mucoepidermoid carcinoma

粘液表皮样癌
  • 文章类型: Journal Article
    目的:探讨头颈部黏液表皮样癌(MEC)患者的预后因素,尤其是治疗方式对生存的影响。
    方法:从监测中获得2000年至2015年的原发性头颈部MEC患者,流行病学,和结束结果(SEER)数据库。与总生存率(OS)和癌症特异性生存率(CSS)相关的预后因素,以及治疗的影响,通过多变量Cox回归分析进行评价。
    结果:我们确定了2692例诊断为头颈部MEC的患者,其中1397人(51.89%)患有腮腺原发性,569人(22.14%)死亡,341例(12.67%)死于MEC。年龄较大(≥53岁),男性,未婚,收入较低,其他头部和颈部区域的肿瘤部位,肿瘤等级更高,肿瘤较大,较高的阶段与较差的OS和CSS有关。未接受手术的患者(HR=3.20,95%CI2.45-4.18)的OS较差,而部分器官切除和全器官切除对患者OS无显著差异(p=0.729)。对于联合治疗,仅接受放疗(HR=3.21,95%CI2.27-4.53)或未接受手术且未接受放疗(HR=2.59,95%CI1.83-3.67)的患者OS较差(vs.仅手术),但仅手术和手术联合放疗对患者的OS无显著差异(p=0.218)。对于CSS,相应结果与OS一致。
    结论:手术切除可能是头颈部MEC更好的生存选择。
    OBJECTIVE: To investigate prognostic factors in patients with head and neck Mucoepidermoid Carcinoma (MEC), especially the impact of treatment modalities on survival.
    METHODS: Patients with primary head and neck MEC between 2000 and 2015 were obtained from the Surveillance, Epidemiology, and End Results (SEER) database. Prognostic factors related to Overall Survival (OS) and Cancer-Specific Survival (CSS), as well as the impact of treatments, were evaluated by multivariable Cox regression analysis.
    RESULTS: We identified 2692 patients diagnosed with head and neck MEC, of whom 1397 (51.89%) had a parotid gland primary, 569 (22.14%) died, and 341 (12.67%) died of MEC. Older age (≥53 years), males, unmarried, lower income, tumor site in other head and neck areas, higher tumor grade, larger tumor size, and higher stage were related to poorer OS and CSS. Patients who did not undergo surgery (HR=3.20, 95% CI 2.45‒4.18) had worse OS, while no significant difference was detected between partial and total organ excision on patients\' OS (p=0.729). For combination therapy, patients who received radiotherapy only (HR=3.21, 95% CI 2.27-4.53) or no surgery and no radiotherapy (HR=2.59, 95% CI 1.83-3.67) were correlated with worse OS (vs. surgery only), but no significant difference was detected between surgery only and surgery combined with radiotherapy on patients\' OS (p=0.218). For CSS, the corresponding results were consistent with OS.
    CONCLUSIONS: Surgical resection only may be a better survival option for head and neck MEC.
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  • 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)通常表现在唾液腺,但是在胰腺中的发生非常罕见。手术切除证明是有效的;然而,胰腺MEC容易转移,缺乏规范的术后治疗。我们讨论了一名51岁的胰腺MEC女性患者接受紫杉醇和吉西他滨作为术后护理的经验。在预定的时间内,该方案成功阻止了转移性肿瘤的扩散,并使肿瘤标志物恢复正常.化疗后6个月内病情稳定。总之,吉西他滨和紫杉醇显示出治疗胰腺MEC的功效。
    Mucoepidermoid carcinoma (MEC) typically manifests in the salivary glands, but occurrences in the pancreatic gland are exceedingly rare. Surgical resection proves effective; however, pancreatic MEC is prone to metastasis, and lacking a standardized postoperative treatment. We discussed the experience of a 51-year-old female patient with pancreatic MEC who received paclitaxel and gemcitabine as postoperative care. Within a predetermined amount of time, this regimen successfully stopped the spread of metastatic tumors and returned tumor markers to normal. A Stable Disease status was achieved within 6 months after chemotherapy. In summary, gemcitabine and paclitaxel display efficacy in treating pancreatic MEC.
