手部恶性腱鞘巨细胞瘤临床研究

Malignant giant cell tumor of the tendon sheaths in the hand

目的研究恶性腱鞘巨细胞瘤的临床、病理、免疫组织化学特点及临床转归。方法1991年1月至2001年12月对我院诊断的10例恶性腱鞘巨细胞瘤患者的临床资料进行分析。复习患者HE切片及X线片，对石蜡包埋组织进行免疫组织化学染色和流式细胞检测。结果10例患者中3例证实为其他类型肉瘤。其余7例患者中仅有1例临床表现出明显的恶性行为：多次复发，最后出现肺部转移死亡，此患者免疫组织化学染色结果符合腱鞘巨细胞瘤的免疫表型，流式细胞检测显示异倍体；其余6例患者组织病理表现显示为恶变，其中4例有免疫组织化学检测结果，显示符合腱鞘巨细胞瘤的免疫特征，流式细胞检测显示1例DNA倍体类型为异倍体类型，3例为二倍体，S期细胞百分比分别为4．5％，11．6％和2．6％。对其肿物切除和扩大切除后，未经化疗和放疗，随访1．5—7．5年，平均4．5年，未见复发和转移。结论恶性腱鞘巨细胞瘤极为罕见，一些患者预后极差。但一些组织病理表现恶性的腱鞘巨细胞瘤，如能彻底切除，仍有较好的临床结果。

Objectives To retrospectively study on malignant giant cell tumor of tendon sheath （MGCTTS） in the hand, and to evaluate its clinical, histologic, immunohistochemical features and biologic evolution. Methods Between January 1991 and December 2001, 10 patients with histologically proven MGCTTS were treated. The clinical material, radiographs and hematoxylin and eosin-stained sections were reviewed. Immunohistochemical studies and nuclear suspensions for flow cytometry were done on paraffin embedded tissue. All patients were followed up. Results Three of 10 patients in which the diagnosis of MGCCTS was originally considered were excluded after the slides reviewed and immunohistochemical examination performed. In the other 7 patients, one showed malignant and aggressive nature： the lesion recurred several times and the patient eventually died with pulmonary metastases. The immunohistochemical profile of the patient was similar to that reported in benign GCTTS, and the flow cytometry DNA analysis detected aneuploidy. Six cases presented histologic features of malignancy, 4 of them undertook the immunohistochemical examination and their profiles were similar to that reported in benign GCTTS. An aneuploidy DNA pattern was detected in one case on flow cytometry evaluation, diploidy DNA pattern was detected in 3 cases, and their S-phase fraction was 4. 5%, 11.6% and 2. 6% respectively. All of them had a benign clinical features, they were alive and without evidence of disease from 1.5 to 7.5 years （averagely, 4. 5 years） after complete surgical excision or resections with wide surgical margins. None of them had received chemotherapy or radiation therapy. Conclusions Malignant giant cell tumor of tendon sheath is an extremely rare malignant tumor, some cases have a poor outcome, the others, despite the histologically malignant features, have a good prognosis if wide surgical excision ablates the tumor completely.