Acanthosis nigricans malin révélant un cholangiocarcinome

IntroductionAcanthosis nigricans is a dermatosis characterized by the presence of a hyperpigmented, velvety cutaneous thickening in the flexural areas, especially axillary and inguinal fossas, and lateral faces of the neck. AN is usually a benign condition but can sometimes reveal an internal malignancy corresponds to a cutaneous paraneoplasic syndrome. Literature shows a predominant association with gastric adenocarcinoma. Here, we report a rare association between AN and cholangiocarcinoma.Case reportWe report a 43-year-old woman who presented an extensive AN associated to a tripe palms syndrome and florid cutaneous papillomatosis. She consulted in dermatology because of the itchiness of the lesions as well as for esthetics reasons. Complementary investigations enabled to diagnose a cholangiocarcinoma without visceral metastasis and she was treated by tumor resection and chemotherapy. Consequently, a slight improvement of the skin condition and the disappearance of pruritus were observed.ConclusionAN should be considered as cutaneous sign either of malignancy or endocrinopathy and therefore requires further investigations. The existence of extensive lesions, pruritus, tripe palms syndrome, florid cutaneous papillomatosis or mucous lesions, associated to an AN is a sign of malignancy should be investigated urgently the early diagnosis of which can lead to a better prognosis.     

Phyllodes tumors of the breast: Adjuvant radiation therapy revisited

BackgroundPhyllodes tumors (PT) are rare entity and surgical resection is the cornerstone of treatment. No standard of care exists regarding adjuvant treatment especially radiation therapy (RT).Patients and methodsWe analyzed all patients with non-metastatic, resected phyllodes tumors who presented to our institution from January 2005 through December 2019. Primary study endpoints included local recurrence free survival (LRFS) and overall survival (OS).ResultsOne hundred and eight patients were analyzed (patients with incomplete treatment and follow up data were excluded). Fifty patients had benign phyllodes, 26 patients had borderline and 32 patients had malignant phyllodes. In the benign group, no significant difference in LRFS was observed between patients who received adjuvant RT (n = 3) and those who did not (5-year LRFS 100% vs. 85% respectively, p = 0.49). The 5 year OS for patients who received RT was 60% vs. 89% for those who did not (p 0.40). In the borderline/malignant group, adjuvant RT significantly improved five year LRFS (90% in the RT group vs. 42% in the no RT group, p = 0.005). The 5 year LRFS in patients treated with margin negative breast conserving surgery and RT was 100% vs. 34.3% in patients who did not receive RT (p 0.022). Patients treated with mastectomy and RT had a 5 year LRFS of 100% vs. 83% for patients who did not receive RT (p 0.24). On multivariate analysis, radiation therapy was independently associated with decreased hazard of local failure (HR 0.21, CI 0.05–0.89, p = 0.03). No difference in OS was found between the RT and no RT groups (5-year OS was 52% vs. 45% respectively, p 0.54).ConclusionThe results of the current study confirm the excellent prognosis of benign phyllodes tumors; warranting no further adjuvant treatment after margin-negative surgical resection. For patients with borderline/malignant phyllodes tumors, adjuvant radiation therapy significantly improved LRFS after margin negative wide local excision; however, patients treated with mastectomy did not attain the same benefit from adjuvant irradiation.     

恶性肿瘤代谢特点及其与炎性介质的相关性研究进展

 恶性肿瘤是一种高能量代谢疾病，因其本身的作用，人体对恶性肿瘤的应激反应以及抗癌治疗过程中的措施导致肿瘤患者的代谢特点具有特殊性，如脂肪分解、脂肪酸氧化增加、瘦组织群减少、胰岛素抵抗以及蛋白质代谢紊乱等。而大量代谢产物如脂多糖（LPS）、乳酸、花生四烯酸代谢物（PGE2）、游离脂肪酸（FFA）等常诱发慢性轻度炎症。而很多肿瘤多发生在感染、慢性刺激和炎性反应的部位，所以炎性微环境对肿瘤的影响已成为科研领域的研究重点。而在肿瘤机体代谢的过程中，许多炎性介质参与其中，故本文就肿瘤能量代谢特点、代谢异常与相关炎性介质的关系作一系统总结。     

早期骨转移分子机制的研究进展

骨转移是恶性肿瘤常见并发症，其中乳腺癌和肺癌多为溶骨性骨转移，具有临床发病率高的特点，其临床表现为疼痛、高钙血症、病理性骨折等，严重影响患者生活质量以及缩短生存时间。然而，肿瘤细胞转移到骨微环境的机制尚未完全明确，最新研究提示骨转移出现临床症状前，肿瘤细胞和骨微环境之间存在相关调控的作用。本文从肿瘤细胞和骨微环境的特异性、肿瘤细胞与骨细胞之间的相互调控等方面进行综述，以期指导临床用药。     

Aberrant expression of Wnt/β-catenin signaling pathway genes in aggressive malignant gastric gastrointestinal stromal tumors

