腹腔上皮样炎性肌纤维母细胞肉瘤临床病理观察  

Retroperitoneal epithelioid inflammatory myofibroblastic sarcoma:a clinicopathological analysis

作　　者：吴惠[1] 孟宇宏[1] 宁浩勇[1] 路平[1] 洪柳[1] 康筱玲[1] 段敏刚[1] 冉志远[1] 

机构地区：[1]海军总医院病理科,北京100048

出　　处：《诊断病理学杂志》2014年第11期680-684,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的 探讨上皮样炎性肌纤维母细胞肉瘤（EIMT）的临床病理特点、诊断与鉴别诊断要点.方法 总结1例腹膜后EIMT的临床、病理形态及免疫表型特点,并复习相关文献.结果 患者女性,47岁.左上腹疼痛半年,疼痛加重伴腹胀1个月.CT示左上腹腔占位.术中见肿瘤位于腹膜后降结肠脾曲肠系膜内,与脾、胰尾、膈肌关系密切.大体见肿瘤呈结节状,位于肠系膜内,侵及肠壁并与周围脂肪、脾、胰及横纹肌组织粘连.镜下见肿瘤细胞排列成片状或结节状,间质明显黏液样变性及中性粒细胞、淋巴细胞、浆细胞和嗜酸性粒细胞浸润.瘤细胞圆形、多角或梭形,胞质丰富,呈上皮样,染色质空泡状,核仁大而明显,核分裂象易见.免疫组化示肿瘤细胞vimentin、desmin、ALK和AAT弥漫强（＋）,部分瘤细胞EMA、CD30、WT-1、D2-40、CD99和p53（＋）,Ki-67阳性指数10％ - 20％.荧光原位杂交示有ALK基因重排.患者术后2个月肿瘤局部复发,出现肝、肺多发转移,于术后4个月死亡.结论 上皮样炎性肌纤维母细胞肉瘤是炎性肌纤维母细胞瘤罕见的恶性上皮样亚型;诊断依据部位、典型的形态学和免疫组化特点,特别是核膜和核周ALK（＋）.治疗以手术切除为首选,术后放、化疗和靶向治疗为辅助治疗.此类肿瘤恶性度高,预后差.Objective To study the clinicopathological characteristics,diagnosis,differential diagnosis,treatment and prognosis of epithelioid inflammatory myofibroblastic sarcoma （EMIS）.Methods The clinical data,pathology and immunohistochemical,FISH features of one case of retroperitoneal EMIS were presented and the relevant literature was reviewed.Results A 47-year-old female presented as left upper abdominal pain for 6 months and abdominal distention for 1 month.CT examination showed a solid mass in the left upper intra-abdomen.Grossly,the tumor was found in the mesenterium of colon with the size of 7.5 × 6.5 × 3.5 cm,and was solid and gray-yellowish on cut surface.Focal myxomatous appearance was observed.Microscopically,stromal myxoid change together with prominant infiltrating lymphocytes,neutrophils and eosinophils were found in the tumor,and the tumor cells were round,polygonal or spindle in shape with vesicular nuclei,large prominant nucleoli and high mitotic rate.Immunohistochemically,the tumor cells were strongly positive for vimentin,desmin,ALK （nuclear membrane staining pattern） and AAT,focally positive for CD99,CD30,WT-1 and D2-40; Ki-67 index was about 20％.FISH analysis showed rearrangement of ALK gene.Conclusions EIMS is a rare and aggressive variant of an inflammatory myofibrobrastic tumor.The diagnosis is based on its location,typical morphology and the immunohistochemical features,especially nuclear membranous immunostaining of ALK.The tumor shows higher aggressive behaviors and a poor prognosis.

关 键 词：上皮样炎性肌纤维母细胞肉瘤 腹腔 ALK 

分 类 号：R730.262[医药卫生—肿瘤]