Sinonasal

鼻窦
  • 文章类型: Journal Article
    背景:高危型人乳头瘤病毒(HR-HPV)感染越来越被认为是鼻窦炎的危险因素。然而,由于研究有限和文献中HPV检测方式的不一致,HPV相关的鼻窦癌的患病率和预后意义尚不为人所知。形态学上,HPV相关的鼻窦癌包括不同组的肿瘤。尚未报道HPV相关的鼻窦腺癌。这项研究的目的是确定患病率,HPV相关的鼻腔鼻窦癌的形态学谱和预后意义。
    方法:该队列包括153例鼻腔鼻窦癌。构建组织微阵列。进行P16免疫组织化学和HR-HPVE6/7原位杂交(ISH)。根据阳性的ISH检测,认为癌与HPV相关。收集临床病理数据。
    结果:28/153(18%)鼻腔鼻窦癌与HPV相关。HPV相关癌包括26(93%)鳞状细胞癌和变种,1(3.5%)HPV相关的多表型鼻腔鼻窦癌和1(3.5%)腺癌。HPV相关腺癌在形态上与HPV相关宫颈腺癌非常相似。HPV相关癌发生于8名(29%)女性和20名(71%)男性,中位年龄为66岁。HPV相关癌主要位于鼻腔。观察到HPV相关癌患者的总生存期和无进展生存期有改善的趋势,但没有统计学意义。
    结论:我们的研究确定了一种新的HPV相关的鼻窦腺癌亚型,突出了HPV相关的鼻窦癌的广泛形态谱,并支持病理实践期间的常规p16检测,无论肿瘤亚型如何,随后进行确认性HR-HPV检测。这种做法对于研究HPV相关的鼻窦癌的临床行为至关重要。
    BACKGROUND: High-risk human papillomavirus (HR-HPV) infection has been increasingly recognized as a risk factor for sinonasal tract carcinomas. However the prevalence and prognostic significance of HPV-associated sinonasal carcinomas is not well known due to limited studies and inconsistency in HPV testing modalities in literatures. Morphologically, HPV-associated sinonasal carcinomas encompass a diverse group of tumors. HPV-associated sinonasal adenocarcinoma has not been reported. The purpose of this study was to determine the prevalence, morphologic spectrum and prognostic implication of HPV-associated sinonasal carcinomas.
    METHODS: This cohort included 153 sinonasal carcinomas. Tissue microarrays were constructed. P16 immunohistochemistry and HR-HPV E6/7 in-situ Hybridization (ISH) were performed. Carcinomas were deemed HPV-associated based on a positive ISH testing. Clinicopathologic data was collected.
    RESULTS: 28/153 (18%) sinonasal carcinomas were HPV-associated. HPV-associated carcinomas consisted of 26 (93%) squamous cell carcinomas and variants, 1 (3.5%) HPV-related multiphenotypic sinonasal carcinoma and 1 (3.5%) adenocarcinoma. The HPV-associated adenocarcinoma closely resembled HPV-associated endocervical adenocarcinoma morphologically. HPV-associated carcinomas occurred in 8 (29%) women and 20 (71%) men with a median age of 66 years old. HPV-associated carcinomas were predominantly located at nasal cavity. A trend toward improved overall survival and progression free survival in HPV-associated carcinomas patients was observed, yet without statistical significance.
    CONCLUSIONS: Our study identifies a novel HPV-associated sinonasal adenocarcinoma subtype, highlights the broad morphologic spectrum of HPV-associated sinonasal carcinomas, and supports routine p16 testing during pathology practice regardless of tumor subtype followed by a confirmatory HR-HPV testing. This practice is critical for studying the clinical behavior of HPV-associated sinonasal carcinomas.
