Department of Internal Medicine, Division of Gastroenterology, Hepatology, and Nutrition, The University of Texas Health Science Center at Houston, Houston, TX 77030, USA.
Dig Dis Sci. 2011 Apr;56(4):951-7. doi: 10.1007/s10620-010-1404-1. Epub 2010 Sep 18.
Small cell carcinomas are among the most aggressive, poorly differentiated, and highly malignant of the neuroendocrine tumors (NETs). Of which, small cell gastric carcinoma is a rare small cell neuroendocrine tumor. The purpose of our study was to present this case and perform a comprehensive literature review.
We review a case of small cell gastric carcinoma that is particularly unusual in that it occurred in a woman from the US when the majority of cases of small cell gastric carcinoma have been reported in men from East Asia, and more specifically, from Japan. The diagnosis was made after endoscopy revealed a large ulcerated mass in the gastric cardia of Borrmann type 3. Biopsies revealed multiple small basophilic cells underlying the squamous epithelium of the esophagus and cardiac mucosa, indicating the presence of a tumor at the gastroesophageal junction. Immunostaining established the diagnosis with positive stains for chromogranin, synaptophysin, and CD56. Our patient is being treated with chemotherapy, but many different treatment regimens have been tried for small cell gastric carcinoma with variable success.
Overall prognosis for small cell gastric carcinoma is dismal. Neuroendocrine tumors in general have variable clinical behaviors and prognosis is dependent on the neuroendocrine tumor type. The adoption of a standardized classification system for neuroendocrine tumors could improve the recognition of infrequently encountered neuroendocrine tumors like small cell gastric carcinoma and will enhance strategies for treatment and thus improve prognosis for patients with these rare and aggressive tumors.
小细胞癌是神经内分泌肿瘤(NETs)中最具侵袭性、分化最差和高度恶性的肿瘤之一。其中,胃小细胞癌是一种罕见的小细胞神经内分泌肿瘤。我们研究的目的是报告这一病例并进行全面的文献复习。
我们回顾了一例胃小细胞癌病例,该病例特别不寻常,因为它发生在美国女性中,而大多数胃小细胞癌病例报告都来自东亚男性,更具体地说,来自日本。诊断是在内镜检查发现胃贲门处 Borrmann 3 型的大溃疡肿块后做出的。活检显示食管和贲门黏膜的鳞状上皮下有多个小嗜碱性细胞,表明胃食管交界处存在肿瘤。免疫组化染色显示嗜铬粒蛋白、突触素和 CD56 阳性,从而确立了诊断。我们的患者正在接受化疗,但对于胃小细胞癌,已经尝试了许多不同的治疗方案,但成功率不一。
胃小细胞癌的总体预后较差。一般来说,神经内分泌肿瘤的临床表现和预后各不相同,取决于神经内分泌肿瘤的类型。采用标准化的神经内分泌肿瘤分类系统可以提高对胃小细胞癌等罕见神经内分泌肿瘤的认识,并增强治疗策略,从而改善这些罕见且侵袭性肿瘤患者的预后。