Hepatic Hemangioendothelioma in Children: Analysis of Thirteen Cases

兒童期之肝臟血管內皮瘤：十三病例分析

陳建彰(Chien-Chang Chen) ； 江文山(Man-Shan Kong) ； 楊兆平(Chao-Ping Yang) ； 洪悠紀(Iou-Jih Hung)

《Acta Paediatrica Taiwanica》 44卷1期 (2003/02) Pp. 8-13

https://doi.org/10.7097/APT.200302.0008

摘要

肝臟血管內皮瘤是好發於在嬰兒及兒期之腫瘤。從1986至2001年，共有13位罹患肝臟血管內皮瘤的兒童(9例男性，4例女性)在本院診斷治療。其中1位的年齡均小於六個月，而僅有一位在診斷時已達兩歲。在這13位病童中，10位病童有臨床症狀，而大部分需要治療的原因乃由於血小板過低、貧血、心衰竭、呼吸窘迫、或是黃疸。有一位病童接受部份肝臟切除手術，另外一位則接受肝動脉栓塞治療。計有7例病童接受類固醇藥物治療，3例病童給予干擾素治療，有一位病童接受化學治療。在這13位病童中，有4位病童僅給予保守療法。本欠的統計，有4例病童不幸死亡，死因是由於敗血症，腫瘤破裂併發出血休克，或是肝臟衰竭。適當的治療，長期的追踪對患有肝臟血管內皮瘤的兒童來說是很重要的。

關鍵字

肝臟血管內皮瘤 ； 電腦斷層掃描 ； 核磁共振檢查 ； 類固醇藥物 ； 干擾素

並列摘要

Hepatic hemangioendothelioma (HE) is a tumor that presents in infancy and toddler. It manifests hepatomegaly, abdominal mass, jaundice, abdominal distention, or high output cardiac failure. We reviewed patients with HE in our hospital in the past 15 years (from July 1986 to June 2001). The diagnosis was made by the histology specimen or various imaging studies. There were thirteen patients (9 males, 4 females) enrolled in our study. Their ages ranged from neonate to 2 years old. The common clinical manifestations included abdominal distention (53%) congestive heartfailure (38.5%), abdominal mass (30.8%), jaundice (30.8%), and skin hemangioma (23.1%). Nine patients had serum alanine aminotransferase examination and were abnormal in 2. Anemia was noted in 7 of 13 (53.8%) patients, thrombocytopenia and hyperconsumptive coagulopathy were found in 4 and 5 patients, respectively. Serum alpha-fetoprotein was elevated in 4 of 7patients. Abdominal ultrasonography (n=13) showed heterogeneous and hypoechoic lesions in the liver. Computed tomography (n=11) revealed central hypointensity with peripheral enhancement after contrast of the liver masses. Magnetic resonance imaging studies of the hepatic masses (n=3) showed decreased signal intensity on Ti images and high signal intensity on T2. Most patients were treated with steroid. Other management included interferon, chemotherapy, embolization and or surgery. Four patients were managed conservatively. Among the other nine patients, four patients died of sepsis, hepatic failure, disseminated intravascular coagulopathy or tumor rupture with hemorrhagic shock. HE appears to be a histologically benign tumor but may have a poor outcome because of complications. For its management, steroid is a first-line medication. Other methods of treatment were interferon, hepatic artery embolization, chemotherapy and surgery. Long term follow up is needed for the evaluation of treatment response.

並列關鍵字

hemangioendothelioma ； computed tomography ； magnetic resonance imaging ； steroids ； interferon