Herrera G A, de Moraes H P
Virchows Arch A Pathol Anat Histopathol. 1984;403(4):361-76. doi: 10.1007/BF00737286.
Thirteen soft tissue neurogenic sarcomas from twelve patients with neurofibromatosis (Von Recklinghausen's disease) were ultrastructurally examined. Electron microscopic studies revealed a wide spectrum of morphological manifestations varying from schwannian to fibroblastic, histiocytic, fibrohistiocytic and relatively undifferentiated cellular proliferations. A similar variation on light microscopic appearances has been previously reported in these neurogenic sarcomas. Neurogenic sarcomas occurring in patients with neurofibromatosis (Von Recklinghausen's disease), represent a heterogeneous group of neoplasms with various patterns of differentiation identified ultrastructurally. The morphologic expressions of these neurogenic neoplasms can be conceptualized as a disorderly growth of the various peripheral nerve cellular components, or, as has been previously suggested, as a result of the multipotential nature and metaplastic ability of Schwann cells. S-100 protein immunohistochemistry was only positive in those neoplasms ultrastructurally proven to represent schwannian cellular proliferations. This study serves to document the range of fine structure that may be found in neurogenic sarcomas, to correlate the ultrastructural findings with the light microscopic appearance of these tumors, to determine the specificity of the electron microscopic findings, and immunohistochemistry for S-100 protein and assess their possible value in differential diagnosis.
对12例患有神经纤维瘤病（冯雷克林霍增氏病）患者的13例软组织神经源性肉瘤进行了超微结构检查。电子显微镜研究显示出广泛的形态学表现，从雪旺氏细胞型到成纤维细胞型、组织细胞型、纤维组织细胞型以及相对未分化的细胞增殖。先前在这些神经源性肉瘤中已报道过光镜下表现的类似变化。发生于神经纤维瘤病（冯雷克林霍增氏病）患者的神经源性肉瘤代表了一组异质性肿瘤，超微结构显示其具有各种分化模式。这些神经源性肿瘤的形态学表现可被概念化为各种周围神经细胞成分的无序生长，或者如先前所提示的，是雪旺细胞多潜能性质和化生能力的结果。S-100蛋白免疫组织化学仅在超微结构证实为雪旺氏细胞增殖的那些肿瘤中呈阳性。本研究旨在记录神经源性肉瘤中可能发现的精细结构范围，将超微结构发现与这些肿瘤的光镜表现相关联，确定电子显微镜发现的特异性，以及S-100蛋白免疫组织化学，并评估它们在鉴别诊断中的可能价值。
