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胃混合性神经内分泌-非神经内分泌肿瘤:一种罕见肿瘤及其在一名年轻成年人中的表现。

Gastric Mixed Neuroendocrine-Non-Neuroendocrine Neoplasm: An Unusual Tumor and Its Presentation in a Young Adult.

作者信息

Carvalho Tânia, Coutada Andreia, Jácome Manuel, Fernandes Dália

机构信息

Department of Gastroenterology, Hospital de Braga, Braga, Portugal.

Department of Pathology, Instituto Português de Oncologia do Porto/Porto Comprehensive Cancer Centre Raquel Seruca, Porto, Portugal.

出版信息

GE Port J Gastroenterol. 2024 Mar 7;31(6):432-436. doi: 10.1159/000536674. eCollection 2024 Dec.

Abstract

INTRODUCTION

Gastric cancer is the fourth most common cause of cancer death, with more than 90% of the cases being adenocarcinomas. Among the diverse subtypes, mixed neuroendocrine-non-neuroendocrine neoplasm (MiNEN) is one of the rarest types. Gastric cancer can manifest with significant bleeding in up to 5% of patients.

CASE PRESENTATION

The authors present a case of a healthy 26-year-old male who experienced two episodes of major upper gastrointestinal bleeding that were resolved with endoscopic treatment. During the second endoscopy, a 15-mm nodular subepithelial lesion was identified at the gastroesophageal junction. Endoscopic ultrasound revealed a homogeneous and hypoechoic lesion with well-defined limits in the deep mucosa. Histological examination of the biopsies showed an adenocarcinoma. The patient later underwent a distal esophagectomy and a total gastrectomy, followed by chemotherapy. Histological examination of the surgical specimen showed a mixed adenoneuroendocrine carcinoma composed of an adenocarcinoma with tubular/glandular pattern and signet ring cells and a large cell-type neuroendocrine carcinoma. The neoplasia had infiltrated the outer muscular layers of the stomach and had disseminated to 3 regional lymph nodes, leading to its classification as stage IIb. Two years following the treatment, there is no evidence of recurrence. All genetic tests applied were negative.

DISCUSSION

A MiNEN occurs when both neuroendocrine and non-neuroendocrine components represent at least 30% of the lesion. Due to its rarity, epidemiology and standard treatment are not well established because most data published are from case reports. In this context, we present a compelling case study, highlighting the patient's young age, the rarity of this specific cancer, and its uncommon presentation.

摘要

引言

胃癌是癌症死亡的第四大常见原因,超过90%的病例为腺癌。在多种亚型中,混合性神经内分泌-非神经内分泌肿瘤(MiNEN)是最罕见的类型之一。高达5%的胃癌患者可出现严重出血。

病例介绍

作者报告了一例26岁健康男性病例,该患者经历了两次上消化道大出血,经内镜治疗后出血得到缓解。在第二次内镜检查时,在胃食管交界处发现一个15毫米的结节状上皮下病变。内镜超声显示深部黏膜有一个边界清晰的均匀低回声病变。活检组织学检查显示为腺癌。患者随后接受了远端食管切除术和全胃切除术,随后进行化疗。手术标本的组织学检查显示为混合性腺神经内分泌癌,由具有管状/腺泡状结构和印戒细胞的腺癌以及大细胞型神经内分泌癌组成。肿瘤已浸润胃的外肌层,并扩散至3个区域淋巴结,因此被归类为IIb期。治疗两年后,无复发迹象。所有应用的基因检测均为阴性。

讨论

当神经内分泌和非神经内分泌成分均占病变的至少30%时,即发生MiNEN。由于其罕见性,流行病学和标准治疗方法尚未明确确立,因为大多数已发表的数据来自病例报告。在此背景下,我们展示了一个引人注目的病例研究,突出了患者的年轻、这种特定癌症的罕见性及其不常见的表现。

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