Uterine sarcoma

子宫肉瘤
  • 文章类型: Meta-Analysis
    背景:本系统综述和荟萃分析旨在确定中性粒细胞与淋巴细胞比率(NLR)作为子宫肉瘤和子宫平滑肌瘤临床区别的评估工具的潜在价值。
    方法:我们全面搜索了WebofScience,Scopus,和PubMed在2023年3月19日之前发表的相关论文。提供了标准化平均差(SMD),以及95%置信区间(CI)。由于高度的异质性,随机效应模型被用来得出合并效应。纽卡斯尔-渥太华量表用于质量评估。我们的研究在PROSPERO(CRD42023478331)注册。
    结果:总体而言,7篇文章被纳入分析.随机效应模型显示,子宫肉瘤患者的NLR水平高于子宫肌瘤患者(SMD=0.60,95%CI=0.22-0.98;p=0.002)。在根据样本量的亚组分析中,在大型研究(SMD=0.58,95%CI=0.04~1.13;P<0.001)或小型研究(SMD=0.64,95%CI=0.33~0.96;P=0.32)中,我们发现子宫肉瘤患者的NLR水平高于子宫肌瘤患者.在敏感性分析中,我们发现,当单个研究被删除时,最终结果没有显著变化,表明这项荟萃分析的发现是稳定的。NLR的合并敏感性为0.68(95%CI=0.61-0.73),合并特异性为0.64(95%CI=0.59-0.69)。
    结论:NLR可用作临床评估工具,以帮助临床医生区分子宫肉瘤和肌瘤患者。
    BACKGROUND: This systematic review and meta-analysis aimed to determine the potential value of neutrophil to lymphocyte ratio (NLR) as an assessment tool in the clinical distinction between uterine sarcoma and uterine leiomyoma.
    METHODS: We comprehensively searched Web of Science, Scopus, and PubMed for relevant papers published before March 19, 2023. The standardized mean difference (SMD) was provided, along with a 95% confidence interval (CI). The random-effects model was employed to derive pooled effects due to the high levels of heterogeneity. The Newcastle-Ottawa scale was used for the quality assessment. Our study was registered in PROSPERO (CRD42023478331).
    RESULTS: Overall, seven articles were included in the analysis. A random-effect model revealed that patients with uterine sarcoma had higher NLR levels compared to those with uterine myoma (SMD = 0.60, 95% CI = 0.22-0.98; p = 0.002). In the subgroup analysis according to sample size, we found that patients with uterine sarcoma had elevated levels of NLR compared to those with uterine myoma in either large studies (SMD = 0.58, 95% CI = 0.04-1.13; P < 0.001) or small studies (SMD = 0.64, 95% CI = 0.33-0.96; P = 0.32). In the sensitivity analysis, we found that the final result was not significantly changed when single studies were removed, suggesting that the finding of this meta-analysis was stable. The pooled sensitivity of NLR was 0.68 (95% CI = 0.61-0.73), and the pooled specificity was 0.64 (95% CI = 0.59-0.69).
    CONCLUSIONS: NLR might be utilized as an assessment tool in clinics to help clinicians differentiate between patients with uterine sarcoma and those with myoma.
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  • 文章类型: Journal Article
    这篇综述旨在全面描述子宫肉瘤的手术方法。子宫肉瘤是罕见的子宫肿瘤。经常,在计划对假定的良性平滑肌瘤进行子宫切除术或子宫肌瘤切除术后进行诊断。子宫肉瘤手术治疗的金标准是子宫切除术和双侧附件卵巢切除术。对于那些希望保持生育能力的患者,可以采取保留生育能力的方法。盆腔淋巴结清扫术的作用是有争议的;事实上,仅在放射学怀疑淋巴结受累的情况下,建议切除淋巴结。使用粉碎器与总复发风险增加有关,腹腔内复发和死亡。鉴于辅助化疗的作用不确定,晚期疾病管理应根据患者的表现状况进行定制。晚期或复发性疾病的治疗仍然是一个争论的话题,但就发病率和死亡率而言,手术是最好的方法。这些子宫肿瘤的治疗方法很少,需要进一步的研究来阐明首次诊断为子宫肉瘤的患者和复发子宫肉瘤的患者的诊断和治疗途径。没有具体证据支持在子宫局限性疾病中采用辅助治疗,和分子/基因组谱分析可能有助于识别有复发风险的患者.
