翻譯 | 上皮樣炎性肌纖維母細胞肉瘤細胞病理學特征以及組織病理學、免疫組織化學和分子細胞遺傳學分析

癌癥—細胞病理學雜志

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

據作者所知，E-IMS細胞學特征目前尚未研究描述。收集具有相關細胞學的E-IMS病例。確定了5位E-IMS患者的6例細胞學樣本（包括1例細針抽吸樣本、2例印片樣本及3例滲出液樣本）。

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

免疫組織化學中，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例為細胞質染色型。通過分子遺傳學研究證實，5例腫瘤均發生了ALK基因重排。 E-IMS細胞學特征概括體現了其組織學特征。E-IMS需納入任何一種腹腔內、大的上皮樣細胞腫瘤的鑒別診斷。確定ALK基因的重排是可取的，因為患者可以受益于靶向治療。

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.