Kato K, Ijiri R, Tanaka Y, Hara M, Sekido K
Division of Pathology, Kanagawa Children's Medical Center, Yokohama, Japan.
Pathol Int. 1999 Aug;49(8):731-6. doi: 10.1046/j.1440-1827.1999.00933.x.
The first Japanese case of nasal chondromesenchymal hamartoma, a rare infantile nasal neoplasm, is presented. A 4-month-old Japanese boy was referred to our Centre because of intranasal mass and oculomotor disturbance. Radiological images showed the intranasal tumor extending to the paranasal sinus, orbit, and anterior frontal fossa. Subtotal resection and radiation therapy to residual tumor were performed. There has been no recurrence of the tumor for 13 years. Histologically, the lesion demonstrated admixture of various mesenchymal elements, including cellular spindle cell stroma with occasional myxoid change, nodules of mature/immature cartilaginous tissue, focal osteoclast-like giant cells, and erythrocyte-filled spaces resembling aneurysmal bone cyst. The histology was consistent with the findings presented by McDermott et al. 1986. Immunohistochemically, the spindle cells were positive for vimentin and smooth muscle actin. Chondrocytes in the mature cartilaginous tissue were positive for S-100 and vimentin; chondrocytes in the immature cartilaginous tissue were positive for S-100, vimentin, and smooth muscle actin. Ultrastructurally, the spindle cells showed features of either fibroblast or myofibroblast.
本文报道了日本首例鼻软骨间叶性错构瘤，这是一种罕见的婴儿期鼻腔肿瘤。一名4个月大的日本男孩因鼻腔肿物和动眼神经功能障碍被转诊至我院。影像学检查显示鼻腔肿瘤延伸至鼻窦、眼眶和额前窝。对其进行了次全切除，并对残留肿瘤进行了放射治疗。13年来肿瘤未复发。组织学检查显示，病变呈现出各种间叶成分的混合，包括伴有偶尔黏液样改变的细胞性梭形细胞基质、成熟/未成熟软骨组织结节、局灶性破骨细胞样巨细胞以及类似动脉瘤样骨囊肿的充满红细胞的间隙。组织学检查结果与McDermott等人1986年报道的一致。免疫组织化学检查显示，梭形细胞波形蛋白和平滑肌肌动蛋白呈阳性。成熟软骨组织中的软骨细胞S-100和波形蛋白呈阳性；未成熟软骨组织中的软骨细胞S-100、波形蛋白和平滑肌肌动蛋白呈阳性。超微结构检查显示，梭形细胞具有成纤维细胞或肌成纤维细胞的特征。
