孤立性纤维性小肠壁肿瘤与肠系膜神经内分泌肿瘤共存1例报告

C. Salazar, Virian D. Serei, M. Grandhi, Zhongren Zhou
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摘要

背景:小肠壁孤立性纤维瘤(SFT)是一种罕见的疾病,迄今为止只有一例报告。据我们所知,在英国文献中,肠系膜神经内分泌肿瘤附近小肠壁的SFT尚未报道。病例介绍:患者是一名82岁的男性,在膀胱超声检查中偶然诊断为右肾周肿块,用于治疗慢性尿路感染。腹部和骨盆的后续CT显示一个位于胰头右侧和十二指肠前方的肿块。随后的内镜超声(EUS)和细针活检诊断为低度神经内分泌肿瘤,CD56、突触素和嗜铬粒蛋白阳性支持了这一诊断。进行了剖腹探查,在中结肠血管根部附近发现了肠系膜肿块,该肿块位于十二指肠和胰头顶部。右半结肠切除术和回肠末端切除术与肠系膜肿块切除术整体进行,推测为大淋巴结转移。小肠粘膜下层内发现一个白色、坚硬的小结节,大小为0.6 x 0.4 x 0.3 cm。肠系膜肿块大小为5.5 x 3.5 x 3.3 cm,位于小肠的肠系膜引流分布中。肠系膜肿瘤细胞突触素和嗜铬粒蛋白阳性,支持神经内分泌肿瘤的诊断。粘膜下小结节细胞STAT6、CD34和CD99阳性,局部BCL-2阳性,证实了小肠SFT的诊断。结论:我们报告了第一例小肠黏膜下层内的SFT与肠系膜内的大型神经内分泌肿瘤共存的病例。
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Coexistence of Solitary Fibrous Tumor in the Small Bowel Wall with Mesentery Neuroendocrine Tumor: A First Case Report
Background: Solitary fibrous tumor (SFT) of the small bowel wall is a rare occurrence with only one case reported to date. SFT in the small bowel wall adjacent to mesenteric neuroendocrine tumor has not been reported, to the best of our knowledge, in the English literature. Case Presentation: The patient is an 82-year old male with a right perinephric mass incidentally diagnosed during a bladder ultrasound for working-up of chronic urinary tract infections. A follow-up CT of the abdomen and pelvis demonstrated a mass located to the right of the pancreatic head and anterior to the duodenum. A subsequent endoscopic ultrasound (EUS) with fine needle biopsy of the mass diagnosed a low-grade neuroendocrine tumor, which was supported by positivity for CD56, synaptophysin, and chromogranin. An exploratory laparotomy was performed, and the mesenteric mass was identified near the root of the middle colic vessels and laying on top of the duodenum and pancreatic head. A right hemicolectomy with terminal ileum resection was performed en bloc with resection of the mesenteric mass, presumed to be a large lymph node metastasis. A small white-tan, firm nodule was located within the small bowel submucosa and identified, measuring 0.6 x 0.4 x 0.3 cm. The mesenteric mass measured 5.5 x 3.5 x 3.3 cm and was in the mesenteric drainage distribution of the small bowel. The mesentery tumor cells were positive for synaptophysin and chromogranin, which supported the diagnosis of neuroendocrine tumor. The small submucosal nodule cells were positive for STAT6, CD34 and CD99, and focally positive for BCL-2, which confirmed the diagnosis of SFT of the small bowel. Conclusion: We report the first case of SFT within the small bowel submucosa coexisting with a large neuroendocrine tumor within the mesentery.
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