Zhang Wei-Guang, Xu Liang-Bi, Xiang Yi-Ning, Duan Chen-Hong
Department of Endoscopy, the Affiliated Hospital of Guizhou Medical University, Guiyang 550001, Guizhou Province, China.
Department of Pathology, the Affiliated Hospital of Guizhou Medical University, Guiyang 550001, Guizhou Province, China.
World J Clin Cases. 2018 Dec 6;6(15):1067-1072. doi: 10.12998/wjcc.v6.i15.1067.
Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have mainly occurred in the gastric antrum and pylorus region, with some cases in the duodenum.
We report here a case of plexiform fibromyxoma in the upper segment of the jejunum, which was continuously followed up for 3 years after surgical removal. Plexiform fibromyxoma showed multinodular or plexiform growth. The cells in the tumor node were spindle-shaped but few in number and mitotic figures. Small blood vessels and mucous matrix were found among the tumor cells. Immunohistochemistry revealed that the plexiform fibromyxoma cells were positive for smooth muscle actin, focally positive for CD10, and negative for cytokeratin, CD117, DOG-1 (discovered on GIST-1) desmin, S-100, epithelial membrane antigen, and CD34. Ki-67 labeling index was < 5%. Plexiform fibromyxoma showed benign biological behavior. After 3 years of consecutive postoperative follow-up, no obvious signs of metastasis or recurrence were found by imaging examination.
Plexiform fibromyxoma is a rare type of mesenchymal tumor. The diagnosis mainly depends on pathological examination, and it should be distinguished from other gastrointestinal mesenchymal tumors.
丛状纤维黏液瘤是一种罕见的特殊类型间叶组织肿瘤。最常见的临床表现为贫血、呕血和便血，无性别或年龄倾向。报道的病例主要发生在胃窦和幽门区，少数病例发生在十二指肠。
我们在此报告一例空肠上段丛状纤维黏液瘤，手术切除后连续随访3年。丛状纤维黏液瘤呈多结节状或丛状生长。肿瘤结节内的细胞呈梭形，但数量较少，未见核分裂象。肿瘤细胞间可见小血管和黏液基质。免疫组化显示，丛状纤维黏液瘤细胞平滑肌肌动蛋白阳性，CD10局灶性阳性，细胞角蛋白、CD117、DOG-1（在胃肠道间质瘤-1上发现）、结蛋白、S-100、上皮膜抗原和CD34阴性。Ki-67标记指数<5%。丛状纤维黏液瘤表现为良性生物学行为。术后连续随访3年，影像学检查未发现明显转移或复发迹象。
丛状纤维黏液瘤是一种罕见的间叶组织肿瘤。诊断主要依靠病理检查，应与其他胃肠道间质瘤相鉴别。
