弥漫性腱鞘巨细胞瘤伴腹膜后巨大肿块1例并文献复习

Diffuse-type tenosynovial giant cell tumor of hip presenting asa retroperitoneal mass:a case report and review of literature

目的 分析弥漫性腱鞘巨细胞瘤 (D TGCT)的临床病理特征。方法 结合文献对 1例罕见的伴腹膜后巨大肿块的D TGCT进行临床、病理形态及免疫组化研究。结果 D TGCT主要由单核细胞组成 ,并见散在的多核巨细胞和含铁血黄素沉积 ,可见单核细胞的梭形化及滑膜样裂隙。在小的组织细胞间可见散在的树突状组织细胞 ,免疫组化 :CD68(- ) ,desmin(+)。结论 D TGCT呈浸润性生长 。

Objective To investigate the clinical and patho logical features of diffuse-type tenosynovial giant cell tumor (D-TGCT) Methods Clinical data, pahological features and immunohistoch emical findings were observed with review of the literature, and differential di agnosis was discussed in one case of D-TGCT Results Uunusual case of D-TGCT involvd in the hip joint pre senting as a large retroperitoneal mass was described. Invasion of the acetabul uni and fovea of the femoral head were also present Histologic analysis showed a prominent mononuclear cell infiltrate, multinucleated giant cells, foamy macr ophages, intra-and extracellular hemosiderin deposits, and stromal fibrosis I n addition to the predominant histiocyte-like cells, a subpopulation of large d endritic, desmin-positive cell was identified in this cases Conclusions D-TGCT has poorly circumscribed borders and infi ltrative margins The cell populations are not uniformly distributed throughout the lesion and, in particular, giant cells, foam cells and hemosiderin deposits can vary regionally and may be difficult to identify