Sinonasal tract

鼻窦道
  • 文章类型: Case Reports
    本报告介绍了一例很少发生的内翻鳞状乳头状瘤,显示鳞状上皮的乳头状增生。内翻性乳头状瘤(IPs)是良性上皮生长,发生在鼻腔和鼻旁窦的下层基质中。而病毒感染,过敏,和慢性鼻窦炎都被认为是潜在的原因,该病变的病理生理学仍然未知。大多数时候,IP反映了残留疾病,然而复发率极高。一名60岁的男性患者主要抱怨右侧鼻塞和过度打喷嚏和从鼻子排出,这会给病人带来不适,使他晚上无法入睡。计算机断层扫描显示了鼻腔右侧的骨道复合体和软组织密度浑浊。诊断这种鳞状乳头状瘤的唯一方法是通过组织病理学检查。在这项工作中,我们评估了鼻窦IP的组织学特征,并提出了一例罕见的鼻腔倒置鳞状乳头状瘤的病例报告。
    This report presents a case of a rarely occurring inverted squamous papilloma, which shows papillary proliferation in squamous epithelium. Inverted papillomas (IPs) are benign epithelial growths that occur in the underlying stroma of the nasal cavity and paranasal sinuses. While viral infections, allergies, and chronic sinusitis have all been proposed as potential causes, the pathophysiology of this lesion is still unknown. Most of the time, IP reflects residual disease, yet the recurrence rates are extremely high. A 60-year-old male patient has chief complaints of right-sided nasal congestion and excessive sneezing and discharge from the nose, which cause discomfort to the patient and make him unable to sleep at night. Computed tomography reveals both the enlargement of the osteomeatal complex and soft-tissue density opacification of the right side of the nasal cavity. The only way to diagnose this type of squamous papilloma is through histopathological examination. In this work, we evaluated the histological characteristics of sinonasal IP and presented a case report of an uncommon instance of inverted squamous papilloma of the nasal cavity.
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  • 文章类型: Journal Article
    软骨骨性呼吸道腺瘤样错构瘤(COREAH)是极其罕见的鼻窦道良性病变。它存在于3至83岁的人群中。它起源于鼻窦道的各个部位;最常见的部位是鼻腔的后侧壁。成像钙化是COREAH的重要发现。在这个案例报告中,我们提出了一个非常罕见的事件,涉及一名55岁的女性患者,表现出鼻塞的症状,左侧鼻腔出血和嗅觉丧失。我们的目标是分享我们在管理这一独特条件方面的经验。诊断性鼻内镜检查显示患者呈红色,左鼻腔中的肉质息肉状块从左鼻腔的侧壁向后产生,并向前延伸到左鼻腔。患者接受了切除活检。经显微镜检查,我们观察到呼吸道型腺体的错构瘤增生,表现为黏液化生。此外,存在许多成熟的骨针,COREAH的一个显著特征。患者接受了6个月的随访,在此期间,在鼻腔内未检测到复发性生长。
    Chondro-osseous respiratory adenomatoid hamartoma (COREAH) are extremely rare benign lesions of the sinonasal tract. It is present in 3 to 83-year-olds. It originates from various sites of the sinonasal tract; the most common site is the posterior lateral wall of the nasal cavity. Imaging calcification is an important finding in COREAH. In this case report, we present an exceptionally rare occurrence involving a 55-year-old female patient who exhibited symptoms of nasal obstruction, bleeding from the left side of the nasal cavity and loss of smell. We aim to share our experience in managing this unique condition. Diagnostic nasal endoscopic examination of the patient revealed a reddish, fleshy polypoidal mass in the left nasal cavity arising from the lateral wall of the left nasal cavity posteriorly and extending anteriorly into the left nasal cavity. The patient underwent an excisional biopsy. Upon microscopic examination, we observed a hamartomatous proliferation of respiratory-type glands exhibiting mucinous metaplasia. Additionally, numerous spicules of mature bone were present, a distinctive feature of COREAH. The patient underwent a 6-month follow-up, during which no recurrent growth was detected within the nasal cavity.
