mucoepidermoid carcinoma

粘液表皮样癌
  • 文章类型: Case Reports
    Warthin样粘液表皮样癌(WL-MEC)是新近报道的粘液表皮样癌的变体。它的组织学特征容易与化生沃辛肿瘤混淆,其与Warthin肿瘤组织发生的关系存在争议。在这项研究中,我们提出了两例WL-MEC,讨论他们的临床病理和分子特征。值得注意的是,1例最初在肿瘤首次发作期间被误诊。病例1是一名60岁的女性,右侧腮腺有肿块。病例2包括一名29岁的男性,他在下颌下腺切除“Warthin肿瘤”6个月后在原始手术部位出现肿块。组织学上,两个肿瘤都表现出突出的淋巴间质和囊性模式,伴随着由鳞片状细胞组成的各种数量的上皮巢,中间细胞和粘液细胞。两种情况下都没有典型地出现Warthin肿瘤的特征性嗜酸性双层上皮。两个病例的MAML2基因重排检测呈阳性。为了了解我们的发现,我们对英语文献中记录的48例WL-MEC病例进行了全面审查,旨在综合可靠的鉴别诊断方法。WL-MEC是一种罕见但临床相关的变体,给病理学家带来一个诊断陷阱。我们的研究强调了对临床和组织学特征进行细致评估的重要性。再加上MAML2重排的检测,作为区分WL-MEC与其他良性和恶性病变的可靠方法,特别是化生Warthin肿瘤.
    Warthin-like mucoepidermoid carcinoma (WL-MEC) is a newly reported variant of mucoepidermoid carcinoma. Its histological feature is easy to confused with metaplastic Warthin Tumor, and its relationship with Warthin tumor in histogenesis is controversial. In this study, we presented two cases of WL-MEC, discussing their clinicopathological and molecular features. Notably, one case was initially misdiagnosed during the first onset of the tumor. Case 1 was a 60-year-old female with a mass in the right parotid gland. Case 2 featured a 29-year-old male who developed a lump at the original surgical site 6 months after a \"Warthin tumor\" resection from the submandibular gland. Histologically, both tumor exhibited a prominent lymphoid stroma and cystic pattern, accompanied by various amounts of epithelial nests composed of squamoid cells, intermediate cells and mucinous cells. The characteristic eosinophilic bilayer epithelium of Warthin tumor was not typically presented in either case. Both cases tested positive for MAML2 gene rearrangement. To contextualize our findings, we conducted a comprehensive review of forty-eight WL-MEC cases documented in the English literature, aiming to synthesizing a reliable differential diagnostic approach. WL-MEC is a rare yet clinically relevant variant, posing a diagnostic pitfall for pathologists. Our study underscores the importance of a meticulous evaluation of both clinical and histological features, coupled with the detection of MAML2 rearrangement, as a credible method for distinguishing WL-MEC from other benign and malignant lesions, particularly metaplastic Warthin tumor.
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  • 文章类型: Letter
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  • 文章类型: Journal Article
    我们的目标是评估儿童/青少年癌症病史对腮腺粘液表皮样癌(MEC)患者总生存期(OS)和疾病特异性生存期(DSS)的影响。对原发腮腺MEC接受手术治疗的患者和患有腮腺MEC第二恶性肿瘤的患者进行回顾性分析。流行病学,和结束结果(SEER)数据库。主要结果变量是OS和DSS。使用Cox回归模型分析与癌症病史相关的这些生存率的风险比(HR)。总的来说,纳入2681例患者,其中263人患有第二恶性肿瘤。原发性恶性肿瘤组(72%)和第二恶性肿瘤组(59%)的10年OS率存在显着差异。Cox回归证实,癌症史倾向于降低OS(p=0.062,HR:1.28,95%置信区间:0.99至1.64)。亚组分析显示,与血液癌症相比,实体瘤病史预示着OS恶化,中枢神经系统肿瘤比其他肿瘤表现出更显著的影响(p=0.030vsp=0.088)。癌症病史与DSS无关。儿童/青少年癌症病史对腮腺MEC患者的预后有负面影响,这种影响主要是由实体恶性肿瘤病史引起的。
    Our goal was to assess the impact of childhood/adolescent cancer history on overall survival (OS) and disease-specific survival (DSS) in patients with parotid mucoepidermoid carcinoma (MEC). Patients who underwent surgical treatment for primary parotid MEC and those with a second malignancy of parotid MEC were retrospectively identified from the Surveillance, Epidemiology, and End Results (SEER) database. The primary outcome variables were OS and DSS. The hazard ratios (HRs) of these survival rates associated with cancer history were analysed using Cox regression models. In total, 2681 patients were included, 263 of whom had a second malignancy. The 10-year OS rates in the primary (72%) and second malignancy groups (59%) were significantly different. Cox regression confirmed that a history of cancer tended to decrease OS (p = 0.062, HR: 1.28, 95% confidence interval: 0.99 to 1.64). Subgroup analyses showed that a history of solid tumour as opposed to haematological cancer predicted worse OS, with central nervous system tumours exhibiting a more significant influence than others (p = 0.030 vs p = 0.088). Cancer history was not related to DSS. A history of childhood/adolescent cancer negatively influenced the prognosis of patients with parotid MEC, and this effect was primarily driven by a history of solid malignancy.
