PDF(Pubmed)
Abstract：
The combination of placental mesenchymal dysplasia and hepatic mesenchymal hamartomas is an extremely rare finding. We present the case of a three-month-old female born at 35 weeks gestation with a history of placental mesenchymal dysplasia who presented with non-bilious, non-bloody emesis, and episodes of respiratory distress due to multiple enlarging abdominal cystic lesions. The patient\'s presentation was unique due to both liver and adrenal solid and cystic lesions. After extensive imaging and multiple biopsies, expert interpretation of biopsy tissue revealed hepatic mesenchymal hamartoma within the liver and the adrenal gland. To our knowledge, this is one of the few documented cases of unresectable hepatic mesenchymal hamartomas with adrenal involvement successfully undergoing a whole liver transplant.
摘要：
胎盘间充质发育不良和肝间充质错构瘤的组合是极其罕见的发现。我们介绍了一个3个月大的女性，在妊娠35周时出生，有胎盘间充质发育不良病史，表现为非胆汁性，非血腥呕吐,和由于腹部多次扩大的囊性病变引起的呼吸窘迫发作。由于肝脏和肾上腺实性和囊性病变，患者的表现是独特的。经过广泛的成像和多次活检，活检组织的专家解释显示肝脏和肾上腺内的肝间质错构瘤。据我们所知,这是少数有记载的不可切除的肝间质错构瘤伴肾上腺受累的病例之一，成功进行了全肝移植。