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  • 文章类型: Journal Article
    血管平滑肌瘤是一种罕见的肿瘤,占所有头颈部良性肿瘤的0.2%,占鼻窦肿瘤总病例的2%。它曾经被认为是平滑肌瘤的一种可能亚型,但是,在2020年世界卫生组织(WHO)软组织肿瘤分类中,它被接受为一个单一的实体。目的根据软组织肿瘤的新分类,系统回顾血管平滑肌瘤的现有文献。数据综合本研究根据系统评价和荟萃分析(PRISMA)声明的优选报告项目进行。在PubMed中进行全面搜索,科克伦,Scopus,GoogleScholar数据库于2022年1月进行。搜索项目包括以下关键词:鼻血管平滑肌瘤或鼻窦血管平滑肌瘤或鼻血管平滑肌瘤或鼻窦血管平滑肌瘤。对87例患者进行了评估。研究中患者的年龄范围为15至88岁(诊断时的平均年龄:55.6岁)。最常见的受累部位是鼻中隔(28.4%),其次是下鼻甲(22.5%)。最常见的症状是鼻塞(66.7%),其次是鼻出血(47.1%)。手术切除是主要的治疗方法,仅1例(0.9%)病理复发。结论据我们所知,先前仅描述了87例鼻腔鼻窦血管平滑肌瘤。本综述的结果似乎证实了这种肿瘤的稀有性和良性性质,他们似乎证实了改善有关鼻窦血管平滑肌瘤的现有数据的必要性。
    Introduction  Angioleiomyoma is a rare neoplasm that represents ∼ 0.2 % of all head and neck benign tumors and ∼ 2% of total cases of tumors of the sinonasal tract. It was once considered a possible subtype of leiomyoma, but, in the 2020 World Health Organization (WHO) classification of soft tissue tumors, it is accepted as a singular entity. Objective  To systematically review the existing literature on angioleiomyoma in the light of the new classification of soft tissue tumors. Data Synthesis  The present study was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A comprehensive search in the PubMed, Cochrane, Scopus, and Google Scholar databases was performed in January 2022. The search items included the following keywords: nasal angioleiomyoma OR sinonasal angioleiomyoma OR nasal vascular leiomyoma OR sinonasal vascular leiomyoma . A total of 87 patients were evaluated. He age of the patients in the studies ranged from 15 to 88 years (mean age at diagnosis: 55.6 years). The most common site of involvement was the nasal septum (28.4 %), followed by the inferior turbinate (22.5%). The most common symptom was nasal obstruction (66.7%), followed by epistaxis (47.1%). Surgical excision represented the main treatment, and there was recurrence of pathology in only 1 case (0.9%). Conclusion  To our knowledge, only 87 cases of sinonasal-tract angioleiomyoma have been previously described. The results of the present review seem to confirm the rarity and the benign nature of this neoplasm, and they seem to confirm the necessity to improve the available data about sinonasal-tract angioleiomyoma.
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  • 文章类型: Journal Article
    目的:选择性颈清扫术(END)在临床N0(cN0)鼻窦鳞状细胞癌(SCC)治疗中的作用尚不清楚。在这次系统审查中,我们评估了有cN0M0肿瘤的鼻窦SCCs隐匿性淋巴结转移的风险,以支持临床决策.
    方法:在以下三个电子数据库中进行了文献检索:Medline/PubMed,ScienceDirect,谷歌学者。根据系统审查和荟萃分析(PRISMA)声明的首选报告项目评估文章的资格。两位独立作者提取了数据。非随机研究的方法学项目(MINORS)工具用于评估每个纳入研究的偏见。
    结果:我们的系统评价包括六项符合纳入标准的研究,所有的设计都是回顾性的。经组织学证实的鼻窦SCC转移至临床阴性颈部的发生率为12.5%。几乎一半的阳性病例在病理上为N2(6.5%)。
    结论:我们的系统评价提供了鼻窦SCC隐匿性转移到颈部的发生率,以便外科医生可以与患者讨论在手术计划中增加选择性颈部管理的风险和可能的优点。
    OBJECTIVE: The role of elective neck dissection (END) in the management of clinical N0 (cN0) squamous cell carcinomas (SCC) of the sinonasal tract is unclear. In this systematic review, we evaluate the risk of occult nodal metastasis in sinonasal SCCs with cN0M0 tumors to support clinical decision making.
    METHODS: A literature search was conducted in the following three electronic databases: Medline/PubMed, ScienceDirect, and Google Scholar. Articles were assessed for eligibility in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) statement. Two independent authors extracted the data. The Methodological Items for Non-Randomized Studies (MINORS) tool was used for the assessment of biases of each included study.