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  • 文章类型: Case Reports
    粘液表皮样癌(MEC)是一种通常由腮腺引起的唾液腺肿瘤。MEC有各种症状,包括无痛的,在耳叶下面或前面缓慢生长的肿块。然而,不寻常的表现也可以是耳后肿胀的形式。耳后肿胀的其他更常见的良性差异包括淋巴结病,表皮样囊肿,还有脂肪瘤.因此,仅根据临床表现将耳廓后肿胀诊断为MEC具有挑战性,高度怀疑,以及具有各种放射学研究的多学科方法,例如计算机断层扫描(CT)和磁共振成像(MRI),需要与组织病理学评估合作才能准确诊断这种恶性肿瘤。预后取决于各种因素,包括肿瘤的分级,病人的年龄,和合并症,以及诊断时的阶段。早期诊断和手术干预是治疗的支柱,之后可以根据恶性肿瘤的分期进行辅助放疗。这是一个患者出现耳后肿胀的报告,最初被误诊为良性坏死淋巴结。经过进一步评估,它被发现是腮腺粘液表皮样癌,通过手术切除和放射治疗。
    Mucoepidermoid carcinoma (MEC) is a salivary gland tumor commonly arising from the parotid gland. MEC has various presenting symptoms, including a painless, slow-growing mass below or anterior to the ear lobule. However, an unusual presentation can also be in the form of post-auricular swelling. Other more common benign differentials for post-auricular swelling include lymphadenopathy, epidermoid cysts, and lipomas. Thus, diagnosing a postauricular swelling as MEC solely based on clinical presentation is challenging, and a high suspicion, as well as a multidisciplinary approach with various radiological investigations such as computed tomography (CT) and magnetic resonance imaging (MRI), are required in collaboration with histopathological assessment for an accurate diagnosis of this malignancy. Prognosis depends on various factors, including the grade of the tumor, the patient\'s age, and comorbidities, as well as the stage at the time of diagnosis. Early diagnosis and surgical intervention are the mainstays of treatment, which can be followed by adjuvant radiotherapy based on the stage of the malignancy. This is a report of a patient who presented with post-auricular swelling, which was initially misdiagnosed as a benign necrotic lymph node. After further evaluation, it was found to be a mucoepidermoid carcinoma of the parotid gland, which was managed by surgical excision and radiotherapy.
