Carcinoma, Medullary

癌,髓质
  • 文章类型: Journal Article
    甲状腺髓样癌(MTC)仅占所有甲状腺癌的3%:在2型多发性内分泌瘤(MEN2)的背景下,75%为散发性MTC(sMTC),25%为遗传性MTC(hMTC)。通过明确结节性甲状腺肿时确定肿瘤标志物降钙素(Ctn)并检测MEN2家族中原癌基因RET的突变,可以进行早期诊断。如果Ctn水平只是稍微升高,女性高达30pg/ml,男性高达60pg/ml,建议进行后续检查。在更高的水平,应考虑手术;在>100pg/ml的水平下,手术总是明智的。选择的治疗方法是全甲状腺切除术,可能是中央淋巴结清扫术.在早期阶段,通过适当的手术可以治愈;在晚期,用酪氨酸激酶抑制剂治疗是一种选择。应对所有MTC患者进行RETA突变分析。随访期间,生化区分为:治愈的(Ctn不是可测量的低),生化不完全(Ctn增加而没有肿瘤检测)和结构性肿瘤检测(成像转移)。MTC手术后,以下结果应可用于后续护理分类:(I)组织学,Ctn免疫组织学,如有必要,(ii)根据pTNM方案进行分类,(iii)RET分析的结果,用于分类为遗传性或散发性变体和(iiii)术后Ctn值。通过评估Ctn倍增时间和RECIST成像标准来确定肿瘤进展。在大多数情况下,“主动监视”是可能的。在进展和症状的情况下,以下情况适用:局部(姑息性手术,放疗)在全身(酪氨酸激酶抑制剂)之前。
    Medullary thyroid carcinoma (MTC) accounts for only 3% of all thyroid carcinomas: 75% as sporadic MTC (sMTC) and 25% as hereditary MTC (hMTC) in the context of multiple endocrine neoplasia type 2 (MEN2). Early diagnosis is possible by determining the tumour marker calcitonin (Ctn) when clarifying nodular goitre and by detecting the mutation in the proto-oncogene RET in the MEN2 families. If the Ctn level is only slightly elevated, up to 30 pg/ml in women and up to 60 pg/ml in men, follow-up checks are advisable. At higher levels, surgery should be considered; at a level of > 100 pg/ml, surgery is always advisable. The treatment of choice is total thyroidectomy, possibly with central lymphadenectomy. In the early stage, cure is possible with adequate surgery; in the late stage, treatment with tyrosine kinase inhibitors is an option. RET A mutation analysis should be performed on all patients with MTC. During follow-up, a biochemical distinction is made between: healed (Ctn not measurably low), biochemically incomplete (Ctn increased without tumour detection) and structural tumour detection (metastases on imaging). After MTC surgery, the following results should be available for classification in follow-up care: (i) histology, Ctn immunohistology if necessary, (ii) classification according to the pTNM scheme, (iii) the result of the RET analysis for categorisation into the hereditary or sporadic variant and (iiii) the postoperative Ctn value. Tumour progression is determined by assessing the Ctn doubling time and the RECIST criteria on imaging. In most cases, \"active surveillance\" is possible. In the case of progression and symptoms, the following applies: local (palliative surgery, radiotherapy) before systemic (tyrosine kinase inhibitors).
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    Medullary thyroid carcinoma (MTC) is a neuroendocrine tumor originating from the parafollicular cells (C cells) of the thyroid gland, classified as sporadic and hereditary. Calcitonin (Ctn) secreted by the C cells is a specific serological marker for MTC, which is of great value in diagnosis, treatment and postoperative management of MTC. The effect of chemoradiotherapy and 131I therapy on MTC is limited, with surgery being the primary therapy. Given the aggressive nature and relatively poor prognosis of MTC, the reasonable surgical extent is crucial for improving cure rate and prognosis of patients. However, there are still some controversies regarding the extent of surgery for MTC. This article elaborates on the research progress and controversies of serum Ctn levels in assisting the evaluation of the extent of surgery for MTC.