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  • 文章类型: Journal Article
    唾液腺癌阳性淋巴结数量的生存意义尚不清楚。因此,本研究旨在确定阳性淋巴结数量对大涎腺cN0黏液表皮样癌(MEC)疾病特异性生存期(DSS)和总生存期(OS)的影响.1975年至2019年期间接受手术治疗的主要唾液腺MEC患者从监测中回顾性登记,流行病学,和最终结果数据库。将总人口随机分为训练组和试验组(1:1)。主要结果变量为DSS和OS。在训练组中,基于使用单变量和多变量Cox分析确定的独立预后因素构建预后模型,并在测试组中使用C指数进行验证。共纳入3317名患者(1624名男性和1693名女性),平均年龄为55±20岁。阳性淋巴结数目是DSS和OS的独立预后因素。但是当发现至少两个DSS阳性淋巴结和三个OS阳性淋巴结时,效果就开始了。训练组DSS和OS的预测模型的C指数分别为0.873(95%置信区间[CI]0.853-0.893)和0.835(95%CI0.817-0.853),分别。测试组的验证显示DSS的C指数为0.877(95%CI0.851-0.902),OS的C指数为0.820(95%CI0.798-0.842)。在cN0大涎腺MEC中,阳性淋巴结的数量与生存率有统计学关联。当前的预后模型可以为患者提供高可靠性的个性化随访策略。
    The survival significance of the number of positive lymph nodes in salivary gland carcinoma remains unclear. Thus, the current study aimed to determine the effect of the number of positive lymph nodes on disease-specific survival (DSS) and overall survival (OS) in cN0 mucoepidermoid carcinoma (MEC) of the major salivary gland. Patients surgically treated for MEC of the major salivary gland between 1975 and 2019 were retrospectively enrolled from the surveillance, epidemiology, and end results database. The total population was randomly divided into training and test groups (1:1). Primary outcome variables were DSS and OS. Prognostic models were constructed based on the independent prognostic factors determined using univariate and multivariate Cox analyses in the training group and were validated in the test group using C-index. A total of 3317 patients (1624 men and 1693 women) with a mean age of 55 ± 20 years were included. The number of positive lymph nodes was an independent prognostic factor for both DSS and OS, but the effect began when at least two positive lymph nodes for DSS and three positive lymph nodes for OS were found. Predictive models for DSS and OS in the training group had C-indexes of 0.873 (95% confidence interval [CI] 0.853-0.893) and 0.835 (95% CI 0.817-0.853), respectively. The validation of the test group showed C-indexes of 0.877 (95% CI 0.851-0.902) for DSS and 0.820 (95% CI 0.798-0.842) for OS. The number of positive lymph nodes was statistically associated with survival in cN0 major salivary gland MEC. The current prognostic model could provide individualized follow-up strategies for patients with high reliability.
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  • 文章类型: Case Reports
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  • 文章类型: Journal Article
    Warthin-like mucoepidermoid carcinoma (MEC) is a recently identified MEC variant of the salivary gland. MEC morphologically mimics Warthin tumor (WT) but harbors the same chromosomal translocation t (11; 19) (q21; p13) as MEC. Thus, differential diagnosis is crucial. MEC involving WT is extremely rare in salivary glands. In this study, we reported a case of Warthin-like MEC, a case of MEC co-existing with WT, and a case of mucinous metaplasia in WT. We also discussed the possible link between WT and MEC.
    Warthin样黏液表皮样癌是一种新近发现的涎腺黏液表皮样癌亚型,形态类似于Warthin瘤,但具有与黏液表皮样癌相似的t(11;19)(q21;p13)染色体易位。因此鉴别诊断非常重要。Warthin瘤合并黏液表皮样癌极为罕见。本文报告了1例Warthin样黏液表皮样癌、1例Warthin瘤合并黏液表皮样癌、1例Warthin瘤伴黏液化生,并讨论了Warthin瘤与黏液表皮样癌之间的可能联系。.