    This review aims to provide a comprehensive description of surgical approaches for the management of uterine sarcomas. Uterine sarcomas are rare uterine neoplasms. Frequently, diagnosis is made after hysterectomy or myomectomy scheduled for presumed benign leiomyomas. The gold standard for surgical treatment of uterine sarcomas is hysterectomy with bilateral salpingo-oophorectomy. It is possible to adopt a fertility-sparing approach for those patients who wish to maintain their fertility. The role of pelvic lymphadenectomy is controversial; in fact, removal of lymph nodes is only recommended in the case of radiological suspicion of nodal involvement. Use of a morcellator is associated with increased risk of total recurrence, intra-abdominal recurrence and death. Advanced disease management should be customized based on the patient\'s performance status given the uncertain role of adjuvant chemotherapy. Treatment of advanced or recurrent disease remains a subject of debate, but surgery is the best approach in terms of morbidity and mortality. There are few options for management of these uterine tumours, and further studies are needed to clarify the diagnostic and therapeutic pathways of patients with a first diagnosis of uterine sarcoma and patients with relapse of uterine sarcoma. No specific evidence supports the adoption of adjuvant therapy in uterine-confined disease, and molecular/genomic profiling may be useful to identify patients at risk of recurrence.
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  • 文章类型: Journal Article
    BACKGROUND: Recently, artificial intelligence (AI) with computerized imaging analysis is attracting the attention of clinicians, in particular for its potential applications in improving cancer diagnosis. This review aims to investigate the contribution of radiomics and AI on the radiological preoperative assessment of patients with uterine sarcomas (USs).
    METHODS: Our literature review involved a systematic search conducted in the last ten years about diagnosis, staging and treatments with radiomics and AI in USs. The protocol was drafted according to the systematic review and meta-analysis preferred reporting project (PRISMA-P) and was registered in the PROSPERO database (CRD42021253535).
    RESULTS: The initial search identified 754 articles; of these, six papers responded to the characteristics required for the revision and were included in the final analysis. The predominant technique tested was magnetic resonance imaging. The analyzed studies revealed that even though sometimes complex models included AI-related algorithms, they are still too complex for translation into clinical practice. Furthermore, since these results are extracted by retrospective series and do not include external validations, currently it is hard to predict the chances of their application in different study groups.
    CONCLUSIONS: To date, insufficient evidence supports the benefit of radiomics in USs. Nevertheless, this field is promising but the quality of studies should be a priority in these new technologies.
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  • 文章类型: Journal Article
    The aim of this article is to summarize the MRI features of each sarcoma subtype and to correlate them with its pathological findings. Literature review through PubMed/Medline database to identify relevant articles on uterine sarcomas, with a special emphasis on their MRI findings and pathological features. While several, more generalistic, MRI findings of a uterine tumour should raise suspicion for malignancy (including irregular contour, intra-tumoral necrosis/hemorrhage and low ADC values), some particular features may suggest their specific histological subtype such as the gross lymphovascular invasion associated with endometrial stromal sarcomas, the \"bag of worms\" appearance of the low-grade endometrial stromal sarcoma and the \"lattice-like\" aspect of adenosarcomas which results from the mixed composition of solid and multiseptated cystic components. Knowledge of the different histological uterine sarcoma subtypes, their specific MRI features and comprehension of their pathological background allows for a more confident diagnosis and may indicate the correct histological subtype.
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  • 文章类型: Case Reports
    OBJECTIVE: Angiosarcoma of primary gynecologic origin is an extremely rare and highly malignant tumor of endothelial origin with a 5-year survival rate of less than 35%. To date, only 61 cases have been described in the literature. The aim of this study was to present more cases and discuss potential therapy options.
