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混合性腺神经内分泌癌是一种罕见但重要的肿瘤,发现于食管。

Mixed Adenoneuroendocrine Carcinoma Is a Rare but Important Tumour Found in the Oesophagus.

作者信息

Kadhim Mohammad Murad Kasim, Jespersen Marie Louise, Pilegaard Hans Kristian, Nordsmark Marianne, Villadsen Gerda Elisabeth

机构信息

Department of Surgery, Regional Hospital Horsens, Aarhus University Hospital, 8700 Horsens, Denmark.

Department of Histopathology, Aarhus University Hospital, 8000 Aarhus C, Denmark.

出版信息

Case Rep Gastrointest Med. 2016;2016:9542687. doi: 10.1155/2016/9542687. Epub 2016 Feb 3.

Abstract

Mixed adenoneuroendocrine carcinoma (MANEC) is a rare tumour of the gastrointestinal tract that consists of a dual adenocarcinomatous and neuroendocrine differentiation, each component representing at least 30% of the tumour. We report a case of a 68-year-old man who presented with two-month history of postprandial pain and vomiting. Gastric endoscopy revealed a polypoid mass in the lower part of the oesophagus. In contrast to the majority of these tumours, this biopsy was immunohistochemically positive for chromogranin A, and synaptophysin and Ki-67 index was 50% and the tumour was diagnosed as poorly differentiated neuroendocrine carcinoma of the oesophagus. The patient underwent surgery and lower oesophagus resection was performed. Based on the histopathology and immunohistochemistry of the tumour in the oesophagogastrectomy specimen, a mixed adenoneuroendocrine carcinoma (MANEC) was diagnosed. The objective of this case report is to advocate for the focus on the MANEC diagnosis as such patients need to be referred to a centre of excellence with expertise in NET tumours, to have the correct diagnostic work-up, treatment, and secondary diagnostic procedures performed at progression, as this will have paramount influence of the choice of treatment.

摘要

混合性腺神经内分泌癌(MANEC)是一种罕见的胃肠道肿瘤,由腺癌和神经内分泌分化组成,每个成分至少占肿瘤的30%。我们报告一例68岁男性,有两个月的餐后疼痛和呕吐病史。胃镜检查显示食管下段有一个息肉样肿物。与大多数此类肿瘤不同,该活检标本嗜铬粒蛋白A免疫组化呈阳性,突触素和Ki-67指数为50%,肿瘤被诊断为食管低分化神经内分泌癌。患者接受了手术,进行了食管下段切除术。根据食管胃切除术标本中肿瘤的组织病理学和免疫组化结果,诊断为混合性腺神经内分泌癌(MANEC)。本病例报告的目的是提倡关注MANEC的诊断,因为此类患者需要转诊至在神经内分泌肿瘤方面有专业知识的卓越中心,以便在疾病进展时进行正确的诊断检查、治疗和二次诊断程序,因为这将对治疗选择产生至关重要的影响。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/878e/4756141/ff723bb0d6c5/CRIGM2016-9542687.001.jpg

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