上皮样黏液纤维肉瘤十例临床病理分析
Epithelioid myxofibrosarcoma:a clinicopathologic analysis of 10 cases
目的:探讨上皮样黏液纤维肉瘤( EMFS)的临床病理学特点、诊断、鉴别诊断及生物学行为。方法收集2008年4月至2014年9月来自复旦大学附属肿瘤医院及苏北人民医院的10例EMFS的临床病理资料,行光镜观察、免疫学标记,并复习相关文献。结果男性5例,女性5例,患者均为中老年,发病年龄53~74岁,平均和中位年龄分别为63.6和62.5岁。肿瘤发生于四肢6例(上肢、下肢各3例),躯干3例,乙状结肠系膜1例。肿瘤直径4.2~7.0 cm,平均5.3 cm。临床上多以无痛性肿块就诊,病程持续时间1~24个月(平均8个月)。10例均行手术切除,其中4例术后予以放疗和/或化疗,1例予以介入治疗,5例未行辅助治疗。镜下观察:8例为高级别,2例为中级别。所有病例的原发性肿瘤均呈多结节状生长,主要由细胞稀疏的黏液样区和细胞丰富的致密区组成。黏液样区域内,瘤细胞呈单个散在分布或小簇状排列,常可见细长的弧形或曲线形血管和假脂母细胞;致密区内,瘤细胞多呈片状分布。肿瘤主要由梭形细胞和数量不等的上皮样细胞组成,后者核呈空泡状,可见明显的核仁,胞质中等量或丰富,嗜酸性,部分区域可见瘤巨细胞和多核性瘤细胞,呈多形性未分化肉瘤形态,特别是复发性肿瘤内。上皮样区域所占比例30%~90%,通常和经典黏液纤维肉瘤成分相混杂。免疫组织化学标记:经检测的6例均弥漫性表达波形蛋白,灶性表达平滑肌肌动蛋白和上皮细胞膜抗原各1例。 Ki-67阳性指数30%~80%,平均58%。10例均获得随访资料,随访时间2~74个月,平均23个月。4例带瘤生存,6例无瘤生存。5例局部复发,2例转移,平均及中位复发/转移时间分别为9个月和7个月。结论黏液纤维肉瘤中出现上皮样区域提示肿瘤具有更高的侵袭性,预后不佳。 EMFS需注意与其他具有上皮样形态的黏液样肉瘤相鉴别。
更多Objective To investigate clinicopathologic features , pathologic diagnosis , differential diagnosis and biological behavior of epitheioid myxofibrosarcoma ( EMFS ).Methods The clinical and pathological data of 10 cases were collected , and microscopic examination and immunostains were performed along with a review of the literatures.Results There were 5 males and 5 females with age ranging from 53 to 74 years, and the mean and median age was 63.6 and 62.5 years, respectively.Six cases developed in the extremities, including upper limbs (n=3) and lower limbs (n=3).Three developed in the trunk and 1 case in the mesentery of sigmoid colon.Tumor size ranged from 4.2 to 7.0 cm ( mean, 5.3 cm).Most patients presented with painless masses with duration of 1 to 24 months ( mean, 8 months).All 10 patients were treated by surgery , with adjunctive chemotherapy and/or radiotherapy in 4 patients and interventional therapy in 1 patient.Histologically , 8 cases were high grade and 2 were intermediate grade.Like the conventional myxofibrosarcomas , all primary tumors presented a multinodular growth pattern consisting of hypocellular myxoid and hypercellular areas.Prominent curvilinear vessels and pseudolipoblasts were observed in the hypocellular myxoid areas.Besides the spindled neoplastic cells , all tumors were characterized by a variable proportion of epithelioid cells with vesicular nuclei , prominent nucleoli and moderate to abundant eosinophilic cytoplasm.They were arranged singly or in small clusters in the myxoid areas, and in compact sheets in the solid areas.The epithelioid component comprised 30% to 90% of the tumors.In addition , areas with resemblance to undifferentiated pleomorphic sarcoma were also noted , especially in the recurrent tumors.Immunohistochemically , tumor cells showed diffuse staining of vimentin in 6 tested cases with focal expression of smooth muscle actin and epithelial membrane antigen in 1 case each.Ki-67 index ranged from 30% to 80% ( mean, 58%).Follow-up data ( range, 2 to 74 months;mean, 23 months) were available in 10 cases:4 patients were alive with unresectable or recurrent disease and 6 patients were alive with no evidence of disease.Five patients experienced local recurrence and 2 cases developed metastasis.The median interval to recurrence/metastasis was 7 months (mean, 9 months). Conclusions The presence of epithelioid cells in a myxofibrosarcomatous background portends an aggressive clinical behavior.EMFS should be differentiated from other myxoid sarcomas with epithelioid morphology .
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