    RESULTS: Our systematic review included six studies that met the inclusion criteria, all retrospective in design. The rate of histologically proven metastasis of sinonasal SCC to the clinically negative neck is 12.5%. Almost half of the positive cases are pathologically staged as N2 (6.5%).
    CONCLUSIONS: Our systematic review provides the rate of sinonasal SCC occult metastasis to the neck so that the surgeons can discuss with patients the risks and possible merits of adding an elective neck management in the surgical plan.
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  • 文章类型: Journal Article
    EWSR1::POU2AF3:(COLCA2)肉瘤是最近发现的一组未分化的圆形/梭形细胞肿瘤,对头颈部区域有好感。在这里,我们报告了我们8例的经验,发生在5名男性和3名女性(年龄范围:37-74岁;中位数60岁)。肿瘤累及头颈部(4例),大腿各一个,胸壁,腓骨和肺。7名患者接受了多模式治疗,一名患者仅接受手术治疗。临床随访(8例;范围4-122个月;中位数:32个月)显示5例转移患者(通常为多灶性,延迟范围为7-119个月),其中3例伴有局部复发。中位无局部复发和无转移生存率分别为24个月和29个月。分别。在这8名患者中,1人死于不明原因,4人患有转移性疾病,1例有不可切除的局部疾病,2例无疾病。肿瘤由2个形态学亚组组成:1)相对温和的肿瘤,由具有不同细胞组成的星状细胞和纤维黏液样基质(2例)和2)明显的恶性肿瘤,由“神经内分泌出现的圆形细胞包围的巢状细胞(6例)。第2组个别病例呈腺体,成骨或横纹肌母细胞分化。免疫组织化学结果包括:CD56(4/4例),GFAP(5/8)SATB2(4/6),角蛋白(AE1/AE3)(5/8)和S100蛋白(4/7)。RNA测序在所有情况下鉴定了EWSR1::POU2AF3基因融合。5例经FISH证实EWSR1基因重排。我们的发现证实了EWSR1::POU2AF3肉瘤的头颈部好感和侵袭性临床行为,并扩大了这些罕见病变的形态范围,包括相对温和的梭形细胞肿瘤和分化不同的肿瘤。
    EWSR1::POU2AF3 (COLCA2) sarcomas are a recently identified group of undifferentiated round/spindle cell neoplasms with a predilection for the head and neck region. Herein, we report our experience with 8 cases, occurring in 5 men and 3 women (age range, 37-74 years; median, 60 years). Tumors involved the head/neck (4 cases), and one each the thigh, thoracic wall, fibula, and lung. Seven patients received multimodal therapy; 1 patient was treated only with surgery. Clinical follow-up (8 patients; range, 4-122 months; median, 32 months) showed 5 patients with metastases (often multifocal, with a latency ranging from 7 to 119 months), and 3 of them also with local recurrence. The median local recurrence-free and metastasis-free survival rates were 24 months and 29 months, respectively. Of the 8 patients, 1 died of an unknown cause, 4 were alive with metastatic disease, 1 was alive with unresectable local disease, and 2 were without disease. The tumors were composed of 2 morphologic subgroups: (1) relatively bland tumors consisting of spindled to stellate cells with varying cellularity and fibromyxoid stroma (2 cases) and (2) overtly malignant tumors composed of nests of \"neuroendocrine-appearing\" round cells surrounded by spindled cells (6 cases). Individual cases in the second group showed glandular, osteogenic, or rhabdomyoblastic differentiation. Immunohistochemical results included CD56 (4/4 cases), GFAP (5/8), SATB2 (4/6), keratin (AE1/AE3) (5/8), and S100 protein (4/7). RNA sequencing identified EWSR1::POU2AF3 gene fusion in all cases. EWSR1 gene rearrangement was confirmed by fluorescence in situ hybridization in 5 cases. Our findings confirm the head/neck predilection and aggressive clinical behavior of EWSR1::POU2AF3 sarcomas and widen the morphologic spectrum of these rare lesions to include relatively bland spindle cell tumors and tumors with divergent differentiation.
