Widening Spectrum of Liver Angiosarcoma in Children
Widening Spectrum of Liver Angiosarcoma in Children
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DOI：
10.1097/mpg.0b013e318230146c
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发表时间：
2011-12-01
影响因子：
2.9
通讯作者：
Bernard, Olivier
中科院分区：
文献类型：
--
作者：
Ackermann, Oanez;Fabre, Monique;Bernard, Olivier
目的：肝血管瘤是一种血管肿瘤，发生于生命最初几个月，具有初始并发症的风险，但组织学上被认为是良性的，并随着时间的推移而退化。组织学研究表明，亚型2型血管内皮瘤与血管肉瘤相似，可能有严重的长期预后。我们报告了5例患有2型肝血管内皮瘤的女孩。方法和结果：3名儿童最初表现为典型的婴儿多结节血管瘤，包括心肺并发症和肿瘤消退，年龄为11/2至2 /2岁。3例患者均在21岁半至3岁时肿瘤复发，并在21岁半至5岁时死亡。肿瘤组织学表现为2型血管内皮瘤。另外两名儿童分别在2岁和3岁时出现肝脏肿瘤。在1中，单个肿瘤的初始活检显示为良性1型血管内皮瘤，但手术切除后剩余肝脏复发，肺转移，死亡。整个肿瘤组织学表现为1型和2型病变。另一名患儿肿瘤活检显示2型病变。她接受了肝移植，3年后没有肿瘤复发。结论：婴儿肝多结节性血管瘤的晚期复发需要仔细的随访。婴儿期后发生的血管性肝肿瘤有可能是恶性的。剩余肝脏复发的高风险表明，如果没有发现转移，在这两种情况下，肝移植比手术切除肿瘤更可取。
Objectives: Liver hemangiomas are vascular tumors, which occur in the first months of life and carry risks of initial complications, but are considered to be benign histologically and to regress with time. Histologic studies suggest that a subtype, type 2 hemangioendothelioma, is akin to angiosarcoma and may have a severe long-term prognosis. We report 5 girls with type 2 hemangioendothelioma of the liver.Methods and Results: Three children initially presented with classical infantile multinodular hemangioma, including cardiac and pulmonary complications and regression of tumors at age 11/2 to 21/2 years. All 3 experienced tumor relapse at ages 21/2 to 3, leading to death at ages 21/2 to 5. Tumor histology showed type 2 hemangioendothelioma. The other 2 children presented with liver tumors at ages 2 and 3 years. In 1, initial biopsy of a single tumor showed benign type 1 hemangioendothelioma, but surgical resection was followed by relapse in the remaining liver, lung metastases, and death. Whole tumor histology showed both type 1 and 2 lesions. In the other child, tumor biopsy showed type 2 lesions. She underwent liver transplantation and is alive without tumor recurrence 3 years later.Conclusions: Careful follow-up is necessary to detect late recurrence in infants with multinodular liver hemangiomas. Vascular liver tumors occurring after infancy are likely to be malignant. The high risk of relapse in the remaining liver suggests that if no metastases are detected, liver transplantation is preferable to surgical tumor resection in both situations.