Sinonasal

鼻窦
  • 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
    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
    上皮-肌上皮癌(EMC)在世界卫生组织分类中被认为是一种独特的病理实体。这是一种极其罕见的涎腺低级别癌,具有特征性的双相管状结构。它主要发生在腮腺,但也可见于鼻咽,泪腺,鼻旁窦,喉部,肺。鼻EMC(不包括鼻旁窦作为原发性肿瘤部位)非常罕见,迄今为止仅报告13例。在这个案例报告中,我们描述了一个鼻EMC延伸到鼻咽的病例,其临床特点和管理。我们还对所有鼻EMC的相关病例进行了文献综述。
    我们在PubMed数据库中搜索了1950年1月至2022年12月之间关于鼻EMC的文章,以进行此评论。
    我们发现了13例相关的鼻EMC病例报告,中位年龄为58岁,女性占优势。我们发现我们的病人是迄今为止最年轻的病人。两个案例,包括目前的研究,显示肿瘤的中心位于鼻腔后部,延伸到choana和鼻咽。最常见的表现是鼻出血,其次是鼻塞。14例中只有4例具有手术切缘状态的信息,其中只有一个手术切缘阳性。5名患者(包括本研究中的患者)接受了辅助放疗;但是6名患者(42.8%)没有接受任何辅助放疗。
    UNASSIGNED: Epithelial-myoepithelial carcinoma (EMC) was recognised as a distinct pathologic entity in World Health Organisation classification. It is an extremely rare low grade carcinoma of salivary gland, with characteristic biphasic tubular structures. It predominantly occurs in Parotid gland but can also be seen in nasopharynx, lacrimal gland, paranasal sinuses, larynx, lung. Nasal EMC (excluding the paranasal sinuses as primary tumour site) are very rare with only 13 cases reported till date. In this case report, we described a case of nasal EMC extending into nasopharynx, its clinical features and management. We have also done a literature review of all the relevant cases of nasal EMC.
    UNASSIGNED: We searched the PubMed database for articles between January 1950 and December 2022 for nasal EMC for this review.
    UNASSIGNED: We found 13 relevant case reports of nasal EMC and median age was 58 years with female preponderance. We found that our patient was the youngest to be reported till date. Two cases, including the current study, showed epicentre of the tumour in posterior nasal cavity, extending to choana and nasopharynx. Most common presentation was epistaxis, followed by nasal obstruction. Only 4 out of 14 cases had information on surgical margin status, out of which only one has positive surgical margin. Five patients (including the patient in the current study) received adjuvant radiotherapy; however 6 patients (42.8%) did not receive any adjuvant radiotherapy.
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  • 文章类型: Case Reports
    鼻窦区域的恶性黑色素瘤是一种罕见的肿瘤,由鼻粘膜中的黑素细胞引起,比皮肤型更具侵袭性,预后不良。我们报告了一名60岁的女性,最初的主诉是鼻腔充盈,持续的鼻出血,和鼻骨畸形在过去的两个月。在初级考试中,发现了一个黑色肿块,在切除活检中,病理学家报告了鼻腔鼻窦恶性黑色素瘤,IHC染色后证实。在头颈部的梭形细胞肿瘤中,我们应该意识到粘膜恶性黑色素瘤的鉴别诊断。
    Malignant melanoma of the sinonasal area is a rare tumor that arises from melanocytes in the nasal mucosa and is more aggressive than the cutaneous type with a poor prognosis. We report a 60-year-old female with the initial chief complaint of nasal cavity fullness, continuous epistaxis, and nasal bone deformity in the past two months. In a primary examination, a black mass was found, and in an excisional biopsy, the pathologist reported sinonasal malignant melanoma, which was confirmed after IHC staining. In spindle cell tumors of the head and neck area, we should be aware of mucosal malignant melanoma as a differential diagnosis.