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  • 文章类型: Journal Article
    背景:本研究旨在评估动态对比增强磁共振成像(DCE-MRI)和弥散加权成像(DWI)参数在区分鼻窦淋巴瘤和鼻窦癌方面的诊断效能。
    方法:42例经组织学证实的鼻腔鼻窦淋巴瘤和52例鼻腔鼻窦癌患者用3.0TMRI扫描仪进行成像。进行了DCE-MRI和DWI,和各种参数,包括时间-强度曲线(TIC)的类型,时间达到顶峰,峰值增强,峰值对比度增强,冲洗率,表观扩散系数(ADC),测量相对ADC。采用二元logistic回归和受试者工作特征(ROC)曲线分析来评估单独和组合指标对鼻窦淋巴瘤和鼻窦癌的诊断能力。
    结果:鼻窦淋巴瘤主要表现为II型TIC(n=20),而鼻腔鼻窦癌主要表现为III型TIC(n=23)。除冲洗比(p<0.05)外,所有参数均存在显着差异。ADC值成为单一参数中最可靠的诊断工具。与个别参数相比,DCE-MRI联合参数显示出更好的诊断效能。当组合DCE-MRI和DWI的所有参数时,效率最高(曲线下面积=0.945)。
    结论:涉及对比增强动态MRI和DWI的多参数评估在区分鼻窦淋巴瘤和鼻窦癌方面具有相当大的诊断价值。
    BACKGROUND: The study aimed to evaluate the diagnostic efficacy of dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) and diffusion-weighted imaging (DWI) parameters in distinguishing sinonasal lymphoma from sinonasal carcinoma.
    METHODS: Forty-two participants with histologically confirmed sinonasal lymphomas and fifty-two cases of sinonasal carcinoma underwent imaging with a 3.0T MRI scanner. DCE-MRI and DWI were conducted, and various parameters including type of time-intensity curve(TIC), time to peak, peak enhancement, peak contrast enhancement, washout rate, apparent diffusion coefficient (ADC), and relative ADC were measured. Binary logistic regression and receiver operating characteristic (ROC) curve analysis were employed to assess the diagnostic capability of individual and combined indices for differentiating nasal sinus lymphoma from nasal sinus carcinoma.
    RESULTS: Sinonasal lymphoma predominantly exhibited type II TIC(n = 20), whereas sinonasal carcinoma predominantly exhibited type III TIC(n = 23). Significant differences were observed in all parameters except washout ratio (p < 0.05), and ADC value emerged as the most reliable diagnostic tool in single parameter. Combined DCE-MRI parameters demonstrated superior diagnostic efficacy compared to individual parameters, with the highest efficiency (area under curve = 0.945) achieved when combining all parameters of DCE-MRI and DWI.
    CONCLUSIONS: Multiparametric evaluation involving contrast-enhanced dynamic MRI and DWI holds considerable diagnostic value in distinguishing sinonasal lymphoma from sinonasal carcinoma.
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  • 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
    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: 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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  • 文章类型: Journal Article
    背景:低分化的鼻窦小圆细胞肿瘤(SRCT)罕见且异质性,在诊断和治疗方面构成挑战。
    方法:分子发现和诊断改进的最新进展促进了对这些肿瘤的更好理解和治疗。
    结果:新定义和新出现的鼻窦实体表现出不同的形态,特定的基因组特征,和常规同行的临床行为。在这篇关于SRCT的综述中,重点放在诊断方法与免疫组织化学研究和/或分子测试的相关小组的就业,微调到最新的WHO5分类的鼻窦/鼻旁肿瘤和个性化治疗。
    结论:具体来说,本文对上皮和神经外胚层衍生肿瘤进行综述。
    BACKGROUND: Poorly differentiated sinonasal small round cell tumors (SRCTs) are rare and heterogeneous, posing challenges in diagnosis and treatment.
    METHODS: Recent advances in molecular findings and diagnostic refinement have promoted better understanding and management of these tumors.
    RESULTS: The newly defined and emerging sinonasal entities demonstrate diverse morphologies, specific genomic signatures, and clinical behavior from conventional counterparts. In this review of SRCTs, emphasis is placed on the diagnostic approach with the employment of a pertinent panel of immunohistochemistry studies and/or molecular tests, fine-tuned to the latest WHO 5 classification of sinonasal/paranasal tumors and personalized treatment.
    CONCLUSIONS: Specifically, this review focuses on tumors with epithelial and neuroectodermal derivation.
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