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苗勒管腺肉瘤:单中心59例这种罕见实体瘤的经验

Müllerian Adenosarcoma: A Single-Centre Experience of 59 Cases of This Rare Entity.

作者信息

Rana Iftikhar Ali, Hassan Usman, Bashir Shaarif, Hussain Mudassar, Shakeel Mehroosh, Mushtaq Sajid

机构信息

Histopathology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore, PAK.

出版信息

Cureus. 2021 Feb 15;13(2):e13360. doi: 10.7759/cureus.13360.

Abstract

Background and objective Müllerian adenosarcomas (MA) are rare biphasic tumors with benign epithelial and sarcomatous stromal components. There is very limited cohort study data on MA in the South Asian countries and no such study has been attempted in Pakistan. Our aim was to evaluate the clinicopathological characteristics of MA and to review the published literature on the condition. Additionally, we also analyzed the impact of various prognostic factors on the overall survival (OS) of patients with MA. Materials and methods This was a retrospective observational study performed at the Shaukat Khanum Memorial Hospital and Research Centre, Lahore from 2003 to 2020. A total of 59 histologically confirmed cases of MA were included in the study and critically reviewed. Results The mean age of the patients was 54 ±16 years, and the most common tumor location was the uterine corpus (48, 81.4%), followed by the cervix (eight, 13.6%), ovary (two, 3.4%), and vagina (one, 1.7%). Sarcomatous overgrowth (SO) was seen in 22 (37.3%) patients, and high-grade cytology was observed in 18 (30.5%) patients. Furthermore, lymphovascular invasion (LVI) was present in six (10.2%) patients, and myometrial invasion was noted in 25 (42.4%) patients. The follow-up details of 29 patients were available, and death was recorded in 13 (44.8%) patients with a median OS of three years. Conclusion MA is a rare and diagnostically challenging entity due to its wide differential diagnosis. It is essential to take note of different morphological features such as SO, cytological features, LVI, and heterologous differentiation because of their significant prognostic impact.

摘要

背景与目的 苗勒管腺肉瘤(MA)是一种罕见的双相性肿瘤,由良性上皮和肉瘤样间质成分组成。南亚国家关于MA的队列研究数据非常有限,巴基斯坦尚未进行此类研究。我们的目的是评估MA的临床病理特征,并回顾关于该疾病的已发表文献。此外,我们还分析了各种预后因素对MA患者总生存期(OS)的影响。材料与方法 这是一项在拉合尔的沙卡特汗姆纪念医院及研究中心进行的回顾性观察研究,时间跨度为2003年至2020年。该研究共纳入59例经组织学确诊的MA病例,并进行了严格审查。结果 患者的平均年龄为54±16岁,最常见的肿瘤部位是子宫体(48例,81.4%),其次是宫颈(8例,13.6%)、卵巢(2例,3.4%)和阴道(1例,1.7%)。22例(37.3%)患者出现肉瘤样过度生长(SO),18例(30.5%)患者观察到高级别细胞学特征。此外,6例(10.2%)患者存在淋巴管侵犯(LVI),25例(42.4%)患者出现肌层侵犯。29例患者有随访详情,13例(44.8%)患者死亡,中位总生存期为3年。结论 MA是一种罕见且诊断具有挑战性的疾病,因其鉴别诊断范围广泛。由于不同形态特征如SO、细胞学特征、LVI和异源性分化具有显著的预后影响,因此必须予以关注。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/71a1/7969483/e39de357909e/cureus-0013-00000013360-i01.jpg

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