    甲状腺髓样癌(MTC)是一种起源于甲状腺滤泡旁细胞(C细胞)的神经内分泌肿瘤,分为散发性和遗传性。C细胞分泌的降钙素(Ctn)是MTC特异性的血清学标志物,在MTC的诊断、治疗及术后管理中具有重要价值。放化疗及131I对MTC的治疗效果十分有限,手术为目前MTC的主要治疗方式。MTC侵袭性较强、预后相对较差,因此,合理的手术范围对于提高患者的治愈率及改善预后具有重要意义。然而,目前关于MTC的手术范围仍存在部分争议,现就血清Ctn水平在协助评估MTC手术范围的研究进展及争议进行阐述。.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    结肠髓样癌(MCC)是结肠癌的一种罕见的组织学亚型,目前还没有公认的最佳治疗方案,其预后仍不清楚。这项研究的目的是分析MCC患者的独立预后因素,并开发和验证列线图以预测总生存期(OS)。从监测中选择2004年至2020年新诊断为MCC的760例患者,流行病学,和结束结果(SEER)数据库。所有患者以7:3的比例随机分配到训练组和验证组。进行单变量和多变量Cox回归分析以确定预后因素并构建列线图。使用受试者工作特征(ROC)曲线评估和验证了列线图预测模型,校正曲线,和决策曲线分析(DCA)。研究发现,老年女性更容易患MCC,升结肠和盲肠是最常见的受累部位。MCC分化差,第二阶段和第三阶段是最常见的。手术是MCC的主要治疗方法。IV期MCC患者的预后较差,中位生存时间仅为10个月。MCC的独立预后因素包括年龄,N级,M阶段,手术,化疗,和肿瘤大小。其中,年龄<75岁和完成化疗是结肠癌的保护因素,而N2(HR=2.18,95CI1.40-3.38),M1(HR=3.31,95CI2.01-5.46),无手术(HR=27.94,95CI3.69-211.75),肿瘤直径>7cm(HR=1.66,95CI1.20-2.30)是结肠癌的危险因素。ROC的结果,AUC,校正曲线,和DCA表明,列线图预测模型具有良好的预测性能。我们更新了结肠髓样癌的人口统计学特征,并确定了年龄,N分期,M分期,手术,化疗和肿瘤大小是结肠髓样癌的独立预后因素。此外,我们建立了预测预后的列线图.这些列线图可以提供个性化的预测,并为临床决策提供有价值的参考。
    Medullary Carcinoma of the Colon (MCC) is a rare histological subtype of colon cancer, and there is currently no recognized optimal treatment plan for it, with its prognosis remaining unclear. The aim of this study is to analyze the independent prognostic factors for MCC patients and develop and validate nomograms to predict overall survival (OS). A total of 760 patients newly diagnosed with MCC from 2004 to 2020 were selected from the Surveillance, Epidemiology, and End Results (SEER) database. All patients were randomly allocated to a training group and a validation group in a 7:3 ratio. Univariate and multivariable Cox regression analyses were conducted to identify prognostic factors and construct nomograms. The nomogram prediction model was evaluated and validated using receiver operating characteristic (ROC) curves, calibration curves, and decision curve analysis (DCA). The study found that elderly women are more susceptible to MCC, and the ascending colon and cecum are the most common sites of involvement. MCC is poorly differentiated, with stages II and III being the most common. Surgery is the primary treatment for MCC. The prognosis for patients with stage IV MCC is poor, with a median survival time of only 10 months. Independent prognostic factors for MCC include age, N stage, M stage, surgery, chemotherapy, and tumor size. Among them, age < 75 years and completion of chemotherapy were protective factors for colon medullary carcinoma, while N2 (HR = 2.18, 95%CI 1.40-3.38), M1 (HR = 3.31, 95%CI 2.01-5.46), no surgery (HR = 27.94, 95%CI 3.69-211.75), and tumor diameter > 7 cm (HR = 1.66, 95%CI 1.20-2.30) were risk factors for colon medullary carcinoma. The results of ROC, AUC, calibration curves, and DCA demonstrate that the nomogram prediction model exhibits good predictive performance. We have updated the demographic characteristics of colon medullary carcinoma and identified age, N staging, M staging, surgery, chemotherapy and tumor size as independent prognostic factors for colon medullary carcinoma. Additionally, we have established nomograms for prognostic prediction. These nomograms can provide personalized predictions and serve as valuable references for clinical decision-making.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    结直肠癌的不同病理类型具有不同的免疫景观,免疫疗法的疗效将完全不同。结直肠髓样癌,占2.2-3.2%,以大量淋巴细胞浸润为特征。然而,对结直肠髓样癌免疫特性的关注不足。
    我们搜索了截止到2030年11月在PubMed上有关结直肠髓样癌的文献,以研究结直肠髓样癌的免疫景观的特征,比较不同器官来源的髓样癌,为精准治疗提供理论依据,包括应用免疫疗法和BRAF抑制剂。
    结直肠髓样癌是一种具有强烈免疫反应的病理亚型,具有六种免疫特性,并有可能从免疫疗法中受益。不匹配修复缺陷,经常发生ARID1A缺失和BRAFV600E突变。IFN-γ途径被激活,PD-L1表达增加。丰富的淋巴细胞浸润发挥肿瘤杀伤功能。此外,BRAF突变在其发生、发展中起着重要作用,我们可以考虑联合BRAF抑制剂和免疫治疗BRAF突变患者。结直肠髓样癌的探索将引起研究者对病理亚型与免疫反应的相关性的关注,推进精准免疫治疗的进程。
    UNASSIGNED: Different pathological types of colorectal cancer have distinguished immune landscape, and the efficacy of immunotherapy will be completely different. Colorectal medullary carcinoma, accounting for 2.2-3.2%, is characterized by massive lymphocyte infiltration. However, the attention to the immune characteristics of colorectal medullary carcinoma is insufficient.
