癌癥—細(xì)胞病理學(xué)雜志

上皮樣炎性肌纖維母細(xì)胞肉瘤（E-IMS）是最近確定的一種罕見炎性肌纖維母細(xì)胞瘤變型,。E-IMS具有特征性的一組臨床,、病理以及分子學(xué)特征，包括幾乎全部位于腹腔內(nèi),、男性多發(fā),、侵襲性臨床過程、上皮樣腫瘤細(xì)胞為主,、以及大多數(shù)病例具有Ran結(jié)合蛋白2（RANBP2）-間變性淋巴瘤酶（ALK）融合,。

據(jù)作者所知，E-IMS細(xì)胞學(xué)特征目前尚未研究描述,。收集具有相關(guān)細(xì)胞學(xué)的E-IMS病例,。確定了5位E-IMS患者的6例細(xì)胞學(xué)樣本（包括1例細(xì)針抽吸樣本、2例印片樣本及3例滲出液樣本）,。

 E-IMS細(xì)胞形態(tài)為較大的單一上皮樣細(xì)胞疏松聚集或單個(gè)排列,，伴有混合性黏液樣間質(zhì)及富于中性粒細(xì)胞的炎性背景。腫瘤細(xì)胞具有一個(gè)大的,、圓形,、偏位細(xì)胞核，染色質(zhì)呈空泡狀，核仁明顯,；細(xì)胞質(zhì)量中等,、淡染,。細(xì)針抽吸樣本中,，小的薄壁分枝狀血管穿過腫瘤聚集區(qū)是其一個(gè)顯著特征。

免疫組織化學(xué)中,，所有5例腫瘤ALK均呈陽性,，其中3例為核膜染色型，而另外2例為細(xì)胞質(zhì)染色型,。通過分子遺傳學(xué)研究證實(shí),，5例腫瘤均發(fā)生了ALK基因重排。 E-IMS細(xì)胞學(xué)特征概括體現(xiàn)了其組織學(xué)特征,。E-IMS需納入任何一種腹腔內(nèi),、大的上皮樣細(xì)胞腫瘤的鑒別診斷。確定ALK基因的重排是可取的,，因?yàn)榛颊呖梢允芤嬗诎邢蛑委煛?/p>

Cytopathologic features of epithelioid inflammatory myofibroblastic sarcoma with correlation of histopathology, immunohistochemistry, and molecular cytogenetic analysis.

Lee JC,Wu JM,Liau JY,Huang HY,Lo CY,Jan IS,Hornick JL,Qian X

Cancer Cytopathology,； August 2015 Volume 123, Issue 8:495-504 

Epithelioid inflammatory myofibroblastic sarcoma (E-IMS) is a recently established rare variant of inflammatory myofibroblastic tumor. It is characterized by a distinctive constellation of clinical, pathological, and molecular features, including a nearly exclusive intraabdominal location, strong male predilection, aggressive clinical course, predominance of epithelioid tumor cells, and Ran-binding protein 2 (RANBP2)-anaplastic lymphoma kinase (ALK) fusion in the majority of cases. To the authors' knowledge, the cytologic features of E-IMS have not been described to date.

Cases of E-IMS that had corresponding cytology were searched. Six cytology samples (1 fine-needle aspiration sample, 2 imprint samples, and 3 effusion fluids) containing tumor cells were identified in 5 patients with E-IMS.

The cytomorphology included large monotonous epithelioid cells arranged in loose aggregates or singly, with admixed myxoid stroma, and an inflammatory background rich in neutrophils. The tumor cells had a large, round, eccentric nucleus with vesicular chromatin, prominent nucleoli, and moderate amounts of pale cytoplasm. Delicate thin-walled branching vessels traversing tumor aggregates was a prominent feature in a fine-needle aspiration sample. Immunohistochemically, ALK was positive in all 5 tumors, with a nuclear membranous staining pattern noted in 3 cases and a cytoplasmic pattern observed in the other 2 cases. ALK rearrangement was confirmed in all 5 tumors by molecular genetic studies.

The cytologic features of E-IMS recapitulate its histologic characteristics. E-IMS merits inclusion in the differential diagnosis of any intraabdominal, large epithelioid cell neoplasm. Confirmation of ALK rearrangement is advisable because patients may benefit from targeted therapies. Cancer (Cancer Cytopathol) 2015;123:495-504. ? 2015 American Cancer Society.