Mentzel T, Beham A, Calonje E, Katenkamp D, Fletcher C D
Department of Pathology, University of Jena, Germany.
Am J Surg Pathol. 1997 Apr;21(4):363-74. doi: 10.1097/00000478-199704000-00001.
Epithelioid hemangioendothelioma of soft tissues (EHE) represents a distinct entity with an unpredictable clinical course. We analyzed the clinicopathologic and immunohistochemical features in a series of 30 patients. Patient age range was 16-74 years (median 50); 18 of 30 patients were female. Eight tumors arose in the lower and two in the upper extremities, seven on the trunk, five each in the head/ neck and anogenital regions, two in the mediastinum, and one in the abdomen. Seventeen neoplasms were located in deep soft tissues, nine were subcutaneous or perifascial, and four were dermal; size ranged from 0.4 to 10 cm; in 11 cases the tumor was > 5 cm. Tumors with an infiltrative growth pattern were more common than entirely circumscribed lesions. The tumors were composed histologically of short strands, cords, or small clusters of epithelioid, round, to slightly spindled endothelial cells that formed at least focally, intracellular lumina and were set in a frequently myxohyaline stroma. Thirteen of 30 lesions showed angiocentric growth, which was occlusive in many cases. Immunohistochemically, all cases tested were positive for at least one endothelial marker (CD31, CD34, factor VIII, Ulex europaeus), six of 23 (26%) were positive for cytokeratin, and five of 11 (45%) were positive for alpha-smooth muscle actin. Median follow-up of 36 months (range 2-96) in 24 cases showed local recurrence in three cases and systemic metastases in five cases (21%); four patients (17%) died of tumor. Although more aggressive histologic features (striking nuclear atypia in eight cases, numerous spindled cells in 10, more than two mitoses per 10 high-power fields in nine, and small, more solid angiosarcomalike foci in four cases) tended to be related to poor clinical outcome, there was no clear correlation. Two metastasizing cases showed no histologically atypical features whatever. We suggest that EHE of soft tissue is better regarded as a fully malignant, rather than borderline, vascular neoplasm, albeit the prognosis is better than in conventional angiosarcoma.
软组织上皮样血管内皮瘤（EHE）是一种具有不可预测临床病程的独特实体。我们分析了30例患者的临床病理和免疫组化特征。患者年龄范围为16 - 74岁（中位年龄50岁）；30例患者中有18例为女性。8例肿瘤发生于下肢，2例在上肢，7例在躯干，头颈部和生殖区域各5例，2例在纵隔，1例在腹部。17例肿瘤位于深部软组织，9例为皮下或筋膜周围，4例为真皮内；大小范围为0.4至10 cm；11例肿瘤直径> 5 cm。具有浸润性生长模式的肿瘤比完全包膜完整的病变更常见。肿瘤组织学上由短束状、条索状或小簇状上皮样、圆形至轻度梭形内皮细胞组成，这些细胞至少局灶性地形成细胞内管腔，并位于经常为黏液样透明的基质中。30例病变中有13例显示血管中心性生长，在许多情况下为闭塞性。免疫组化方面，所有检测病例至少对一种内皮标志物（CD31、CD34、因子VIII、荆豆凝集素）呈阳性，23例中有6例（26%）细胞角蛋白呈阳性，11例中有5例（45%）α-平滑肌肌动蛋白呈阳性。24例患者的中位随访时间为36个月（范围2 - 96个月），3例出现局部复发，5例发生全身转移（21%）；4例患者（17%）死于肿瘤。尽管更具侵袭性的组织学特征（8例有明显核异型性，10例有大量梭形细胞，9例每10个高倍视野有超过2个有丝分裂象，4例有小的、更实性的血管肉瘤样病灶）往往与不良临床结局相关，但并无明确相关性。2例发生转移的病例无论在组织学上均无非典型特征。我们建议，软组织EHE更好地被视为一种完全恶性的血管肿瘤，而非交界性肿瘤，尽管其预后优于传统血管肉瘤。
