低度恶性黏液纤维肉瘤一例并文献复习

Low grade Myxofibrosarcoma： a case report and literature review

目的研究低度恶性黏液纤维肉瘤的临床特征、病理、分型、预后及治疗进展。方法对1例低度恶性黏液纤维肉瘤患者的临床资料、影像学表现、病理、治疗和预后进行报道，并结合国内外文献复习近年来有关低度恶性黏液纤维肉瘤的诊治进展。结果临床表现为四肢或躯干无痛缓慢生长的肿块，活检病理示：肿瘤细胞呈梭形浸润生长，呈结节状，可见黏液样背景及多核巨细胞、核分裂像，Vim（＋）、SMA（＋）、S-100（-）、Ki-67（10％．20％＋）、CD34（血管＋）、CD68（＋）。结论低度恶性黏液纤维肉瘤较少见，易复发，可发生转移，病理上易与其他黏液变的良恶性肿瘤相混淆，故正确的诊断和及时采取以手术切除为主的综合性治疗十分重要。

Objective To study the clinical characteristics, pathology, classification, prognosis and treatment of myxofibrosarcoma. Methods The clinical data, imaging findings, pathology, treatment and prognosis of the patient with low grade myxofibrosarcoma was analyzed. Results It frequently presents as a slowly enlarging painless mass often affects the extremities. An excisional biopsy showed spindle-shaped invasive growth of tumor cells in the loose myxoid stroma with muhinucleated giant cells and mitotic. Immunohistochemical staining showed that the tumor cells were positive for the markers Vim （ ＋ ） ,SMA（ ＋ ） ,S-100（ - ） ,Ki-67（10% -20% ＋ ） ,CD34（ ＋ ） ,CD68（ ＋ ）. Conclusions MFS is a very rare soft tissue tumor that is often misdiagnosed as benign, and need to be differentiated from other tumor. Definite diagnosis of the malignancy depends on pathological examination. Low grade myxofibro- sarcoma is malignancy with surgical resection mainly combined with postoperative radiotherapy and chemotherapy treatments if necessary.