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  • 文章类型: Case Reports
    目的:鼻窦和鼻咽的原发性黏液表皮样癌(MEC)是一种罕见的诊断挑战,尤其是活检样本.在这里,我们提供了一系列的鼻腔鼻窦和颅底MECs及其模拟物,以评估其临床病理和分子特征,以避免误诊。
    方法:我们回顾了2014年至2022年确诊患者的病理记录。在此期间连续诊断出30例MEC。
    结果:基于形态学和荧光原位杂交(FISH)分析,将30例最初诊断为MECs的肿瘤分为MAML2融合阳性(7例)和MAML2融合阴性组(23例),其中14个肿瘤EWSR1::ATF1融合阳性;这些肿瘤被重新分类为透明透明细胞癌(HCCC)。其余9例MAML2FISH阴性病例再次确认为鳞状细胞癌(SCC,3例)显示角质化和Ki-67高表达;DEK::AFF2癌(2例),其中通过FISH检测到DEK基因重排;如前所述的MECs(4例)具有典型的形态学特征。包括7个MAML2重排肿瘤,11例MEC病例的男女比例为4.5:1,6例肿瘤起源于鼻咽区,而5个肿瘤来自鼻窦区域。这一系列唾液腺型MEC的预后良好。
    结论:我们的研究证实,HCCC在鼻窦和鼻咽部存在被误诊为MEC的风险,特别是活检标本。仔细的组织学评估与支持分子测试可以促进病理诊断。
    OBJECTIVE: Primary mucoepidermoid carcinomas (MECs) of the sinonasal tract and nasopharynx are rare entities that represent a diagnostic challenge, especially in biopsy samples. Herein, we present a case series of MECs of the sinonasal and skull base and its mimics to evaluate the clinicopathological and molecular characteristics in order to avoid misdiagnosis.
    METHODS: We reviewed the pathology records of patients diagnosed from 2014 to 2022. Thirty MECs were consecutively diagnosed during that period.
    RESULTS: Based on morphological and fluorescence in situ hybridization (FISH) analyses, 30 tumors originally diagnosed as MECs were separated into MAML2 fusion-positive (7 cases) and MAML2 fusion-negative groups (23 cases), in which 14 tumors were positive for the EWSR1::ATF1 fusion; these tumors were reclassified to have hyalinizing clear cell carcinoma (HCCC). The remaining nine MAML2 FISH negative cases were reconfirmed as squamous cell carcinoma (SCC, 3 cases) which showed keratinization and high Ki-67 expression; DEK::AFF2 carcinomas (2 cases), in which DEK gene rearrangement was detected by FISH; and MECs as previously described (4 cases) with typical morphological features. Including 7 MAML2 rearrangements tumors, 11 MEC cases had a male-to-female ratio of 4.5:1, and 6 tumors arose from the nasopharyngeal region, while 5 tumors arose from the sinonasal region. The prognosis of this series of salivary gland-type MECs was favorable.
    CONCLUSIONS: Our study confirmed that HCCC runs the risk of being misdiagnosed as MEC in the sinonasal tract and nasopharynx, particularly with biopsy specimens. Careful histological evaluation with supporting molecular testing can facilitate pathological diagnoses.