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  • 文章类型: Systematic Review
    背景:鼻窦NUT是一种极其罕见的,致命的恶性肿瘤,文献有限。
    方法:一个病例系列是2010年至2022年在单个机构中所有患有鼻窦NUT癌的患者的传导。评估生存率和相关性。对文献进行了系统回顾。
    结果:在12名患者中,随后的中位数为1.5年,中位总生存期(OS)和疾病特异性生存期(DSS)均为14.6个月.患有上颌窦肿瘤的患者存活的可能性增加了91%(风险比[HR]:0.094,95%置信区间[CI]:0.011-0.78,p=0.011)。疾病分期较高的患者OS较差(IVb-c期与III-IVa,p=0.05)。所有三名没有疾病迹象的活着的患者都接受了诱导化疗。
    结论:对于鼻窦NUT癌患者,中位生存期为15个月,但较低分期和上颌骨肿瘤更好.诱导化疗可能是有益的。
    BACKGROUND: Sinonasal NUT carcinoma is an extremely rare, lethal malignancy with limited literature.
    METHODS: A case series was conduction of all patients with sinonasal NUT carcinoma at a single institution between 2010 and 2022. Survival and associated were evaluated. A systematic review of the literature was performed.
    RESULTS: In 12 patients, followed for a median of 1.5 years, the median overall survival (OS) and disease-specific survival (DSS) were both 14.6 months. Patients with maxillary sinus tumors were 91% more likely to survive (hazard ratio [HR]: 0.094, 95% confidence interval [CI]: 0.011-0.78, p = 0.011). Patients with higher-stage disease stage had worse OS (stage IVb-c vs. III-IVa, p = 0.05). All three patients who were alive with no evidence of disease received induction chemotherapy.
    CONCLUSIONS: For patients with sinonasal NUT carcinoma, the median survival was 15 months but better with lower-stage and maxillary tumors. Induction chemotherapy may be beneficial.
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  • 文章类型: Case Reports
    鼻窦神经内分泌癌是一种罕见的头颈部肿瘤,仅占鼻窦肿瘤的5%。该病变有很高的侵入邻近结构的风险,例如轨道,颅底,还有软组织,症状通常是非特异性的。大多数病例是晚期诊断的,不治疗降低总生存率。迄今为止,鉴于其患病率较低,在管理上没有达成共识;然而,研究表明,多模式疗法,以正确的手术方式为主,提供更好的无病预后。
    一名46岁女性,有1年的鼻部症状史,以阻塞和鼻出血为特征。影像学检查显示轴外肿块导致颅底侵蚀和右额眶区移位,不侵入脑实质或脑膜。进行了活检,并诊断出不可切除的低分化鼻腔鼻窦神经内分泌癌。开始用放疗和化疗治疗,随着肿瘤体积的减少,她被转诊为神经外科介入治疗;进行了鼻内镜入路.完全切除,患者出院,无术后并发症,影像学上无残留病灶。
    我们描述了一种罕见的晚期肿瘤的演变过程。它强调了尽管接受了不可切除的肿块的初步诊断,多模式疗法,和适当的手术方法认为整个病变被切除。尽管临床进展良好,神经内分泌癌的随访优先考虑复发和转移率高的肿瘤。
    UNASSIGNED: Sinonasal neuroendocrine carcinoma is a rare head and neck tumor that represents only 5% of sinonasal neoplasms. This lesion has a high risk of invasion to adjacent structures such as the orbit, skull base, and soft tissues, with symptoms usually being nonspecific. Most cases are diagnosed in late stages, decreasing overall survival without treatment. To date, there is no consensus on management given its low prevalence; however, it has been shown that multimodal therapy, with the correct surgical approach as the mainstay, offers a better disease-free prognosis.
    UNASSIGNED: A 46-year-old woman presented with a 1 year history of nasal symptoms, characterized by obstruction and epistaxis. Imaging studies showed an extra-axial mass causing skull base erosion and displacement of the right fronto-orbital region, without invasion of brain parenchyma or meninges. A biopsy was performed and an unresectable poorly differentiated sinonasal neuroendocrine carcinoma was diagnosed. Treatment with radio and chemotherapy was initiated and, as the tumoral volume decreased, she was referred for neurosurgical intervention; an endonasal endoscopic approach was performed. Gross total resection was achieved and the patient was discharged without postoperative complications and no residual lesion on imaging.
    UNASSIGNED: We describe the evolution of a rare advanced-stage neoplasm. It highlights that despite receiving an initial diagnosis of an unresectable mass, multimodal therapy, and an adequate surgical approach deemed the entire lesion to be resected. Despite the favorable clinical evolution, the follow-up of neuroendocrine carcinoma is prioritized as a neoplasm with a high rate of recurrence and metastasis.
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