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  • 文章类型: Journal Article
    背景:头颈部粘膜黑素瘤是罕见的恶性肿瘤。尽管由于远处转移的发生率高,预后较差,局部控制仍然很重要。由于其稀有性,很难在大型队列中获得结果。这项研究旨在阐明在日本接受手术治疗的头颈部粘膜黑色素瘤患者的生存结果。
    方法:对2007年至2021年在美国国家癌症中心医院接受手术治疗的头颈部粘膜黑色素瘤患者进行回顾性分析。
    结果:本研究共纳入47例患者。5年总生存率,疾病特异性生存,局部控制和无复发生存率为42%,50%,79%和13%,分别。口腔粘膜黑色素瘤组的疾病特异性生存率明显优于鼻窦粘膜黑色素瘤组(5年疾病特异性生存率:70%对37%,分别为;P=0.04)。多因素分析显示,鼻腔鼻窦粘膜黑色素瘤是独立的显著不良预后因素,总生存率和疾病特异性生存率。口腔粘膜黑色素瘤患者的淋巴结转移发生率高于鼻窦粘膜黑色素瘤患者(P<0.0001)。
    结论:本研究证明了日本过去20年中在单一机构手术治疗的最大头颈部粘膜黑素瘤患者队列的生存结果。我们发现,生存结果和淋巴结转移的发生率因部位而异。
    BACKGROUND: Head and neck mucosal melanomas are rare malignancies. Although the prognosis is poor owing to the high incidence of distant metastases, locoregional control remains important. It is difficult to obtain results in a large cohort because of its rarity. This study aimed to elucidate the survival outcomes of patients with head and neck mucosal melanoma treated with surgery in Japan.
    METHODS: Patients with head and neck mucosal melanoma who were surgically treated between 2007 and 2021 at the National Cancer Center Hospital were retrospectively analyzed.
    RESULTS: A total of 47 patients were included in this study. The 5-year overall survival, disease-specific survival, locoregional control and relapse-free survival rates were 42%, 50%, 79% and 13%, respectively. The disease-specific survival of the oral mucosal melanoma group was significantly better than that of the sinonasal mucosal melanoma group (5-year disease-specific survival rate: 70% versus 37%, respectively; P = 0.04). Multivariate analyses revealed that sinonasal mucosal melanoma were independently significant adverse prognostic factor, for overall survival and disease-specific survival. Patients with oral mucosal melanoma patients had a higher incidence of lymph node metastasis than those with sinonasal mucosal melanoma patients (P < 0.0001).
    CONCLUSIONS: This study demonstrated the survival outcomes of the largest cohort of patients with head and neck mucosal melanomas treated surgically at a single institution within the past 20 years in Japan. We found that survival outcomes and incidence of nodal metastases varied by site.
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  • 文章类型: Journal Article
    目的:癌症的Warburg效应已通过18F-氟脱氧葡萄糖(18F-FDG)在正电子发射断层扫描(PET/CT)上的摄取来检测各种癌症。肺癌的18F-FDG-PET/CT预测表皮样生长因子受体(EGFR)的突变状态。本研究旨在探讨18F-FDG摄取参数是否与鼻腔鼻窦鳞状细胞癌(STSCC)患者EGFR突变状态显著相关。
    方法:收集经明确治疗的原发性STSCC患者的29例肿瘤标本。
    结果:在Mann-WhitneyU检验或受试者工作曲线上,原发性肿瘤的18F-FDG摄取在EGFR的突变状态和野生状态之间没有差异。STSCC特异性存活的对数秩检验中p值最小≥25的代谢性肿瘤体积与STSCC特异性明显较短相关。无病,单变量和多变量分析的局部无复发生存率,针对临床分期进行了调整,治疗,和EGFR状态。
    结论:18F-FDG-PET/CT不能预测STSCC中EGFR状态的突变。
    OBJECTIVE: The Warburg effect of cancer has been applied to detect various carcinomas though the 18F-fluorodeoxyglucose (18F-FDG) uptake on positron emission tomography with computed tomography (PET/CT). 18F-FDG-PET/CT in lung cancer predicted the mutation status of epidermoid growth factor receptor (EGFR). This study aimed to investigate whether 18F-FDG uptake parameters were significantly related to EGFR mutation status in patients with sinonasal tract squamous cell carcinoma (STSCC).
    METHODS: Twenty-nine tumor specimens of primary STSCC from patients with definitive treatment were collected.
    RESULTS: The 18F-FDG uptake from primary tumors was not different between mutant- and wild-status of EGFR on either Mann-Whitney U-test or the receiver operating curve. A metabolic tumor volume of ≥25 with the minimum p-value from the log-rank test for STSCC-specific survival was associated with a significantly shorter STSCC-specific, disease-free, local recurrence-free survival on the univariate and multivariate analyses adjusted for the clinical stage, treatment, and EGFR status.
