胃神经内分泌-非神经内分泌混合肿瘤:一种罕见肿瘤及其在一名年轻人身上的表现

Tânia Carvalho, Andreia Coutada, Manuel Jácome, Dália Fernandes
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引用次数: 0

摘要

导言胃癌是第四大常见癌症死因,90% 以上的病例为腺癌。在各种亚型中,神经内分泌-非神经内分泌混合肿瘤(MiNEN)是最罕见的类型之一。多达 5%的胃癌患者会出现大量出血。病例介绍:作者介绍了一例健康的 26 岁男性病例,他曾两次出现上消化道大出血,经内镜治疗后均得到缓解。在第二次内镜检查中,发现胃食管交界处有一个 15 毫米的结节状上皮下病变。内镜超声检查显示,病变部位均匀、低回声,粘膜深层界限清晰。活组织检查显示为腺癌。患者随后接受了食管远端切除术和全胃切除术,并进行了化疗。手术标本的组织学检查显示,这是一种混合型腺内分泌癌,由具有管状/腺状形态和标志环细胞的腺癌和大细胞型神经内分泌癌组成。肿瘤已浸润胃的外层肌肉,并扩散到 3 个区域淋巴结,因此被列为 IIb 期。治疗两年后,没有复发迹象。所有基因检测结果均为阴性。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,即为 MiNEN。由于其罕见性,流行病学和标准治疗方法尚未得到很好的确定,因为发表的大多数数据都来自病例报告。在这种情况下,我们提出了一个令人信服的病例研究,强调了患者的年轻、这种特殊癌症的罕见性及其不常见的表现形式。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Gastric Mixed Neuroendocrine-Non-Neuroendocrine Neoplasm: An Unusual Tumor and Its Presentation in a Young Adult
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.
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