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子宫内膜神经内分泌肿瘤:临床病理分析及文献复习的病例系列研究

Neuroendocrine Tumors of the Endometrium: a Case Series with Clinico-Pathological Analysis and Review of Literature.

作者信息

Das Sumanta, Ahlawat Sunita, Jain Priti, Panda Arun Kumar, Sarangi Jayati, Joshi Rama, Gogi Ramana, Jain Ashutosh

机构信息

Department of Pathology, Agilus Diagnostics Ltd, Fortis Memorial Research Institute, Gurugram, India.

Department Obstetrics and Gynecology, Fortis Memorial Research Institute, Gurugram, India.

出版信息

Indian J Surg Oncol. 2025 Feb;16(1):211-220. doi: 10.1007/s13193-024-02047-0. Epub 2024 Aug 17.

Abstract

Neuroendocrine carcinomas are rare aggressive tumors that are either mixed with endometrial carcinomas or pure neuroendocrine carcinoma. They show at least one or two neuroendocrine markers by immunohistochemistry. Here, we describe a case series of six cases that arise from endometrium. The mean age of presentation was 57 years with the most common type being mixed endometrioid and neuroendocrine carcinoma. Immunohistochemistry showed all cases positive for Pan-cytokeratin and INSM-1 while the majority were positive for synaptophysin and chromogranin. At least two neuroendocrine markers were positive in all cases in > 20% of tumor cells. The majority of the cases presented at FIGO (International Federation of Gynecology and Obstetrics) Stage III & IV. Three patients had a recurrence, one patient had bone metastasis, one patient died, and one patient was free of disease. Four patients with Stage IV disease were not alive for more than 18 months. A multidisciplinary approach is required for these aggressive tumors for better management of patients.

摘要

神经内分泌癌是罕见的侵袭性肿瘤,可与子宫内膜癌混合或为纯神经内分泌癌。通过免疫组织化学,它们显示至少一种或两种神经内分泌标志物。在此,我们描述了一系列6例起源于子宫内膜的病例。发病的平均年龄为57岁,最常见的类型是子宫内膜样和神经内分泌癌混合。免疫组织化学显示所有病例的全细胞角蛋白和INSM-1均为阳性,而大多数病例的突触素和嗜铬粒蛋白为阳性。在所有病例中,超过20%的肿瘤细胞中至少有两种神经内分泌标志物呈阳性。大多数病例在国际妇产科联盟(FIGO)III期和IV期就诊。3例患者复发,1例患者发生骨转移,1例患者死亡,1例患者无疾病。4例IV期疾病患者存活时间不超过18个月。对于这些侵袭性肿瘤,需要多学科方法以更好地管理患者。

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