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女性生殖道恶性苗勒管混合瘤(癌肉瘤):来自印度北部单一中心的回顾性研究

Malignant Mixed Mullerian Tumor (Carcinosarcoma) in the Female Genital Tract: A Retrospective Study From a Single Center in North India.

作者信息

Raghuvanshi Shivanjali, Gupta Nidhi, Yadav Kusum, Chaudhary Sneha, Kumar Manish, Sharma Akanksha

机构信息

Pathology, King George's Medical University, Lucknow, IND.

Pathology, Dr. KNS Memorial Institute of Medical Sciences, Lucknow, IND.

出版信息

Cureus. 2024 Oct 24;16(10):e72317. doi: 10.7759/cureus.72317. eCollection 2024 Oct.

Abstract

Background Carcinosarcoma (malignant mixed Mullerian tumor (MMT)) is a rare tumor of the uterus, even rarer in the ovary. Presenting symptoms are indistinguishable from high-grade uterine endometrium carcinoma. Histologically, tumors are biphasic, composed of both epithelial and mesenchymal (sarcomatous) components. The diagnosis of carcinosarcoma presents a challenge due to the need to exclude commonly occurring mimickers, necessitating the application of a broad panel of immunohistochemistry markers. Material and methods This retrospective descriptive study of malignant MMT was conducted over a three-year period (August 2021 to July 2024). A total of six (three ovarian and three uterine) cases diagnosed as malignant MMT were included based on morphology and confirmation by expressing positivity of cytokeratin (CK) and vimentin. All the demographic, clinical, and radiological data, treatment protocols, and survival data were obtained from the medical record department. Dako (Agilent Diagnostics and Genomics Group, Santa Clara, California) antibodies were used for immunohistochemical (IHC) analysis with 1:100 dilutions. Results The mean age of presentation was 51.5 years (35-65 years). The primary tumor site was the uterus in four cases, bilateral ovaries in one case, and unilateral ovaries in one case. Metastasis was seen in two cases. Microscopically, the tumor was biphasic, displaying high-grade carcinomatous and sarcomatous components. Pan-CK was positive in the epithelial component of the tumor, while vimentin was positive in high-grade sarcomatous components. Epithelial membrane antigen (EMA) was positive in three cases, while myogenic was positive in heterologous rhabdomyosarcomatous component in one case. The mean ki-67 was approximately 52% (30%-80%). Conclusions Carcinosarcomas are highly aggressive tumors commonly occurring in elderly females. Uterine carcinosarcomas are rare, and ovarian carcinosarcomas are even rarer. Clinical and histological features resemble high-grade endometrial carcinoma, necessitating an extensive IHC panel to narrow down differentials and confirm the diagnosis. This study highlights the histological and IHC features and emphasizes that early diagnosis plays a crucial role in disease management.

摘要

背景 癌肉瘤(恶性苗勒管混合瘤(MMT))是一种罕见的子宫肿瘤,在卵巢中更为罕见。其临床表现与高级别子宫内膜癌难以区分。在组织学上,肿瘤具有双相性,由上皮和间充质(肉瘤样)成分组成。由于需要排除常见的类似物,癌肉瘤的诊断具有挑战性,因此需要应用广泛的免疫组化标志物。

材料与方法 这项对恶性MMT的回顾性描述性研究为期三年(2021年8月至2024年7月)。根据形态学以及细胞角蛋白(CK)和波形蛋白表达阳性进行确诊,共纳入6例(3例卵巢和3例子宫)诊断为恶性MMT的病例。所有人口统计学、临床和放射学数据、治疗方案以及生存数据均从病历科获取。使用达科(安捷伦诊断与基因组学集团,加利福尼亚州圣克拉拉)抗体进行免疫组化(IHC)分析,稀释度为1:100。

结果 出现症状的平均年龄为51.5岁(35~65岁)。原发肿瘤部位4例在子宫,1例在双侧卵巢,1例在单侧卵巢。2例出现转移。显微镜下,肿瘤具有双相性,显示高级别癌性和肉瘤样成分。广谱CK在肿瘤的上皮成分中呈阳性,而波形蛋白在高级别肉瘤样成分中呈阳性。上皮膜抗原(EMA)在3例中呈阳性,而肌源性标记物在1例异源性横纹肌肉瘤样成分中呈阳性。平均Ki-67约为52%(30%~80%)。

结论 癌肉瘤是常见于老年女性的高侵袭性肿瘤。子宫癌肉瘤罕见,卵巢癌肉瘤更为罕见。临床和组织学特征类似于高级别子宫内膜癌,需要广泛的免疫组化检查来缩小鉴别范围并确诊。本研究突出了组织学和免疫组化特征,并强调早期诊断在疾病管理中起着关键作用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7779/11586871/39c71c8b0cfe/cureus-0016-00000072317-i01.jpg

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