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  • 文章类型: Journal Article
    背景与目的:粘蛋白通过多种机制参与肿瘤细胞的发育和生长。然而,唾液腺肿瘤中粘蛋白表达的研究是有限的,尤其是小唾液腺的样本。这项研究旨在调查和比较粘蛋白在次要和主要唾液腺起源的良性和恶性唾液腺肿瘤中的表达。材料和方法:使用特殊染色剂对中性粘蛋白(高碘酸希夫)进行染色,来自六个正常唾液腺和73个唾液腺肿瘤的组织内的唾液酸粘蛋白(AlcianBlue)和磺粘蛋白(AldehideFuschin),包括31个多形性腺瘤,27例粘液表皮样癌,和15例腺样囊性癌.半定量方法用于评估导管腔内的粘蛋白表达。唾液酸粘蛋白是所有唾液腺肿瘤中表达最多的粘蛋白,不管起源。结果:良性和恶性唾液腺肿瘤之间的粘蛋白表达存在显着差异,与粘液表皮样癌和腺样囊性癌相比,多形性腺瘤显示唾液粘蛋白的表达明显高出三倍(p=0.028)。与次要腺体相比,主要腺体的多形性腺瘤显示出42倍的唾液酸粘表达(p=0.000)。结论:与小腺体相比,大腺体多形性腺瘤中的唾液酸蛋白含量大大增加。唾液粘蛋白在良性和恶性唾液腺肿瘤中的差异表达表明在诊断交界性唾液腺肿瘤中的作用。
    Background and Objectives: Mucin has been implicated via various mechanisms in the development and growth of tumour cells. However, mucin expression studies in salivary gland tumours are limited, especially with samples from minor salivary glands. This study aims to investigate and compare mucin expression in benign and malignant salivary gland tumours of minor and major salivary gland origins. Materials and Methods: Special stains were used to stain neutral mucin (Periodic acid Schiff), sialomucin (Alcian Blue) and sulfomucin (Aldehyde Fuschin) within tissues from six normal salivary glands and 73 salivary gland tumours including 31 pleomorphic adenomas, 27 mucoepidermoid carcinomas, and 15 adenoid cystic carcinomas. A semi-quantitative approach was used to evaluate mucin expression within ductal lumens. Sialomucin was the most expressed mucin in all salivary gland tumours, regardless of origin. Results: A significant difference was observed in the mucin expression between benign and malignant salivary gland tumours, as pleomorphic adenoma showed three times significantly higher expression of sialomucin compared to mucoepidermoid carcinoma and adenoid cystic carcinoma (p = 0.028). Pleomorphic adenomas of major glands showed 42 times significantly higher expression of sialomucin compared to those of minor glands (p = 0.000). Conclusions: Sialomucin content in pleomorphic adenomas of major glands was vastly increased compared to that in minor glands. Differential sialomucin expression in benign and malignant salivary gland tumours suggests a role in diagnosing of borderline salivary gland tumours.
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  • 文章类型: Case Reports
    背景:涎腺型肺癌是罕见的肺部肿瘤,占所有肺部肿瘤的不到1%。其中最常见的两种是腺样囊性癌和粘液表皮样癌。虽然他们通常有懒惰的行为,腺样囊性癌可能更具侵袭性,5年生存率低至55%。文献中报道的病例很少。我们报告了类似的唾液腺型肺癌罕见病例,该病例首次出现左半胸单侧混浊。
    方法:一名38岁的北印度裔男子,他是一个不吸烟的人,出现呼吸急促和咳嗽1年的投诉,在过去2个月中有所增加,并与显着的体重减轻有关。进行了胸部的额叶X光片和胸部的计算机断层扫描,显示左肺上叶有肿块,其中心位于左主支气管。进行了支气管镜引导活检,病理证实诊断为涎腺型肺癌(腺样囊性癌)。主要船只入侵,因此,患者被提供并开始接受姑息治疗,而不是手术治疗。尽管化疗和放疗两个周期的姑息治疗,患者在诊断后2个月内死于该疾病。
    结论:涎腺型肺癌(尤其是腺样囊性癌)通常在晚期出现。肿瘤的可切除性取决于周围主要血管的受累。有趣的是,这些癌症与吸烟无关。预后取决于诊断时的疾病程度。因此,影像学在决定进一步的管理计划中起着重要作用。
    BACKGROUND: Salivary gland-type lung carcinomas are uncommon neoplasms of the lung, representing less than 1% of all lung tumors. The two most common among them are adenoid cystic carcinoma and mucoepidermoid carcinoma. Although they usually have an indolent behavior, adenoid cystic carcinomas can be more aggressive, with 5-year survival as low as 55%. Very few cases are reported in literature. We report a similar rare case of salivary gland type lung carcinoma that presented for the first time with unilateral opacification of left hemithorax.
