Povýsil C, Habanec B
II. patologickoanatomický ústav 1. LF UK a VFN, Praha.
Cesk Patol. 2000 Jul;36(3):106-10.
Plexiform fibrohistiocytic tumor is a rare lesion which has been reported only in the dermis and subcutis so far. We present two cases in this location and an additional one localized in the proximal epiphysis of the tibia. A case with crural location showed predominance of plexiform tumorous nodes with fibrohistiocytic arrangement. The nodes were composed of spindle-shaped myofibroblasts with admixture of histiocytes and giant multinucleated osteoclast-like cells. In another case the tumor of axillary soft tissues featured mostly plexiform bundles of spindle-shaped myofibroblast-like cells reacting positively with actin and CD 68 antibodies; ultrastructurally, they contained numerous dense lysosomal inclusions with myelin figures. The bone tumor was composed of hyalinized fibroblastic component with disperse fibrohistiocytic nodes. Despite a semimalignant character of the lesion there was no recurrence in our cases during the 2-6 year postoperative period.
丛状纤维组织细胞瘤是一种罕见的病变，迄今为止仅在真皮和皮下组织中有报道。我们在此部位报告了两例病例，另外还有一例位于胫骨近端骨骺。一例位于小腿的病例显示以丛状肿瘤结节为主，呈纤维组织细胞样排列。这些结节由梭形肌成纤维细胞组成，混有组织细胞和巨大的多核破骨细胞样细胞。另一例腋窝软组织肿瘤主要表现为梭形肌成纤维细胞样细胞的丛状束，对肌动蛋白和CD 68抗体呈阳性反应；超微结构上，它们含有许多带有髓鞘样结构的致密溶酶体包涵体。骨肿瘤由玻璃样变的纤维母细胞成分和散在的纤维组织细胞结节组成。尽管该病变具有半恶性特征，但在我们的病例中，术后2至6年期间均未复发。
