Schaldenbrand J D, Appelman H D
Hum Pathol. 1984 Mar;15(3):229-32. doi: 10.1016/s0046-8177(84)80184-7.
Neurofibromatosis (von Recklinghausen's disease) is occasionally associated with large, solid stromal tumors of the gastrointestinal tract. The authors examined by electron microscopy two such cases of cellular spindle cell neoplasms of the small bowel histologically that resembled leiomyomas, in an attempt to clarify the cell of origin of these lesions. Ultrastructurally, the tumor cells predominantly contained moderate to large numbers of intracellular filaments, small cell processes, discontinuous adherent dense basement-membrane-like material, and abundant intercellular collagen. Definite fusiform dense bodies or structures highly suggestive of them and pinocytotic vesicles were seen in rare cells of each lesion after viewing multiple blocks. While patients with neurofibromatosis are certainly at risk of developing gastrointestinal Schwann cell neoplasms, these two cases suggest that they are also at risk for developing poorly differentiated stromal tumors, resembling leiomyomas by light microscopy, which may show only characteristic cytoplasmic differentiation of smooth muscle cells after ultrastructural examination of many sections.
神经纤维瘤病（冯雷克林霍增氏病）偶尔与胃肠道的大型实性间质瘤相关。作者通过电子显微镜检查了两例组织学上为小肠细胞性梭形细胞瘤的病例，这些病例在组织学上类似于平滑肌瘤，旨在阐明这些病变的细胞起源。在超微结构上，肿瘤细胞主要含有中等至大量的细胞内细丝、小细胞突起、不连续的附着致密基底膜样物质以及丰富的细胞间胶原。在观察多个切片后，在每个病变的罕见细胞中可见明确的梭形致密体或高度提示它们的结构以及吞饮小泡。虽然神经纤维瘤病患者肯定有发生胃肠道施万细胞瘤的风险，但这两个病例表明，他们也有发生低分化间质瘤的风险，这些低分化间质瘤在光学显微镜下类似于平滑肌瘤，在对许多切片进行超微结构检查后可能仅显示平滑肌细胞的特征性细胞质分化。