    METHODS: A 38-year-old man of North Indian origin, who was a a nonsmoker, presented with complaints of shortness of breath and cough for 1 year, which has increased in the last 2 months and was associated with significant weight loss. A frontal radiograph of the chest and computed tomography of the chest were performed, which showed a mass in the left upper lobe of the lung with its epicenter in the left main bronchus. A bronchoscopic guided biopsy was performed, and histopathology confirmed the diagnosis of lung carcinoma of salivary gland type (adenoid cystic carcinoma). There was invasion of major vessels, hence the patient was offered and started on palliative management instead of surgical treatment. In spite of palliative management of two cycles of chemotherapy and radiotherapy, the patient succumbed to the disease within 2 months from the time of diagnosis.
    CONCLUSIONS: Lung carcinoma of the salivary gland type (especially adenoid cystic carcinoma) usually presents at a later stage. The resectability of the tumor depends on the involvement of the surrounding major vessels. Interestingly, these cancers have no association with smoking. The prognosis depends on the extent of the disease at the time of diagnosis. Hence, imaging plays a major role in deciding the further plan of management.
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  • 文章类型: Journal Article
    肝胆粘液表皮样癌是一种罕见的恶性肿瘤,包括粘液,中间,和表皮样细胞。在这里,我们介绍了1例原发性肝黏液表皮样癌术前误诊为常规肝内胆管癌。一名67岁男性入院。术前实验室检查显示天冬氨酸转氨酶升高,丙氨酸转氨酶,和糖类抗原19-9.腹部计算机断层扫描显示VI段有4.8×4.9cm的肝脏肿块。初步诊断为肝内胆管癌,接受部分肝切除术。然而,在组织病理学上,肿瘤由表皮样混合物组成,粘液,和中间细胞在肿瘤边缘弥漫性浸润。在特殊污渍上,粘液细胞和中间细胞对粘液碱和阿尔辛蓝呈阳性,而表皮样细胞对角蛋白5/6和p63呈阳性。中间细胞对p63也是阳性的。所有肿瘤细胞对角蛋白7呈阳性。Ki-67指数为35%。最终诊断为原发性肝粘液表皮样癌。虽然罕见,肝黏液表皮样癌在肝内胆管癌的鉴别诊断中应考虑。我们回顾了以前的研究,发现肝胆粘液表皮样癌更可能起源于肿瘤附近的胆道。
    Hepatobiliary mucoepidermoid carcinoma is a rare malignant tumor comprising mucous, intermediate, and epidermoid cells. Herein, we presented a case of primary liver mucoepidermoid carcinoma preoperatively misdiagnosed as conventional intrahepatic cholangiocarcinoma. A 67-year-old male was admitted to our hospital. Preoperative laboratory tests showed increased aspartate transaminase, alanine transaminase, and carbohydrate antigen 19-9. Abdominal Computer Tomography revealed a 4.8 × 4.9 cm liver mass in segment VI. A preliminary diagnosis of intrahepatic cholangiocarcinoma was made, with undergoing partial hepatectomy. However, on histopathology, the tumor comprised a mixture of epidermoid, mucous, and intermediate cells with diffuse infiltrating at the tumor margin. On special stains, mucous and intermedia cells were positive for mucicarmine and Alcian blue, whereas epidermoid cells were positive for Keratin 5/6 and p63. Intermediate cells are also positive for p63. All tumor cells were positive for Keratin 7. The Ki-67 index was 35%. The final diagnosis was primary hepatic mucoepidermoid carcinoma. Although rare, hepatic mucoepidermoid carcinoma should be considered in the intrahepatic cholangiocarcinoma differential diagnosis. We reviewed previous studies and found that hepatobiliary mucoepidermoid carcinoma is more likely to originate from the biliary tract adjacent to the tumor.
