Murray J D, Ricketts R R
Egleston Children's Hospital at Emory University, Atlanta, Georgia, USA.
Am Surg. 1998 Nov;64(11):1097-103.
Mesenchymal hamartomas of the liver are the second most common benign liver tumor of childhood. The experience with this tumor at Egleston's Children Hospital at Emory University from 1989 to 1994 is reviewed. Eight patients presented with abdominal distention or an upper abdominal mass. Six patients presented at a mean age of 8 months, and two patients presented at 17 and 23 years of age, respectively. Four patients displayed normal alpha-fetoprotein levels, whereas one patient had an elevated level. Liver function studies were normal in all patients. Abdominal ultrasonography and CT scans revealed a cystic, septated mass within the liver or on a pedicle in all patients. Five patients had simple excision of the tumor, and two had major hepatic resections. The cysts were multiloculated and lined with cuboidal bile duct epithelium surrounded by stroma containing proliferating bile ducts, blood vessels, and compressed liver tissue with no calcifications. In one patient, some pathologists favored the diagnosis of malignant myxoid fibrous histiocytoma because of similar-appearing stroma. Follow-up (mean, 35 months) revealed one symptomatic recurrence after initial resection was incomplete. There were no other recurrences and no malignant transformations. A septated, noncalcified, cystic hepatic mass in an infant with normal liver function studies and characteristic ultrasound or CT is likely a benign mesenchymal hamartoma that can be cured by total local excision.
肝间叶性错构瘤是儿童期第二常见的肝脏良性肿瘤。本文回顾了1989年至1994年埃默里大学埃格尔斯顿儿童医院对该肿瘤的诊治经验。8例患者表现为腹胀或上腹部肿块。6例患者就诊时的平均年龄为8个月，另外2例患者分别为17岁和23岁。4例患者甲胎蛋白水平正常，1例患者甲胎蛋白水平升高。所有患者的肝功能检查均正常。腹部超声和CT扫描显示，所有患者肝脏内或蒂部有一个囊性、有分隔的肿块。5例患者行肿瘤单纯切除术，2例行肝大部切除术。囊肿为多房性，内衬立方上皮胆管，周围间质内有增生的胆管、血管和受压的肝组织，无钙化。1例患者，部分病理学家因间质表现相似而倾向于诊断为恶性黏液样纤维组织细胞瘤。随访（平均35个月）发现，1例患者在初次切除不彻底后出现症状性复发。无其他复发及恶变情况。肝功能检查正常、超声或CT表现典型的婴儿，肝脏内有分隔、无钙化的囊性肿块，很可能是良性间叶性错构瘤，可通过局部完整切除治愈。