    UNASSIGNED: We searched the literature about colorectal medullary carcinoma on PubMed through November 2023to investigate the hallmarks of colorectal medullary carcinoma\'s immune landscape, compare medullary carcinoma originating from different organs and provide theoretical evidence for precise treatment, including applying immunotherapy and BRAF inhibitors.
    UNASSIGNED: Colorectal medullary carcinoma is a pathological subtype with intense immune response, with six immune characteristics and has the potential to benefit from immunotherapy. Mismatch repair deficiency, ARID1A missing and BRAF V600E mutation often occurs. IFN-γ pathway is activated and PD-L1 expression is increased. Abundant lymphocyte infiltration performs tumor killing function. In addition, BRAF mutation plays an important role in the occurrence and development, and we can consider the combination of BRAF inhibitors and immunotherapy in patients with BRAF mutant. The exploration of colorectal medullary carcinoma will arouse researchers\' attention to the correlation between pathological subtypes and immune response, and promote the process of precise immunotherapy.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    背景:甲状腺髓样癌(MTC)是一种罕见的恶性肿瘤,预后不良。由于竞争风险的影响,MTC预后因素分析可能存在偏倚。
    方法:通过提取监测中登记的MTC诊断患者的数据,流行病学,在1998年至2016年的最终结果(SEER)数据库中,我们建立了Cox比例风险和竞争风险模型,回顾性分析了相关因素对淋巴结的影响。
    结果:共有2,435名患者被纳入分析,其中198人死于MTC。多因素竞争风险模型的结果表明,总淋巴结数(19-89),阳性淋巴结(1-10,11-75)和阳性淋巴结比率(25%-53%,>54%),年龄(46-60,>61),化疗,放疗模式(其他),肿瘤大小(2-4cm,>4cm),病灶数大于1是MTC的不良预后因素.对于总淋巴结数,与多元Cox比例风险模型的结果不同,我们发现,在排除竞争性风险因素后,它成为独立的风险因素。竞争性危险因素对阳性淋巴结数量影响不大。对于阳性淋巴结的比例,我们发现,在排除竞争风险因素后,Cox比例风险模型高估了其对预后的影响。竞争风险模型在分析预后因素的影响时通常更准确。
    结论:排除竞争风险后,淋巴结的数量,阳性数量和阳性比例是甲状腺髓样癌的不良预后因素,有助于临床医生更准确地评估甲状腺髓样癌患者的预后,为治疗决策提供参考依据。
    Medullary thyroid carcinoma (MTC) is an infrequent form malignant tumor with a poor prognosis. Because of the influence of competitive risk, there may suffer from bias in the analysis of prognostic factors of MTC.
    By extracting the data of patients diagnosed with MTC registered in the Surveillance, Epidemiology, and End Results (SEER) database from 1998 to 2016, we established the Cox proportional-hazards and competing-risks model to retrospectively analyze the impact of related factors on lymph nodes statistically.
    A total of 2,435 patients were included in the analysis, of which 198 died of MTC. The results of the multifactor competing-risk model showed that the number of total lymph nodes (19-89), positive lymph nodes (1-10,11-75) and positive lymph node ratio (25%-53%,>54%), age (46-60,>61), chemotherapy, mode of radiotherapy (others), tumor size(2-4cm,>4cm), number of lesions greater than 1 were poor prognostic factors for MTC. For the number of total lymph nodes, unlike the multivariate Cox proportional-hazards model results, we found that it became an independent risk factor after excluding competitive risk factors. Competitive risk factors have little effect on the number of positive lymph nodes. For the proportion of positive lymph nodes, we found that after excluding competitive risk factors, the Cox proportional-hazards model overestimates its impact on prognosis. The competitive risk model is often more accurate in analyzing the effects of prognostic factors.