    CONCLUSIONS: 18F-FDG-PET/CT did not predict mutation of the EGFR status in STSCC.
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  • 文章类型: Journal Article
    唾液导管癌(SDC)是一种罕见但高度侵袭性的肿瘤,预后不良。SDC主要来自主要唾液腺,通常是腮腺。这里,我们报告了一例罕见的病例,在一名54岁的男性患者中,可能起源于下鼻甲。患者出现左侧鼻塞和鼻漏。在内窥镜介入后,组织病理学检查显示诊断为SDC,以形成实体癌巢和中央粉刺型坏死为特征。鉴于SDC的高度侵袭性和不良预后,必须将其作为单侧鼻腔肿瘤的鉴别诊断。
    Salivary duct carcinoma (SDC) is an uncommon but highly aggressive tumor with a poor prognosis. SDC mainly arises from the major salivary glands, typically the parotid gland. Here, we report a rare case of sinonasal SDC in a 54-year-old male patient that might have originated from the inferior turbinate. The patient presented with left nasal congestion and rhinorrhea. Following an endoscopic intervention, the histopathological examination revealed a diagnosis of SDC, characterized by the formation of solid cancer nests and central comedo-type necrosis. Given the highly aggressive nature and unfavorable prognosis of SDC, it is essential to consider it as a differential diagnosis for unilateral nasal tumors.
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  • 文章类型: Journal Article
    全世界每年的鼻窦肿瘤发病率为每100,000人中0.5至1.0名患者。P63在上皮发育中起作用,并用作基底和肌上皮细胞的标记。p16的表达是由于人乳头瘤病毒(HPV)E7蛋白对视网膜母细胞瘤蛋白(pRb)的功能性失活而发生的。
    本研究旨在研究良恶性鼻窦肿块病变的组织学谱,并研究p63在不同类型鼻窦肿块病变中的免疫组织化学表达。它还旨在通过p16免疫组织化学确定原发性鼻窦肿块病变中高危HPV的发生率,并描绘HPV相关鼻窦病变的组织学谱。
    这项横断面研究是在2018年6月至2020年6月在一家三级保健医院对80例病例进行的。在研究形式中收集包括人口统计学参数在内的临床病史。记录标本的总体发现,并通过H&E染色进行组织病理学检查。对所有病例进行p63和p16表达的免疫组织化学染色。
    受影响的最常见年龄组为41-60岁,男女比例为1.67:1。非肿瘤性病变(38.7%)占大多数病例,其次是良性肿瘤性病变(31.3%)和恶性肿瘤性病变(30%)。在恶性肿瘤性病变中,p63阳性表达率为75%(p=0.005),p16阳性表达率为41.7%(p=0.023)。在良性和非肿瘤性病变中,p63阳性为21.4%(p=0.000),p16阳性为44.6%(p=0.040)。
    我们分析了p63和p16在不同谱系中的表达,例如癌,乳头状瘤,和鼻窦引起的神经外胚层分化,以及与其他临床病理参数的关系。这项研究表明p63的表达与鳞状细胞癌和鼻咽癌的相关性更高。鼻腔鼻窦恶性肿瘤也与HPV感染相关,可通过p16免疫染色识别,因此,可以为确定与HPV相关的任何明确的生物学和临床特征以及该患者人群的靶向治疗提供新的前景。
    UNASSIGNED: The worldwide annual incidence of carcinomas of the sinonasal tract is 0.5 to 1.0 patients per 100,000 per year. P63 plays a role in epithelial development and is used as a marker for basal and myoepithelial cells. Expression of p16 occurs as a result of functional inactivation of the retinoblastoma protein (pRb) by the human papilloma virus (HPV) E7 protein.
    UNASSIGNED: This study aims to study the histological spectrum of benign and malignant sinonasal mass lesions and to study the immunohistochemical expression of p63 in different type of sinonasal mass lesions. It also aims to ascertain the incidence of high-risk HPV in primary sinonasal mass lesions with p16 immunohistochemistry and delineate the histological spectrum of HPV-related sinonasal lesions.
    UNASSIGNED: This cross-sectional study was conducted on 80 cases from June 2018 to June 2020 at a tertiary care hospital. Clinical history including demographic parameters were collected in the study proforma. The gross findings of the specimens noted and histopathological examination by H&E staining done. Immunohistochemistry staining for p63 and p16 expression was performed on all cases.
