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翻译 | 上皮样炎性肌纤维母细胞肉瘤细胞病理学特征以及组织病理学、免疫组织化学和分子细胞遗传学分析

2016-04-08  黄7510

译者:魏建国【水若寒】浙江省绍兴市人民医院病理科

癌症—细胞病理学杂志

上皮样炎性肌纤维母细胞肉瘤(E-IMS)是最近确定的一种罕见炎性肌纤维母细胞瘤变型。E-IMS具有特征性的一组临床、病理以及分子学特征,包括几乎全部位于腹腔内、男性多发、侵袭性临床过程、上皮样肿瘤细胞为主、以及大多数病例具有Ran结合蛋白2(RANBP2)-间变性淋巴瘤酶(ALK)融合。


据作者所知,E-IMS细胞学特征目前尚未研究描述。收集具有相关细胞学的E-IMS病例。确定了5位E-IMS患者的6例细胞学样本(包括1例细针抽吸样本、2例印片样本及3例渗出液样本)。


 E-IMS细胞形态为较大的单一上皮样细胞疏松聚集或单个排列,伴有混合性黏液样间质及富于中性粒细胞的炎性背景。肿瘤细胞具有一个大的、圆形、偏位细胞核,染色质呈空泡状,核仁明显;细胞质量中等、淡染。细针抽吸样本中,小的薄壁分枝状血管穿过肿瘤聚集区是其一个显著特征。


免疫组织化学中,所有5例肿瘤ALK均呈阳性,其中3例为核膜染色型,而另外2例为细胞质染色型。通过分子遗传学研究证实,5例肿瘤均发生了ALK基因重排。 E-IMS细胞学特征概括体现了其组织学特征。E-IMS需纳入任何一种腹腔内、大的上皮样细胞肿瘤的鉴别诊断。确定ALK基因的重排是可取的,因为患者可以受益于靶向治疗。

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.

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