    After excluding the competitive risk, the number of lymph nodes, the number of positive and the positive proportion are the poor prognostic factors of medullary thyroid cancer, which can help clinicians more accurately evaluate the prognosis of patients with medullary thyroid cancer and provide a reference for treatment decision-making.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    髓样乳腺癌(MBC)患者是否接受化疗存在争议。因此,我们研究的目的是筛选从化疗获益的MBC患者.我们从监测中招募了618名连续的MBC患者,流行病学,和最终结果(SEER)数据库(2010-2018年)。Cox回归分析用于确定独立的预后因素。接下来,使用校准图和受试者工作特征(ROC)曲线的曲线下面积(AUC)构建并评估列线图.使用Kaplan-Meier曲线评估不同风险组化疗的总生存期(OS)获益。共有618例MBC患者参与了我们的研究,使用8:2的比例将它们随机分为训练队列(n=545)和验证队列(n=136).接下来,根据五个独立因素(诊断时的年龄,T级,N状态,亚型和辐射)。3年和5年OS的列线图AUC(训练集:0.793和0.797;验证集:0.781和0.823)和校准图表现出良好的判别和预测能力。此外,针对MBC患者的新型风险分类系统表明,我们没有足够的证据支持化疗对高危组的获益效应,因为结果无统计学意义(总人口:p=0.180;训练集:p=0.340),但可以改善低危组的OS(总人口:p=0.001;训练集:p=0.001).我们的结果表明,应根据多种因素对高危人群更仔细地选择化疗,并且将来应通过更多的临床试验来证实免除化疗的可能性。
    Whether patients with medullary breast carcinoma (MBC) receive chemotherapy is controversial. Therefore, the aim of our study was to screen out patients with MBC who benefit from chemotherapy. We enrolled 618 consecutive patients with MBC from The Surveillance, Epidemiology, and End Results (SEER) database (2010-2018). Cox regression analysis was used to identify independent prognostic factors. Next, a nomogram was constructed and evaluated using calibration plots and the area under the curve (AUC) of receiver operating characteristic (ROC) curves. Kaplan‒Meier curves were used to evaluate the overall survival (OS) benefit of chemotherapy in different risk groups. A total of 618 MBC patients were involved in our study, and an 8:2 ratio was used to randomly split them into a training cohort (n = 545) and a validation cohort (n = 136). Next, a nomogram predicting 3- and 5-year OS rates was constructed based on the five independent factors (age at diagnosis, T stage, N status, subtype and radiation). The nomogram AUCs for 3- and 5-year OS (training set: 0.793 and 0.797; validation set: 0.781 and 0.823) and calibration plots exhibited good discriminative and predictive ability. Additionally, a novel risk classification system for MBC patients demonstrated that we do not have enough evidence to support the benefit effect of chemotherapy for the high-risk group as the result is not statistically significant (total population: p = 0.180; training set: p = 0.340) but could improve OS in the low-risk group (total population: p = 0.001; training set: p = 0.001). Our results suggested that chemotherapy should be selected more carefully for high-risk groups based on a combination of factors and that the possibility of exemption from chemotherapy should be confirmed by more clinical trials in the future.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    未经证实:降钙素(Ct)阴性的甲状腺髓样癌(MTC)是一种罕见的神经内分泌肿瘤。本研究旨在阐明其发病率,临床病理特征,管理,和治疗结果。
    UNASSIGNED:我们回顾性分析了原发性MTC患者的资料。根据术前血清Ct水平将患者分为两组(Ct阴性和Ct阳性)。人口统计,病理性,和分子特征,比较两组的治疗效果。在Ct阴性组中,我们分析了手术类型与治疗结果之间的相关性.
    未经批准:在总共312名患者中,24例诊断为Ct阴性MTC。Ct阳性组的淋巴结转移率明显高于Ct阴性组(47.9%vs.0%,p<0.001)。Ct阴性组Ki-67≤10%的患者比例明显高于Ct阳性组(87.5%vs.38.2%,p<0.001)。在Ct阴性和Ct阳性组中,91.7%和34.7%的患者达到了良好的反应,分别(p<0.001)。在Ct阴性组中,所有女性患者均获得了出色的反应,但只有50%的男性患者。
    UNASSIGNED:Ct阴性MTC是罕见的,不太可能发生淋巴结转移。单侧肺叶切除术往往会提供令人满意的良好反应机会;然而,这需要进一步验证。
    Calcitonin (Ct)-negative medullary thyroid carcinoma (MTC) is a rare neuroendocrine tumor. This study aimed to clarify its incidence, clinicopathologic characteristics, management, and treatment outcome.
