疗策略,其次是放射治疗,特别是强度调节疗法。化疗的使用非常先进,转移性,和不可切除的肿瘤。
    BACKGROUND: Intestinal adenocarcinoma accounts for less than 0.1-4% of all malignancies in the region. It is common among woodworkers and leather workers. Sinonasal adenocarcinoma usually arises from the ethmoid sinus (40%) or nasal cavity (25%). Extension to nearby structures is common, but intracranial spread is very rare. These tumors are usually treated with surgery, with a reported 5-year survival rate of 59% to 80%.
    METHODS: This is a 60-year-old Black African male patient who presented with globalized headache, nasal obstruction with snoring during sleep, anosmia, change in mentation, sometimes agitation and left-side visual loss of one-year duration with worsening his above symptoms over the last one month. He couldn\'t smell soap bilaterally; in his left eye he could see only hand movement at nearly 30 cm. On brain magnetic resonance imaging, there was a T1 hypo- and T2 hyper-intense anterior cranial fossa mass arising from the left ethmoid sinuses and sphenoid sinuses and compressing the left optic structures, and brain computed tomography demonstrated heterogeneous hypo- to isodense mass. Complete tumor excision achieved and discharged with significant improvement and linked to oncology unit for radiotherapy.
    CONCLUSIONS: The management of these patients is multidisciplinary, involving neurosurgeons, otolaryngologists, oncologists, and maxillofacial surgeons. Surgical resection is the main treatment strategy, followed by radiotherapy, particularly intensity-modulated therapy. Chemotherapy is used in highly advanced, metastatic, and unresectable tumors.
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