腱鞘巨细胞瘤clusterin表达对病理诊断和组织发生的意义

Value of clusterin expression in pathologic diagnosis and histogenesis of giant cell tumor of tendon sheath

目的通过对腱鞘巨细胞瘤（GCTS）的免疫组织化学研究，分析各型GCTS中各种细胞成分的免疫表型，探讨clusterin对GCTS的病理鉴别诊断和组织发生的研究价值。方法收集上海交通大学附属第六人民医院病理科2003年1月至2008年12月间104例GCTS，用EnVision法检测各种类型GCTS中不同细胞成分对clnsterin、Ki－67、CD163、CD68、结蛋白、p63、CD35和p53的表达差异。结果104例GCTS中局限型（L—GCTS）44例（42．3％）、弥漫型（D—GCTS）32例（30．8％）、色素绒毛结节性滑膜炎（PVNS）26例（25．0％）、恶性（M—GCTS）2例（1．9％）。各型GCTS女性均多于男性，总的男女之比为38：66。L—GCTS好发于小关节（90．9％，40／44），D—GCTS、PVNS和M—GCTS好发于大关节[68．8％（22／32）、100％（26／26）、2／2]。随访74例，其中L—GCTS、D—GCTS、PVNS和M—GCTS的复发率分别为30．3％（10／33）、30．4％（7／23）、18．8％（3／16）和2／2。各型GCTS的细胞构成基本相同，有大的滑膜样单核细胞、小的组织细胞样单核细胞、泡沫状组织细胞、以淋巴细胞为主的炎性细胞、纤维母细胞和破骨细胞样多核巨细胞。GCTS中的各种细胞成分免疫表型有明显差异：大的滑膜样单核细胞clusterin强阳性，部分表达结蛋白和Ki-67，不表达CD163。小的组织细胞样单核细胞CD163强阳性，不表达clusterin和结蛋白。破骨细胞样多核巨细胞CD68强阳性，不表达elusterin、CD163、Ki一67和结蛋白。正常滑膜细胞clusterin强阳性，部分表达结蛋白。D—GCTS中elustetin强阳性的滑膜样单核细胞数量明显多于L—GCTS（P〈0．01）和PVNS（P〈0．05）。结论GCTS是一种滑膜肿瘤，并非纤维组织细胞肿瘤，其真正的肿瘤细胞可能是大的滑膜样单核细胞，这种细胞的数量在D—GCTS中明显多于L—GCTS和PVNS，可能是D—GCTS的生物学行为更具有侵袭性、破坏性和复发性的重要原因。clusterin有助于CCTS的病理鉴别诊断。

Objective Analyze the immunophenotype of the different cells in the various subtypes of giant cell tumor of tendon sheath （GCTS） and investigate the value of clusterin in pathological diagnosis and histogenesis of giant cell tumor of tendon shealh. Methods A total of 104 cases of GCTS from the surgical pathology files of Shanghai Jiaotong university affiliated the sixth people＇s hospital were identified. Immunohistoehemistry （IHC） for elusterin, desmin, CD163, CD68, p63, p53, Ki-67 and CD35 was performed on all cases, using EnVision technique. Results All cases of GCTS were researched, including 44 eases of localized type （L-GCTS）, 32 cases of diffused type （D-GCTS）, 26 eases of pigmented villonodular synovitis （PVNS） and 2 eases of malignant type. There was a slight female predominance in all these subtypes, and the male to female ratio was about 38： 66. L-GCTS usually oeeured within the small joints（90. 9% ,40/44） , while D-GCTS, PVNS and M-GCTS commonly oecured within the large weightbearing joints I68- 8% （22/32）, 100% （26/26） and 2/2 respectively 1. Of 74 eases with follow-up, the recurrence rates of L-GCTS, D-GCTS, PVNS and M-GCTS respectively were 30. 3% （10/33） , 30.4% （7/23） , 18. 8% （3/16） and 2/2. The different subtypes of GCTS had the same cell components, including the large synovial-like mononuelear cells, the small histioeytoid cells, foamy histioeytes cells, inflammatory cells, fibroblasts and the osteoclast-like muhinucleated giant cells. There were obvious differences among immunophenotype of the various cell components in GCTS： the large synovial-like mononuclear cells were strong positive for clusterin, partly positive for desmin and Ki-67, and negative for CD163. The small histiocytoid cells were strong positive for CD163 but negative for clusterin and desmin. The osteoclast-like muhinucleated giant cells were strong positive for CD68 but negative for clusterin, CD163 and desmin. Normal synoviocytes were strong positive for clusterin, partly positive for desmin. The number of the large synovial-like mononuclear cells that were positive for clusterin in D-GCTS were more than that in L-GCTS （ P 〈 0. 01 ） and PVNS （ P 〈 0. 05 ）. Conclusions GCTS was synovial tumors, not belonged to the category of fibrohistiocytic lesions. The true tumor cells may be the large synovial-like mononuclear cells, and the number of the ceils in the D-GCTS was obviously more than that in L-GCTS and PVNS. This may be the reason that the biological behavior of D-GCTS was more aggressive, destructive and recurrent. Clusterin was an useful marker in pathological differential diagnosis of GCTS.