Shimizu T, Teshima S, Fukushima N, Ebihara Y
Division of Pathology, Fraternity Memorial Hospital, Tokyo, Japan.
Pathol Int. 1994 Jul;44(7):551-8. doi: 10.1111/j.1440-1827.1994.tb02607.x.
A long-term (24 years) follow-up case of extraskeletal Ewing's sarcoma is reported. The light microscopic examination showed features hardly indistinguishable from Ewing's sarcoma of the bone, that is, the tumor cells were diffusely arranged and uniform in size and shape, and possessed glycogen in the cytoplasm. Homer-Wright rosettes were found only in the autopsy material. An immunohistochemical study using a neural marker (neuron-specific enolase) demonstrated positive staining in most tumor cells. An ultrastructural study revealed intracytoplasmic glycogen particles and incomplete neural characters as follows: the cytoplasmic processes resembled neural processes without neurosecretory granules, microtubules or neurofilaments. These findings suggest that this case finally acquired an incomplete neural character with repeated recurrence. This tumor was diagnosed extraskeletal Ewing's sarcoma, but in future it may be categorized as primitive neuroectodermal tumor (PNET).
报道了1例骨外尤文肉瘤的长期（24年）随访病例。光镜检查显示其特征与骨尤文肉瘤几乎难以区分，即肿瘤细胞呈弥漫性排列，大小和形状一致，细胞质内含有糖原。仅在尸检材料中发现霍纳-赖特菊形团。使用神经标志物（神经元特异性烯醇化酶）的免疫组织化学研究显示大多数肿瘤细胞呈阳性染色。超微结构研究揭示了胞质内糖原颗粒和不完全的神经特征如下：细胞质突起类似于神经突起，但没有神经分泌颗粒、微管或神经丝。这些发现表明，该病例经反复复发最终获得了不完全的神经特征。该肿瘤被诊断为骨外尤文肉瘤，但未来可能会归类为原始神经外胚层肿瘤（PNET）。