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  • 文章类型: Journal Article
    乳腺粘液表皮样癌(MEC)是一种罕见的实体。由于乳腺MEC的稀有性,其分子特征尚未得到充分研究。我们对1000例乳腺癌患者进行了回顾性研究,并确定了4例乳腺MEC。收集临床和人口统计学数据。还进行了用于诊断唾液腺MEC和乳腺癌的免疫组织化学面板。通过FISH检测MAML2重排,并通过RNA测序鉴定融合配偶体。使用全外显子组测序(WES)来揭示这四个乳腺MEC的基因组。然后,进一步比较了乳腺MEC与浸润性乳腺癌和唾液腺MEC的生物学功能和特征。根据Ellis和Auclair的方法,这4例乳腺MEC可归类为低度乳腺MEC.所有的病人都还活着,无病生存期(PFS)为20个月至67个月。在这四个乳房MEC中,两例为三阴性,另外两例被发现是ER阳性,通过免疫组织化学染色也显示HER2模棱两可,但在鱼中没有扩增。FISH分析证实在四个肿瘤中的三个肿瘤中存在MAML2易位,和CRTC1-MAML2融合在其中两个通过RNA测序得到证实。用于肿瘤突变负荷估计的WES的平均覆盖大小为32Mb。在至少三种肿瘤中发现MUC4、RP1L1和QRICH2突变,这些突变也存在于乳腺浸润性癌数据库中(TCGA,Cell2015;TCGA,《自然》2012年)。结果显示,乳腺MEC中有许多基因与上述乳腺浸润性癌数据库重叠,范围从5到63个基因(中位数:21个基因)。接下来,我们评估了这些肿瘤的免疫细胞浸润水平.在所有这些肿瘤中,M2巨噬细胞和浆细胞均为高浸润组。我们的乳腺MEC显示出与唾液腺MEC不同的结果,其浆细胞处于低浸润组。总的来说,我们首先分析了乳腺粘液表皮样癌的基因组学和肿瘤微环境,并提出了我们的假设,即尽管在乳腺中产生的MEC在表型上类似于其唾液腺对应物,我们的研究结果表明,乳腺MECs在遗传水平和免疫细胞浸润水平上可能与浸润性乳腺癌相似.更多的病例和在深刻的研讨中须要进一步懂得这一罕见癌。
    Mammary mucoepidermoid carcinoma (MEC) is a rare entity. The molecular characteristics of breast MEC have not been fully investigated due to its rarity. We performed a retrospective study among 1000 patients with breast carcinomas and identified four cases of breast MEC. Clinical and demographic data were collected. Immunohistochemistry panels which were used to diagnose salivary gland MEC and breast carcinomas were also performed. MAML2 rearrangements were detected by FISH and fusion partners were identified by RNA sequencing. Whole-exome sequencing (WES) was used to reveal the genomes of these four breast MEC. Then, the biological functions and features of breast MEC were further compared with those of invasive breast carcinomas and salivary gland MEC.According to Ellis and Auclair\'s methods, these four breast MEC could be classified as low-grade breast MEC. All the patients were alive, and disease-free survival (PFS) ranged from 20 months to 67 months. Among these four breast MEC, two cases were triple-negative, and the other two cases were found to be ER positive, with one also showing HER2 equivocal by immunohistochemical staining, but no amplification in FISH. FISH analysis confirmed the presence of the MAML2 translocation in three of four tumors, and CRTC1-MAML2 fusion was confirmed in two of them by RNA-sequencing. The average coverage size of WES for the tumor mutation burden estimation was 32 Mb. MUC4, RP1L1 and QRICH2 mutations were identified in at least three tumors, and these mutation also existed in breast invasive carcinoma databases (TCGA, Cell 2015; TCGA, Nature 2012). The results showed that there were many genes in breast MEC overlapping with the breast invasive carcinoma databases mentioned above, range from 5 to 63 genes (median:21 genes). Next, we assessed immune cell infiltration levels in these tumors. In all these tumors, M2 macrophages and plasma cell were in the high infiltration group. Our breast MEC showed different results from the salivary gland MEC, whose plasma cells were in the low infiltration group. Overall, we first analyzed the genomics and tumor microenvironment of breast mucoepidermoid carcinoma and proposed our hypothesis that although MECs arising in the breast resemble their salivary gland counterparts phenotypically, our findings indicate that breast MECs probably resemble invasive breast carcinomas at the genetic level and immune cell infiltration levels. More cases and in deep research need to be done to further understand this rare carcinoma.
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