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肝脏血管内皮瘤(病例报告)(作者译)

[Hemangio-endothelioma of the liver (case report) (author's transl)].

作者信息

Zürcher B, Caflisch U, Hofer B, Schärli A, Laissue J

出版信息

Z Kinderchir. 1982 Jan;35(1):26-31. doi: 10.1055/s-2008-1059895.

Abstract

The infantile hemangio-endothelioma (IHE) of the liver, a rare neoplasm, may lead the clinician and the surgeon to suspect the presence of a malignant tumor, whereas histologic examination often reveals a benign condition; our case report illustrates this discrepancy in a 3 1/2-month-old girl suffering from multiple nodules in the liver. Conservative steroid treatment resulted in a marked reduction in tumor size; the girl is in good health more than two years after diagnosis. More than 80% of hepatic IHE are diagnosed during the first six months of life. Hepatomegaly, congestive heart failure and hemangiomas of the skin combine to the classic symptomatic triad. Since the mortality is high in untreated cases, early diagnosis is of importance. IHE may regress spontaneously. Therefore, conservative therapy, such as steroid medication and treatment of the secondary, tumor-induced complications appear indicated. Alternatively, embolization and/or resection of the tumor may be considered. Signs of cardiac or hepatic insufficiency at diagnosis and a low degree of histologic differentiation are of unfavorable prognostic significance.

摘要

肝脏婴儿型血管内皮瘤(IHE)是一种罕见的肿瘤,可能会让临床医生和外科医生怀疑存在恶性肿瘤,而组织学检查往往显示为良性病变;我们的病例报告展示了一名3个半月大、肝脏有多个结节的女孩身上的这种差异。保守的类固醇治疗使肿瘤大小显著缩小;该女孩在诊断后两年多健康状况良好。超过80%的肝脏IHE在出生后的前六个月被诊断出来。肝肿大、充血性心力衰竭和皮肤血管瘤构成了典型的症状三联征。由于未经治疗的病例死亡率很高,早期诊断很重要。IHE可能会自发消退。因此,似乎需要采取保守治疗,如类固醇药物治疗以及对肿瘤引发的继发性并发症的治疗。或者,也可以考虑对肿瘤进行栓塞和/或切除。诊断时出现心脏或肝脏功能不全的迹象以及组织学分化程度低具有不良的预后意义。

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