Ishida T, Wada I, Horiuchi H, Oka T, Machinami R
Department of Pathology, Faculty of Medicine, University of Tokyo, Japan.
Pathol Int. 1996 Sep;46(9):689-95. doi: 10.1111/j.1440-1827.1996.tb03673.x.
A case of multiple small intestinal stromal tumors (SIST) with skeinoid fibers of the jejunum arising in a 50 year old male with neurofibromatosis 1 (NF-1) is reported. Seven small tumors of the jejunal wall were incidentally found and excised during an operation for abdominal and retroperitoneal neurofibromas. Histologically, the tumors were composed of uniform spindle-shaped cells with fascicular pattern, almost indistinguishable from the histology in leiomyoma. Periodic acid Schiff stain-positive hyaline globules were observed among the tumor cells. Ultrastructurally, these globules were stromal tangles of curvilinear, fluffy fibrils, consistent with skeinoid fibers. The electron-dense granules, possibly neuro-secretory granules, were found in the cytoplasm of the tumor cells. Immunohistochemically, the tumor cells were positive for vimentin, neuron specific enolase and CD34, but negative for muscle markers and S100 protein. The association of NF-1 and multiple SIST with skeinoid fibers may have clinical implications. The multiple occurrence of SIST with skeinoid fibers seems to be often cited as one of the gastrointestinal manifestations of NF-1. The possible site of origin of SIST with skeinoid fibers in NF-1 may be the enteric autonomic nerve plexus in the small intestinal wall.
报告了一例50岁患有神经纤维瘤病1型（NF-1）的男性空肠出现伴有束状纤维的多发性小肠间质瘤（SIST）。在一次腹部和腹膜后神经纤维瘤手术中，偶然发现并切除了7个空肠壁小肿瘤。组织学上，肿瘤由形态一致的梭形细胞组成，呈束状排列，在组织学上与平滑肌瘤几乎无法区分。在肿瘤细胞中观察到过碘酸希夫染色阳性的透明小球。超微结构上，这些小球是由曲线状、蓬松的原纤维组成的间质缠结，与束状纤维一致。在肿瘤细胞的细胞质中发现了电子致密颗粒，可能是神经分泌颗粒。免疫组织化学显示，肿瘤细胞波形蛋白、神经元特异性烯醇化酶和CD34呈阳性，但肌肉标志物和S100蛋白呈阴性。NF-1与伴有束状纤维的多发性SIST之间的关联可能具有临床意义。伴有束状纤维的SIST的多发似乎常被认为是NF-1的胃肠道表现之一。NF-1中伴有束状纤维的SIST的可能起源部位可能是小肠壁的肠自主神经丛。