    METHODS: The following case series presents three cases of gynecologic angiosarcomas that were under therapy at the Charité - University medicine of Berlin from June 2014 to February 2018.
    RESULTS: Two of the cases deal with primary angiosarcomas of the uterus whereas the third case was diagnosed after the suspicion of a recurrence of a poorly differentiated squamous cell carcinoma of the cervix uteri. In case one a 75-year old patient with initial postmenopausal bleeding and a tumor mass of the uterus is described. After surgery a hemangiosarcoma of the uterus was confirmed. After two months the patient presented with a presacral peritoneal sarcomatosis. Chemotherapy of weekly paclitaxel was administered. Case two deals with a patient presenting with abdominal pain. A uterine sarcoma with infiltration of the parametry and angiosarcomatosis peritonei was diagnosed during an emergency laparotomy because of spontaneous peritoneal bleeding. Moreover, osseous metastasis was found. The patient underwent weekly paclitaxel. Due to tumor progression, chemotherapy was changed to doxorubicin and olaratumab and radiotherapy was induced. The patient died 33 months after initial diagnosis. Case three describes a 34-year old patient with suspected local recurrence of cervical cancer with infiltration of the bladder. During TURB an angiosarcoma was found. Following laparoscopy revealed peritoneal metastasis. The patient underwent weekly paclitaxel followed by a paclitaxel and pazopanib maintainance therapy which showed a regression. Due to progression afterwards, chemotherapy was changed to gemcitabine and docetaxel and gemcitabine monotherapy. The patient died 33 months after initial diagnosis.
    CONCLUSIONS: Even though there is no evidence on standard treatment of this extremely rare and aggressive tumor entity of the female genital tract the patients showed the longest stability of disease during chemotherapy with weekly paclitaxel.
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  • 文章类型: Journal Article
    背景:尽管过去几年来辅助化疗(AC)的使用似乎在增加,多项临床试验和之前的荟萃分析未能确定AC是否能改善子宫平滑肌肉瘤(uLMS)的临床结局.这项系统评价和荟萃分析的目的是比较早期uLMS初次手术后的AC(有或没有放疗)与观察(obs)。
    方法:检索随机对照研究(RCT)和非随机研究(NRS)。感兴趣的结果如下:远处复发率,局部复发率和总体复发率。关于远处复发率的结果,通过计算比值比(ORs)和95%置信区间(CIs)来比较局部区域复发率和总体复发率;ORs与Mantel-Haenszel方法相结合.
    结果:九项研究被纳入分析,涉及545例患者(AC:252,obs:293)。与obs相比,AC并没有降低局部和远处的复发率,合并OR分别为1.36和0.63。同样,与obs相比,给予AC并未降低总复发率.
    结论:根据我们的结果,AC(有或没有放疗)在早期uLMS中没有降低复发率;因此,AC在这种情况下的作用尚不清楚.
    BACKGROUND: Although the use of adjuvant chemotherapy (AC) appears to be increasing over the past few years, several clinical trials and previous meta-analyses failed to determine whether AC could improve clinical outcomes in uterine leiomyosarcoma (uLMS). The aim of this systematic review and meta-analysis was to compare AC (with or without radiotherapy) versus observation (obs) after primary surgery in early stage uLMS.
    METHODS: Randomized controlled (RCTs) and non-randomized studies (NRSs) were retrieved. Outcomes of interest were as follows: distant recurrence rate, locoregional recurrence rate and overall recurrence rate. Results about distant recurrence rate, locoregional recurrence rate and overall recurrence rate were compared by calculating odds ratios (ORs) with 95% confidence intervals (CIs); ORs were combined with Mantel-Haenszel method.
    RESULTS: Nine studies were included in the analysis, involving 545 patients (AC: 252, obs: 293). Compared with obs, AC did not reduce locoregional and distant recurrence rate, with a pooled OR of 1.36 and 0.63, respectively. Similarly, administration of AC did not decrease overall recurrence rate in comparison to obs.
    CONCLUSIONS: According to our results, AC (with or without radiotherapy) did not decrease recurrence rate in early stage uLMS; thus, the role of AC in this setting remains unclear.