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  • 文章类型: Journal Article
    涎腺肿瘤相对罕见,可以表现出各种临床行为。这项研究旨在了解自然史,病理学,诊断检查,和这些肿瘤的治疗策略,以改善患者的预后。审核包括通过放射学或细胞学检查发现的唾液腺肿瘤患者。患者接受了手术,一些人接受辅助治疗。人口统计信息,治疗干预措施,采用SPSS软件分析生存结局。总共有89例恶性唾液腺肿瘤被审计恶性肿瘤主要在腮腺发现,在小唾液腺和下颌下腺的病例较少。就诊年龄中位数为47岁,大多数患者是男性。该研究检查了各种病理和临床因素,包括肿瘤分期,节点状态,和面部麻痹的存在。记录了手术程序和肿瘤的组织学类型。不利的组织学特征,如阳性边缘,淋巴结阳性,淋巴管浸润,囊外扩散,并注意到神经周浸润。对高危患者进行术后RT。大多数恶性唾液腺肿瘤见于腮腺,而小唾液腺肿瘤在审计中的代表性不足。外科手术方式多种多样。放射治疗方案相对标准化。研究发现某些组织学特征,如淋巴结阳性,边缘积极性,淋巴管浸润,神经周浸润,囊外扩散,与DFS和OS的不良反应相关。研究结果表明,特定的组织学特征,包括LVI和ECE已成为DFS和OS的独立预后因素。
    Salivary gland tumors are relatively rare and can exhibit various clinical behaviors. The study aims to understand the natural history, pathology, diagnostic workup, and treatment strategies for these tumors to improve patient outcomes. The audit included patients with salivary gland tumors detected through radiology or cytology. Patients underwent surgery, with some receiving adjuvant treatment. Demographic information, treatment interventions, and survival outcomes were analyzed using SPSS software. A total 89 as malignant salivart gland tumours were audited Malignant tumors were predominantly found in the parotid gland, with fewer cases in the minor salivary gland and submandibular gland.The median age of presentation was 47 years, and the majority of patients were male. The study examined various pathological and clinical factors, including tumor stage, nodal status, and the presence of facial palsy. Surgical procedures and histological types of tumors were documented. Adverse histological features like positive margins, lymph node positivity, lympho-vascular invasion, extracapsular spread, and perineural invasion were noted. POSTOP RT was administered to high-risk patients. Most malignant salivary gland tumors were found in the parotid gland, while minor salivary gland tumors were underrepresented in the audit. Surgical practices were diverse. Radiotherapy protocols were relatively standardized. The study found that certain histological features, such as lymph node positivity, margin positivity, lympho-vascular invasion, perineural invasion, and extracapsular spread, were associated with adverse effects on DFS and OS. The findings suggest that specific histological features, including LVI and ECE have emerged as independent prognostic factors for DFS and OS.
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  • 文章类型: Case Reports
    粘液表皮样癌是一种起源于唾液腺的恶性肿瘤。推荐的治疗策略通常包括手术干预,有时辅以放射治疗,取决于肿瘤的组织学分级。描述了一例没有病史的22岁女性患者。临床检查显示位于硬腭上的蓝色病变。组织学检查证实了低度粘液表皮样癌的诊断。切除病变,并立即通过假体闭塞器关闭口鼻连通,然后通过旋转pa瓣关闭。患者随访12个月,没有任何复发的证据.本文强调了及时临床诊断此类病变的重要性,并提供了一个机会来回顾这些癌症治疗措施以降低术后发病率。
    Mucoepidermoid carcinoma is a malignant tumor that arises from the salivary glands. The recommended treatment strategy typically involves surgical intervention, sometimes complemented by radiotherapy, depending on the histological grade of the tumor. A case of a 22-year-old female patient without medical history was described. The clinical examination revealed a bluish lesion located on the hard palate. The histological examination confirmed the diagnosis of a low-grade mucoepidermoid carcinoma. Resection of the lesion was performed and oro-nasal communication was immediately closed by a prosthetic obturator and later on by a rotational palate flap. The patient was followed up for 12 months, and there was no evidence of any recurrence. This article highlights the importance of prompt clinical diagnosis of such lesions and provides an opportunity to review these cancer therapeutic measures to reduce postoperative morbidity.
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  • 文章类型: Journal Article
    以大量粘蛋白产生为特征的唾液腺肿瘤很少见,但早已得到认可。由于它们的稀缺性,精确分类长期以来一直困扰着这些富含粘蛋白的肿瘤。最近的分子发现,然而,对富含粘蛋白的唾液腺肿瘤的遗传基础有了相当大的了解。本手稿将回顾有关这组迷人的唾液腺肿瘤的最新信息。
    Salivary gland neoplasms characterized by abundant mucin production are rare but have long been recognized. Due to their scarcity, precise classification has long eluded these mucin-rich tumors. Recent molecular discoveries, however, have shed considerable light on the genetic underpinnings of mucin-rich salivary gland neoplasms. This manuscript will review the most up-to-date information on this fascinating group of salivary gland neoplasms.
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