    UNASSIGNED: Most common age group affected was 41-60 years with male:female ratio of 1.67:1. Nonneoplastic lesions (38.7%) comprised majority of the cases followed by benign neoplastic lesions (31.3%) and malignant neoplastic lesions (30%). Among the malignant neoplastic lesions, p63 showed positive expression in 75% (p = 0.005) and p16 showed positive expression in 41.7% (p = 0.023). Among benign and nonneoplastic lesions, p63 showed positivity in 21.4% (p = 0.000) and p16 showed positivity in 44.6% (p = 0.040).
    UNASSIGNED: We analyzed p63 and p16 expression in varied lineages like carcinomas, papillomas, and neuroectodermal differentiation arising from the sinonasal tract and also in relation to other clinicopathological parameters. This study revealed p63 expression was associated more with the squamous cell carcinomas and nasopharyngeal carcinomas. Sinonasal tract malignancies are also associated with HPV infections that are identifiable by p16 immunostaining and, thus, could provide new prospects in identifying any definite biological and clinical characteristics associated with HPV as well as advancement in the targeted therapies for this patient population.
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  • 文章类型: Review
    背景:低级别乳头状Schneiderian癌(LGPSC)是一种相对较新的鼻窦道实体,其特征是模拟鼻窦乳头状瘤的形态学平淡,具有推动边界的侵入性生长模式,以及具有多次复发和转移潜力的侵袭性临床行为。最近,DEK::在LGPSC中鉴定了AFF2融合体。然而,一些LPGSCs缺乏DEK::AFF2融合,这些肿瘤的分子特征尚未明确。
    方法:一名69岁的男子左脸颊流脓。计算机断层扫描显示肿块累及左上颌窦,筛窦,和鼻腔与眶壁的破坏。活检标本显示肿瘤以外生性为主,乳头状生长,没有明显的基质侵入。肿瘤由多层上皮组成,形态温和,呈圆形至多边形,丰富的嗜酸性细胞浆,和统一的原子核。集中存在密集的嗜中性粒细胞浸润。免疫组织化学,CK5/6呈强烈和弥漫性阳性,p16为阴性。p63在基底层主要呈阳性,EMA主要在最外层细胞层表达。基于DNA的靶向测序显示TP53R175H突变,而未发现EGFR和KRAS突变.逆转录聚合酶链反应和荧光原位杂交未发现DEK::AFF2融合。
    结论:我们描述了TP53突变LGPSC的首例病例,并回顾了文献。LGPSC是一个遗传异质性实体,对这种罕见实体的认识以及对临床病理和分子检查结果的全面评估对于正确的病理诊断和临床管理至关重要。
    BACKGROUND: Low-grade papillary Schneiderian carcinoma (LGPSC) is a relatively new entity of the sinonasal tract and is characterized by a bland morphology simulating sinonasal papilloma, invasive growth pattern with pushing borders, and aggressive clinical behavior with multiple recurrences and metastatic potential. Recently, DEK::AFF2 fusions were identified in LGPSC. However, some LPGSCs lack DEK::AFF2 fusion, and the molecular features of these tumors have not been clarified.
    METHODS: A 69-year-old man presented with a discharge of pus from his left cheek. Computed tomography revealed a mass involving the left maxillary sinus, ethmoid sinus, and nasal cavity with the destruction of the orbital wall. The biopsy specimens showed that the tumor had a predominantly exophytic, papillary growth and did not have an apparent stromal invasion. The tumor was composed of multilayered epithelium that showed bland morphology with a round to polygonal shape, abundant eosinophilic cytoplasm, and uniform nuclei. Dense neutrophilic infiltrates were focally present. Immunohistochemically, CK5/6 was strongly and diffusely positive, and p16 was negative. p63 was mainly positive in the basal layer, and EMA was predominantly expressed in the outermost cell layer. DNA-based targeted sequencing showed TP53 R175H mutation, whereas neither EGFR nor KRAS mutation was identified. Reverse transcription polymerase chain reaction and fluorescence in situ hybridization revealed no DEK::AFF2 fusion.
    CONCLUSIONS: We describe the first case of TP53-mutant LGPSC and review the literature. LGPSC is a genetically heterogeneous entity, and the recognition of this rare entity and comprehensive assessment of clinicopathological and molecular findings are crucial for the correct pathological diagnosis and clinical management.
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