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  • 文章类型: Case Reports
    Uterine leiomyosarcoma (uLMS) is a rare and aggressive malignancy with poor clinical outcomes. Even when localized, uLMS is associated with high rates of local and distant recurrences that are usually fatal. Common sites of recurrence are lung, liver, pelvic lymph nodes, and vertebral and long bones, though atypical patterns of recurrence have been described. Among them, intracranial recurrence appears as a rare finding, almost exceptional in skull and dura. We describe the case of a solitary skull metastasis from uLMS in a 39-year-old woman, which represents the third reported case of skull recurrence in literature. After multidisciplinary discussion, the patient underwent surgery and received adjuvant radiotherapy. After 4 months, she is currently alive, without evidence of extracranial disease. This case highlights the importance of suspecting and recognizing atypical and extremely rare metastasis to this region. We encourage the need for large case series in order to provide further information about cranial recurrences of uLMS taking into account the paucity of data currently available in literature and the frequently unpredictable behavior of this rare and highly lethal disease.
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  • 文章类型: Case Reports
    SMARCA4缺陷型子宫肉瘤(SDUS)是一种新发现的未分化的子宫间充质恶性肿瘤,其SMARCA4的表达缺失。
    一名46岁女性在过去5个月内出现严重的不规则阴道出血。计算机断层扫描和磁共振成像显示子宫内有一个巨大的盆腔肿块,怀疑有区域转移性淋巴结病的恶性肿瘤。活检证实SDUS,患者接受化疗。治疗3个月后症状好转。
    据报道,这种新描述的实体极为罕见。认识到SDUS的特征性影像学和病理学表现对于准确诊断至关重要。这可能会影响患者的生存。
    UNASSIGNED: SMARCA4-deficient uterine sarcoma (SDUS) is a newly discovered undifferentiated uterine mesenchymal malignancy which has loss of expression of SMARCA4.
    UNASSIGNED: A 46-year-old woman presented with heavy irregular vaginal bleeding over the previous 5 months. Computed tomography and magnetic resonance imaging showed a large pelvic mass centered within the uterus, suspicious of malignancy with regional metastatic lymphadenopathy. Biopsy confirmed SDUS and patient underwent chemotherapy. Her symptoms improved 3 months after treatment.
    UNASSIGNED: An extremely rare case of this newly described entity is reported. Recognizing the characteristic imaging and pathology findings of SDUS is essential for an accurate diagnosis, which may affect patient survival.
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  • 文章类型: Case Reports
    CIC重排的圆形细胞肉瘤(CRS)是一种罕见的实体,存在于各种解剖位置,并涉及深层软组织结构和皮肤。尽管通常与尤因肉瘤(ES)混淆并在临床上相似,研究人员最近表明,这种独特的疾病保持了与ES不同的形态学和病理学特征.在这份报告中,我们介绍并讨论了子宫CRS的病例,这是在英语文献中首次报道的。我们敦促科学界继续调查,阐明这一实体的特征,因为患有这种疾病的年轻女性预后不佳,目前无法获得治愈的治疗选择。
    CIC-rearranged round cell sarcoma (CRS) is a rare entity that presents in various anatomical locations and involves deep soft-tissue structures and skin. Although commonly confused with and clinically similar to Ewing sarcoma (ES), investigators have recently shown that this unique condition maintains morphologic and pathologic features that are distinct from ES. In this report, we present and discuss a case of CRS of the uterus, the first of its kind to be reported in the English-language literature. We urge the scientific community to continue its investigations in elucidating the features of this entity, as young women who suffer from this condition have dismal prognoses and currently do not have access to therapeutic options for cure.
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  • 文章类型: Journal Article
    MRI plays an essential role in patients before treatment for uterine mesenchymal malignancies. Although MRI includes methods such as diffusion-weighted imaging and dynamic contrast-enhanced MRI, the differentiation between uterine myoma and sarcoma always becomes problematic. The present paper discusses important findings to ensure that sarcomas are not overlooked in magnetic resonance (MR) images, and we describe the update in the differentiation between uterine leiomyoma and sarcoma with recent reports.
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