Srouji M N, Chatten J, Schulman W M, Ziegler M M, Koop C E
Cancer. 1978 Nov;42(5):2483-9. doi: 10.1002/1097-0142(197811)42:5<2483::aid-cncr2820420552>3.0.co;2-0.
Mesenchymal hamartoma of the liver is a rare benign tumor of childhood which has been confused with various other benign liver lesions, particularly vascular hamartomas which are mesodermal but not mesenchymal. The mass, frequently cystic, is characterized by an admixture of epithelial structures in a loose connective tissue stroma with fluid accumulation suggestive of lymphangiomatous channels. Four patients are presented and reviewed with 25 previously reported cases. The patients, usually asymptomatic, present during the first two years of life with progressive abdominal distention, which may be rapid because of increasing fluid content in the connective tissue stroma and cysts. Exploration and biopsy may be necessary before definitive excision. The present cases include a newborn, the youngest known, and a case in which radiation resulted in hyalinization of the mesenchyme, decrease in fluid content, and easlier resection. Respiratory distress and signs of vena caval obstruction due to intra-abdominal pressure were noted. Prognosis after extirpation is very good.
肝间叶性错构瘤是一种儿童期罕见的良性肿瘤，它曾被与多种其他良性肝脏病变相混淆，尤其是血管错构瘤，后者是中胚层来源但并非间叶组织。该肿块通常为囊性，其特征是在疏松结缔组织基质中有上皮结构混合存在，伴有提示淋巴管瘤样通道的液体蓄积。本文报告了4例患者，并对之前报道的25例病例进行了回顾。这些患者通常无症状，在出生后的头两年出现进行性腹胀，由于结缔组织基质和囊肿内液体增多，腹胀可能进展迅速。在进行确定性切除之前，可能需要进行探查和活检。目前的病例包括1例已知最年幼的新生儿，以及1例经放射治疗后间叶组织发生透明变性、液体含量减少且更易于切除的病例。还注意到有因腹内压力导致的呼吸窘迫和腔静脉阻塞的体征。切除术后预后非常好。
