摘要
[目的]总结肌纤维母细胞肉瘤的临床及组织病理特点,探讨其诊疗方法及预后情况。[方法]分析2例罕见的肌纤维母细胞肉瘤的临床特征、组织病理学特点、诊断治疗方法,并对患者的预后情况进行随访。同时复习近年来的国内外相关文献。[结果]患者1在活检诊断后,行大腿中段截肢术,术后在当地医院行化疗(具体方案不详)。术后1年,患者因肺转移,死亡。患者2行肿瘤切除术,术后3个月肿瘤复发,再次行手术切除并行化疗,术后11个月发生肺转移,3个月后死亡。两例最终病理诊断均为肌纤维母细胞肉瘤。复习近30年的文献,共有54例同样的病例报道。患者中以成年男性多见,肿瘤好发于头颈部、躯干以及四肢。逐渐增大的无痛性包块是其最常见的临床表现。依照特殊的形态学特征及肌源性标记物,即可以作出诊断。[结论]肌纤维母细胞肉瘤是一种罕见的恶性梭形细胞肉瘤,具体发病率不详。诊断时应与滑膜肉瘤、平滑肌肉瘤和纤维肉瘤等鉴别。治疗以手术切除为主,化疗效果不肯定。该肿瘤为低度恶性,具有侵袭性,术后局部复发率高,可以发生远处转移。预后尚不肯定。
[ Objective] To investigate the clinical and histopathologieal characteristics of myofibrosarcoma, and study its treatment and prognosis. [ Method ] The clinical features, histopathologieal characteristics, diagnosis, treatment and prognosis of two rare myofibrosarcoma patients were observed, and relative literatures were reviewed. [ Result] After biopsy, the frist patient was treated with amputation in her middle thigh, postoperative chemotherapy was performed in the local hospital, but the drug and its dose didn＇t know in detail, one year latter, the patient died because of lung metastasis. The second patient was treated with tumor resection, but three months latter, the tumor recurred and was resected again, and the patient accepted chemotherapy, eleven nonthns latter, lung metastasis was found, then the patient died. About 54 patients with myofibrosarcoma were found in the literatures published in recent years. This neoplasms often involved the head and neck region, trunk and extremities in male patient A expandable and painless mass is the chief clinical features. Myofibrosarcoma can be identified by its specific morphologie features and positive myoid markers. [ Conclusion ] Myofibrosarcoma is a rare spindle cell neoplasm, Its morbidity dosen＇t know in detail. When making diagnisis, it is important to distinguished from synovial sarcoma, fibrosarcoma and leiomyosarcoma et al, Surgical resection is the mainstay of therapy, but the result of chemotherapy is not affirmed, The tumor is lower grade malignant one, and is invariable. The rate of local recurrence is high, and distant metastasis could be found. Its prognosis is not affirmed.
出处
《中国矫形外科杂志》
CAS
CSCD
北大核心
2007年第9期666-668,680,共4页
Orthopedic Journal of China
