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骶骨含巨细胞的“纤维性”病变。三例病例的X线、病理及超微结构研究

Giant-cell containing "fibrous" lesion of the sacrum. A roentgenographic, pathologic, and ultrastructural study of three cases.

作者信息

Dehner L P, Risdall R J, L'Heureux P

出版信息

Am J Surg Pathol. 1978 Mar;2(1):55-70. doi: 10.1097/00000478-197803000-00007.

Abstract

Three similar and unique, predominantly osteolytic lesions are reported in young patients between the ages of 4 and 19 years. Progressive back pain and neurologic deficits were the principal clinical features. The initial roentgenographic impression was that of a malignant tumor either primary or metastic. Despite incomplete resections of a firm, intraosseous and extradural tumor, healing has occurred during the follow-up interval with one interim exception. A spindle-cell stroma containing a variable number of giant cells was the consistent microscopic finding. Ultrastructural studies of one case revealed that the mononuclear spindle cells and giant cells contained cytoplasmic microfilaments similar to those of the myofibroblast. The possible relationship of this tumor to nodular fasciitis and desmoplastic fibroma is hypothesized in the light of the role of the myofibroblast in the fibromatoses and nodular fasciitis.

摘要

本文报告了3例年龄在4至19岁之间的年轻患者,其病变相似且独特,主要为溶骨性病变。进行性背痛和神经功能缺损是主要的临床特征。最初的X线印象是原发性或转移性恶性肿瘤。尽管对一个坚硬的骨内和硬膜外肿瘤进行了不完全切除,但在随访期间除了一个临时例外情况外均已愈合。一致的显微镜下表现是含有数量不等的巨细胞的梭形细胞基质。对1例病例的超微结构研究显示,单核梭形细胞和巨细胞含有与肌成纤维细胞相似的细胞质微丝。鉴于肌成纤维细胞在纤维瘤病和结节性筋膜炎中的作用,推测了这种肿瘤与结节性筋膜炎和促结缔组织增生性纤维瘤的可能关系。

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