Selby D M, Stocker J T, Waclawiw M A, Hitchcock C L, Ishak K G
Office of the Deputy Director, Armed Forces Institute of Pathology, Washington, D.C. 20306.
Hepatology. 1994 Jul;20(1 Pt 1):39-45. doi: 10.1016/0270-9139(94)90132-5.
Clinical and morphological findings in 91 patients with infantile hemangioendothelioma of the liver are reported. The study comprised 56 girls and 35 boys ranging in age from premature infant to 3 yr; one outlier patient was 18 yr old. Most patients with infantile hemangioendothelioma (87%) were first seen before the age of 6 mo. Congestive heart failure was evident in 15%. Skin hemangiomas were noted in 11%. Anemia, hyperbilirubinemia and increased AST level were present. Solitary lesions were more common than multiple ones (3:2). Immunohistochemical staining of tumor cells for factor VIII was positive in 20 of 21 cases tested; testing for blood group antigen was positive in 8 of 28 cases. Cytokeratin staining verified the presence of bile ducts, some of which appeared to be the result of transformation of injured liver. No pericytes were identified on electron microscopy. The 6-mo survival rate, based on 71 cases, was 70%. Average time of follow-up for the survivors was 7.7 yr. All deaths occurred during the initial presentation/hospitalization of infants, with the exception of two patients who died 3 mo and 7 mo after diagnosis. More recent analytic methods, including immunohistochemical stains and flow cytometric studies, do not contribute to the practical assessment of this tumor. Covariates with significant value in predicting death 6 mo after diagnosis included presence of congestive heart failure, jaundice, multiple tumor nodules and absence of cavernous differentiation.
报告了91例婴儿肝血管内皮瘤患者的临床和形态学表现。该研究包括56名女孩和35名男孩，年龄从早产儿到3岁不等；1例异常患者为18岁。大多数婴儿肝血管内皮瘤患者（87%）在6个月龄前首次就诊。15%的患者出现充血性心力衰竭。11%的患者有皮肤血管瘤。存在贫血、高胆红素血症和AST水平升高。孤立性病变比多发性病变更常见（3:2）。在21例检测病例中，20例肿瘤细胞因子VIII免疫组化染色呈阳性；28例中的8例血型抗原检测呈阳性。细胞角蛋白染色证实存在胆管，其中一些似乎是受损肝脏转化的结果。电子显微镜检查未发现周细胞。基于71例病例，6个月生存率为70%。幸存者的平均随访时间为7.7年。除2例患者在诊断后3个月和7个月死亡外，所有死亡均发生在婴儿初次就诊/住院期间。包括免疫组化染色和流式细胞术研究在内的最新分析方法对该肿瘤的实际评估没有帮助。诊断后6个月预测死亡的有显著意义的协变量包括充血性心力衰竭、黄疸、多个肿瘤结节以及无海绵状分化。
