Chen Yunxin, Shen Danhua, Sun Kunkun, Bao Dongmei, Song Qiujing, Wang Gongwei, Chen Dingbao, Yan Taiqiang, Guo Wei
Department of Pathology, Peking University People’s Hospital, No. 11 Xizhimen South Street, Xicheng District, Beijing, 100044, China.
Tumori. 2011 Sep-Oct;97(5):585-9. doi: 10.1177/030089161109700508.
Epithelioid angiosarcoma is a rare histopathologic variant of angiosarcoma characterized by an epithelioid morphology. This subset can histologically mimic non-vascular neoplasms and impose serious challenges in reaching a correct diagnosis, especially in the context of limited tissue sampling (e.g., needle core biopsy). To improve recognition of epithelioid angiosarcoma - and the spectrum of morphologic diversity associated with this rare variant - and to avoid a misdiagnosis, we describe the clinical, histopathologic, and immunohistochemical findings of cases of epithelioid angiosarcoma diagnosed at our institution.
Seven cases of epithelioid angiosarcoma with appropriate pathologic material were identified from our archives. Immunohistochemistry was used to detect the expression of CD31, CD34, Factor VIII, cytokeratin, epithelial membrane antigen, vimentin, HMB45, CD1a, CD68, lysozyme, CD45, desmin, and smooth muscle actin in all cases. Follow-up information was obtained by reviewing medical records or by direct communication with family members.
The lesions involved the bone (n = 4) and soft tissues (n = 3). Microscopically, all tumors had a predominantly diffuse growth pattern, with a focal nested architecture in 6 cases, which closely mimicked metastatic carcinoma. The initial biopsy was performed in 2 of 6 patients and revealed the presence of a malignant neoplasm suggestive of metastatic carcinoma. Immunohistochemically, the epithelioid endothelial cells usually showed strong reactivity for CD31 (7/7), variable or focal positive staining for CD34 (5/7), Factor VIII (4/7), cytokeratin (6/7), epithelial membrane antigen (2/7), vimentin (7/7), and CD68 (3/7). In contrast, they were negative for CD1a, HMB45, lysozyme, CD45, desmin, and smooth muscle actin. Three patients died of disease within one year of the diagnosis, 2 patients developed local recurrence or metastases, and another 2 were disease-free at this writing.
With any unusual epithelioid neoplasm displaying some or all of the morphologic features described above, epithelioid angiosarcoma should be included in the differential diagnosis. In such an instance, endothelial markers should be incorporated in the immunohistochemical analysis to avoid misdiagnosis, particularly with limited sampling.
上皮样血管肉瘤是血管肉瘤一种罕见的组织病理学变异类型，其特征为上皮样形态。这一亚型在组织学上可模仿非血管性肿瘤，在做出正确诊断时面临严峻挑战，尤其是在组织取样有限的情况下（如针芯活检）。为提高对上皮样血管肉瘤及其相关罕见变异型形态学多样性谱的认识，并避免误诊，我们描述了在本机构诊断的上皮样血管肉瘤病例的临床、组织病理学和免疫组化结果。
从我们的档案中识别出7例有合适病理材料的上皮样血管肉瘤病例。所有病例均采用免疫组化检测CD31、CD34、凝血因子VIII、细胞角蛋白、上皮膜抗原、波形蛋白、HMB45、CD1a、CD68、溶菌酶、CD45、结蛋白和平滑肌肌动蛋白的表达。通过查阅病历或直接与家庭成员沟通获取随访信息。
病变累及骨骼（4例）和软组织（3例）。显微镜下，所有肿瘤主要呈弥漫性生长模式，6例有局灶性巢状结构，酷似转移性癌。6例患者中有2例进行了初次活检，结果显示存在提示转移性癌的恶性肿瘤。免疫组化方面，上皮样内皮细胞通常对CD31呈强反应性（7/7），对CD34（5/7）、凝血因子VIII（4/7）、细胞角蛋白（6/7）、上皮膜抗原（2/7）、波形蛋白（7/7）和CD68（3/7）呈可变或局灶性阳性染色。相比之下，它们对CD1a、HMB45、溶菌酶、CD45、结蛋白和平滑肌肌动蛋白呈阴性。3例患者在诊断后1年内死于疾病，2例患者出现局部复发或转移，另有2例在撰写本文时无疾病进展。
对于任何表现出上述部分或全部形态学特征的不寻常上皮样肿瘤，上皮样血管肉瘤应列入鉴别诊断。在这种情况下，应将内皮标记物纳入免疫组化分析以避免误诊，尤其是在取样有限时。
