罕见部位低度恶性纤维黏液样肉瘤3例临床病理观察  

Low-grade fibromyxoid sarcoma in rare locations:3 cases report and review of literature

作　　者：蒙国照[1] 丁宇[2] 陆明深[3] 陈贤永[1] 

机构地区：[1]湘南学院病理学教研室/附属医院病理科,湖南郴州423000 [2]粤北人民医院病理科,广东韶关512026 [3]桂林医学院附属医院病理科,广西桂林541001

出　　处：《诊断病理学杂志》2016年第1期57-61,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨低度恶性纤维黏液样肉瘤（LGFMS）的临床病理特征及鉴别诊断。方法收集3例发生于罕见部位的LGFMS，对其临床表现、影像学特点、组织学特征、免疫组化及预后进行分析。结果3例LGFMS分别发生于上颌翼腭窝、乳腺和腹膜后。肿瘤边界清楚，包膜完整，最大直径3～7cm；切面灰白、灰红色，局部区域有黏液。镜下见肿瘤组织由纤维母细胞构成，细胞密集区和细胞稀疏区交替排列，其间过渡突然。细胞密集区间质胶原纤维丰富，稀疏区间质黏液样变，可见较多鸡爪样或树枝状管腔闭合的毛细血管。肿瘤细胞形态温和，胞核无明显异型，核分裂象罕见。免疫组化：肿瘤细胞MUCA特异性（＋）。发生于上颌翼腭窝病例术后3年局部复发，发生于腹膜后和乳腺的病例分别随访5年和2年均无瘤生存。结论LGFMS因细胞形态温和及组织形态多样性极易误诊为其他具有黏液样间质的梭形细胞肿瘤。该瘤发病部位广泛，最常见于年轻人；其生物学行为呈惰性经过，治疗上对肿瘤作局部切除已足够。Objective To investigate the clinicopathological features of low-grade fibromyxoid sarcoma （LGFMS） and discuss its differential diagnosis. Methods Three cases of LGFMS occurring in rare locations were studied with respect to clinical presentation, radiological characteristics, histological features, immun@enotype and prognosis. Results The locations of 3 cases of LGFMS occurred in the right maxilla, breast and retroperitoneum, respectively. Grossly, the tumors were typically well-circumscribed with a fibrous capsule. The cut surface was grayish to white collagenous areas admixed with myxoid areas. Microscopically, LGFMS was composed of bland spindle cells arransed in a whorled pattern, and showed alternating myxoid zones and fibrous stroma zones with prominent arcade curvilinear capillaries. The transition between fibrous and myxoid zones was characteristically abrupt. Cytological atypia, mitotic figures and tumor necrosis were absent in all 3 cases. Follow-up information was available in 3 cases with a duration ranging from 2 to5 years. One case experienced local recurrence 3 years postoperatively, the other 2 cases were alive with no evidence of disease. Conclusions LGFMS is easy to be misdiagnosed as spindle cell tumors with myxoid stroma owing to the deceptively bland histological feature and diverse histological appearance. The tumor can occur in various locations, and affects preferentially the young or middle-aged adults. LGFMS is considered to undergo a protracted clinical course with frequent local recurrence and rare metastasis, and so local tumor marginal excision is suggested for those without metastasis.

关 键 词：低度恶性纤维黏液样肉瘤 诊断 鉴别诊断 

分 类 号：R738.6[医药卫生—肿瘤]