IntroductionRecent reports on gene expression profiling (GEP) show several genes associated with malignant progression of GIST. However, genes associated with malignant transformation have not been clarified. Here, we aimed to reveal distinct genes in aggressive malignant GIST, using comprehensive gene expression analysis.Materials and methodsWe investigated GEP obtained by microarrays for 43 gastric GISTs, which mostly harbored KIT and PDGFRA mutations and integrated clinicopathological risk information. RT-PCR and immunohistochemistry were performed for FZD7, a receptor of Wnt ligands.ResultsGEP divided 43 gastric GISTs into two clusters. A cluster included seven of eight high-risk GISTs (88%) in modified NIH classification and was defined as high-risk cluster; the other cluster was defined as low-risk cluster. The number of probes with over 3-fold changes between the two clusters was 1,177, in which probes corresponding to 16 oncogenes were included. Genes involved in the Wnt signaling pathway were the most abundant among the 16 oncogenes. Focusing on 73 Wnt signaling pathway genes of the 21,578 probes, 12 upregulated and 5 downregulated genes were found in the high-risk cluster. Major cascade genes promoting the Wnt/β-catenin signaling pathway, including WNT11, FZD family, and DVL2, were upregulated in the high-risk cluster. SNAI1, SNAI2, and BIRC5, which are activated by this pathway and increase cell proliferation, were also upregulated. These gene expression alterations were consistent in the positive direction of this pathway. GISTs in high-risk cluster strongly expressed FZD7.ConclusionWnt/β-catenin signaling pathway may play an important role in malignant transformation of indolent GIST.     

多模态MRI对子宫恶性中胚叶混合瘤的影像学特征与病理对照的分析

目的探讨多模态MRI对子宫恶性中胚叶混合瘤(MMMT)的影像学特征与病理对照的关系,以提高对该病的认识及诊断准确率。方法选取28例经手术病理证实MMMT患者的临床资料,讨论分析临床特点、MRI平扫+联合动态增强(DCE-MRI)及弥散加权像成像DWI(ADC值)检查、病理学及免疫组化检查。结果MMMT癌成分为子宫内膜样腺癌12例,鳞状细胞癌及低分化浆液性腺癌8例,腺鳞癌伴鳞状分化6例,透明细胞腺癌2例;肉瘤成分为内膜间质肉瘤、平滑肌肉瘤、软骨肉瘤、纤维肉瘤、血管肉瘤、横纹肌肉瘤及成骨肉瘤;同源性为25例,以内膜间质肉瘤多见;异源性为3例,软骨肉瘤1例,横纹肌肉瘤及骨肉瘤2例。临床分期:Ⅰ期6例,Ⅱ期7例,Ⅲ期12例,Ⅳ期3例。28例MMMT的部位:原发病灶13例位于宫体内,8例位于宫底,3例位于宫颈,4例位于左侧卵巢。结论MMMT在MRI平扫+DCE-MRI及ADC值检查更能够清晰显示肿瘤的部位、范围、形态、大小、边界、信号特点及强化程度、弥散系数ADC值、淋巴结转移等情况,因此,结合临床特征、病理学及免疫组化检查为临床更进一步早期诊治、疗效评估及术前的准确诊断和制定合理的治疗计划提供更准确的参考依据,以提高患者生存率。     

Retrospective study of malignant phyllodes tumors of the breast: Younger age,prior fibroadenoma surgery,malignant heterologous elements and surgical margins may predict recurrence

PurposeThe potential recurrence rate of malignant phyllodes tumors (MPTs) of the breast is high, and the prognostic factors are still unclear. We therefore aim to study the factors affecting the outcome of MPTs.MethodsA retrospective review of MPT patients treated from 2006 to 2020 at our institution was conducted. Univariate and multivariate Cox proportional hazard models were used to examine the influence of different variables on RFS. Moreover, significant prognostic factors were combined to construct the nomogram to predict the probability of relapse occurring in MPT patients. The 5-year and 10-year RFS rates were estimated using the Kaplan–Meier method.ResultsDuring the study period, 188 MPT patients were identified. The presence of malignant heterologous elements was observed in 23 (12.2%) patients with MPT, and the patients with malignant heterologous elements who received chemotherapy had longer RFS, which could reduce the risk of recurrence (p = 0.022). Recurrence occurred in 56/188 (29.8%) patients, of whom 47 experienced local recurrence and 11 experienced distant metastases. The 5-year and 10-year cumulative RFS rates were 77.5% and 70.1%, respectively. Age (p = 0.041), fibroadenoma surgery history (p = 0.004), surgical margins (p = 0.001) and malignant heterologous elements (p < 0.001) were independent risk factors for postoperative RFS. Subsequently, a nomogram was built, with a C-index of 0.64 (95% CI: 0.629–0.661), to predict the risk of recurrence.ConclusionThe results of this study showed that younger age, fibroadenoma surgery history, malignant heterologous elements and surgical margins <1 cm predict a higher incidence of recurrence in MPT patients. Patients with malignant heterologous elements treated with chemotherapy could have a reduced risk of recurrence.