    We retrospectively analyzed data of patients with primary MTC. Patients were divided into two groups according to the preoperative serum Ct level (Ct-negative and Ct-positive). The demographic, pathologic, and molecular characteristics, and treatment outcomes were compared between the two groups. In the Ct-negative group, we analyzed the association between the operation type and treatment outcome.
    Of the total 312 patients, 24 were diagnosed with Ct-negative MTC. The rate of lymph node metastasis was significantly higher in the Ct-positive than in the Ct-negative group (47.9% vs. 0%, p<0.001). The proportion of patients with Ki-67 ≤10% was significantly higher in the Ct-negative than in the Ct-positive group (87.5% vs. 38.2%, p<0.001). Excellent response was achieved by 91.7% and 34.7% of patients in the Ct-negative and Ct-positive groups, respectively (p<0.001). In the Ct-negative group, excellent response was achieved by all female patients, but only 50% of male patients.
    Ct-negative MTC is rare and unlikely to develop lymph node metastasis. Unilateral lobectomy tends to provide a satisfactory chance of excellent response; however, this requires further validation.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    暂无摘要。
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

    求助全文

  • 文章类型: Journal Article
    优化的术前诊断工具与降钙素测试,超声特征,功能成像模式,和检测遗传形式的基因检测导致甲状腺髓样癌(MTC)的早期诊断和手术率增加。这有助于使初级手术适应肿瘤阶段,避免局部肿瘤生长的手术过度治疗,即,偏离常规推荐的甲状腺切除术,双侧中央区淋巴结清扫术,而采用有限的单侧入路。为了限制初级手术治疗,至关重要的是,MTC在临床上是单一的,零星的,局限于甲状腺,降钙素水平表明手术后生化恢复。这种有限方法的主要要求是冷冻切片研究的可用性,可靠地表明(i)MTC的R0切除,(ii)器官囊没有浸润,(iii)缺乏血管增生(即,MTC转移潜能的证据),(iii)没有对侧疾病或癌前病变。患者必须知情同意,谁已经充分了解了优势,缺点,以及不接受“经典”外科手术的潜在风险。本文的目的是审查早期MTC管理指南。
    Optimized preoperative diagnostic tools with calcitonin tests, ultrasound features, functional imaging modalities, and genetic testing to detect hereditary forms have led to an increased rate of earlier diagnosis and surgery for medullary thyroid cancer (MTC). This helps to adapt the primary surgery to the tumor stage and avoid surgical overtreatment for localized tumor growth, i.e., deviating from the regularly recommended thyroidectomy with bilateral central lymph node dissection in favor of a limited unilateral approach. To limit primary surgical therapy, it is crucial that the MTC is clinically unifocal, sporadic, and confined to the thyroid, and that calcitonin levels indicate biochemical recovery after surgery. The main requirement for such a limited approach is the availability of frozen section studies that reliably indicate (i) R0 resection of the MTC, (ii) absence of infiltration of the organ capsule, (iii) lack of desmoplasia (i.e., evidence of the metastatic potential of the MTC), (iiii) absence of contralateral disease or precancerous lesions. Informed consent is mandatory from the patient, who has been fully informed of the advantages, disadvantages, and potential risks of not undergoing the \"classic\" surgical procedure. The aim of this article is to review the guidelines for the management of early-stage MTC.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Case Reports
    BACKGROUND: Most gastric cancer patients are diagnosed at mid- to late-stage and lose the chance of radical surgery, medical treatment is especially important to prolong the survival of patients. Apatinib mesylate, which is a small molecule vascular endothelial growth factor receptor 2 tyrosine kinase inhibitor, could be used as antiangiogenesis therapy for gastric cancer.
    UNASSIGNED: A 67-year-old man sought medical care for upper abdominal discomfort.
    METHODS: The patient was diagnosed as mixed medullary differentiated gastric adenocarcinoma, and immunohistochemistry suggested HER-2 (2+).
    METHODS: The patient received chemotherapy consisting of oxaliplatin combined with S-1 as first-line treatment, and targeted therapy with apatinib mesylate as second-line treatment.
    RESULTS: After 4 months of first-line chemotherapy, the patient received apatinib treatment immediately at a dose of 500 mg/d orally and died of cardiac arrest with 8.5 months of overall survival. During this period of targeted therapy with apatinib mesylate, this male patient suffered mammary gland development besides other common adverse reactions.
    CONCLUSIONS: This case report is the first to report the case of male mammary gland development after oral apatinib.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Sci-hub)

       PDF(Pubmed)

公众号