Aktekın Ali, Özkara Selvinaz, Merıç Kaan, Günay Gürleyık Meryem, Aker Fügen, Sağlam Abdullah
Department of General Surgery, Haydarpaşa Numune Education and Research Hospital, İstanbul, Turkey.
Turk J Gastroenterol. 2012 Aug;23(4):385-9. doi: 10.4318/tjg.2012.0403.
Plexiform schwannoma is a benign peripheral nerve sheath tumor and is composed of Schwann cells arranged in a plexiform pattern. Most plexiform schwannomas are skin tumors, and there has been no case report of this tumor originating in the duodenum. We describe the first known case of plexiform schwannoma of the duodenum. A 60-year-old man presented with a short history of food intolerance, epigastric discomfort, fullness and bloatedness, sometimes vomiting, and weight loss, without any clinical picture of neurofibromatosis. Upper gastrointestinal endoscopy revealed pyloric stenosis with normal mucosal lining. The computed tomography demonstrated circumferentially and concentrically thickened pylorus up to 18 mm with narrowed lumen and limited contrast passage. Antrectomy and gastrojejunostomy were performed due to unknown etiology of the obstruction. The cut surface of the lesion revealed thickened pylorus up to 15 mm in a circumferential manner. It contained a 5 mm tumor consisted of multiple white nodules in the submucosal and subserosal layers with overlying duodenal mucosa. Microscopic examination revealed nodular structures composed of spindle cells within fascicular pattern without any atypia or mitosis. Immunohistochemical examination revealed that the cells diffusely and strongly expressed S100 proteins in a nuclear and cytoplasmic pattern, but not CD117, smooth muscle actin, desmin, or CD34, confirming plexiform schwannoma.
丛状神经鞘瘤是一种良性周围神经鞘膜肿瘤,由呈丛状排列的施万细胞组成。大多数丛状神经鞘瘤是皮肤肿瘤,尚无该肿瘤起源于十二指肠的病例报告。我们描述了首例已知的十二指肠丛状神经鞘瘤病例。一名60岁男性,有食物不耐受、上腹部不适、饱胀感和腹胀的病史较短,有时呕吐,体重减轻,无任何神经纤维瘤病的临床表现。上消化道内镜检查显示幽门狭窄,但黏膜正常。计算机断层扫描显示幽门周向和同心性增厚至18毫米,管腔狭窄,造影剂通过受限。由于梗阻病因不明,进行了胃窦切除术和胃空肠吻合术。病变的切面显示幽门周向增厚至15毫米。其包含一个5毫米的肿瘤,由黏膜下层和浆膜下层的多个白色结节组成,上方覆盖十二指肠黏膜。显微镜检查显示由束状排列的梭形细胞组成的结节状结构,无任何异型性或有丝分裂。免疫组织化学检查显示细胞以核和胞质模式弥漫性、强烈表达S100蛋白,但不表达CD117、平滑肌肌动蛋白、结蛋白或CD34,证实为丛状神经鞘瘤。