上皮样血管肉瘤的临床病理分析

目的 分析上皮样血管肉瘤的临床病理特点及需进行鉴别诊断的肿瘤。方法 利用HE染色,免疫组织化学抗生物素蛋白-生物素复合物法（ABC法）观察15例上皮样血管肉瘤,电镜观察其中12例,结果 15例中12例为男性,3例为女性,发生部位;腹膜后3例,四肢软组织3例,肌骨4例,甲状腺2例,眶上缘1例,胸壁软组织1例,颈部软组织1例。临床症状包括局部肿物及其引起的疼痛,消瘦,乏力。影像学检查显示肿瘤多为实性或囊实性,直径可达3-20cm。镜下观察瘤组织或弥漫成片或聚集成巢,伴有出血和多灶坏死,瘤组织内常见大小不等的不规则腔隙,衬附异型瘤细胞,单个瘤细胞或多个瘤细胞形成空腔包绕红细胞。电镜可观察到胞质内空泡,W-P小体,中间丝及细胞基底膜的存在,瘤细胞可表达CD34,CD31,Ⅷ因子相关抗原,细胞角蛋白,上皮膜抗原,血管周细胞表达平滑肌肌动蛋白,临床主要采用手术切除的治疗方法,偶尔辅以放疗。随访/2例存活,术后存活期分别为19个月,7年;12例已死亡,术后存活期最短为2周,最长38个月,平均19个月;1例失访。8例患者发生肺,骨,淋巴结和腹腔转移。结论 上皮样血管肉瘤是高度恶性肉瘤,免疫组织化学检测和电镜观察有助于确定肿瘤的内皮分化,上皮样血管肉瘤需与转移癌,上皮样血管内皮细胞瘤,恶性黑色素瘤,上皮样肉瘤等鉴别。

A clinicopathological study of fifteen epithelioid angiosarcoma

Objective To study the clinicopathological features of epithelioid angiosarcoma (EA) and to propose its differential diagnosis. Methods 15 EAs were observed by means of HE staining, immunohistochemical staining (ABC method) and in part by electron microscopy. Results Twelve cases occurred in men and three in women. Clinical symptoms included a focal mass with pain, weight loss and weakness. The tumors were located in the retroperitoneum (n=3), extremities (n=3), femur (n=4), thyroid (n=2), supraobit (n=1), soft tissue of thorax (n=1), soft tissue of neck (n=1). Radiographic evaluation demonstrated solid to cystic neoplasms ranging from 3 to 20 cm in diameter. Histologically, the epithelioid cells were arranged diffusely or in nests with hemorrhage and focal necrosis. Atypical tumor cells lined the irregular spaces and single or small nests of cells grouped around a lumen containing red blood cells. FVIII, CD34, CD31,CK and EMA were positive in tumor cells and SMA was positive in pericytes. Ultrastructurally, intracytoplasmic vacuoles, Weibel-Palades, intermediate filaments and basal lamina could be seen. Surgical resection was the treatment of choice, occasionally with adjuvant chemotherapy. Follow-up. Two patients are presently alive 19 months and 7 years following diagnosis. Twelve died from 2 weeks to 38 months with a median interval of 19 months after lumpectomy. One patient was lost to follow up. There were 8 cases which had metastases to lungs, bones, lymph nodes and abdominal cavity. Conclusions EA is a high-grade sarcoma. Endothelial derivation can be confirmed by immunohistochemical staining and ultrastractural findings. The differential diagnosis of EA includes metastatic carcinoma, epithelioid hemangioendothelioma, melanoma, epithelioid sarcoma etc.