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成人肝脏巨大间叶性错构瘤

Giant mesenchymal hamartoma of the liver in an adult.

作者信息

Mori Ryutaro, Morioka Daisuke, Morioka Kaori, Ueda Michio, Sugita Mitsutaka, Takeda Kazuhisa, Matsuo Ken-ichi, Tanaka Kuniya, Endo Itaru, Sekido Hitoshi, Togo Shinji, Shimada Hiroshi

机构信息

Department of Gastroenterological Surgery, Yokohama City University Graduate School of Medicine, 3-9 Fukuura, Yokohama, 236-0004, Japan.

出版信息

J Hepatobiliary Pancreat Surg. 2008;15(6):667-9. doi: 10.1007/s00534-007-1286-6. Epub 2008 Nov 7.

Abstract

We report a case of hepatic mesenchymal hamartoma in an adult; this condition is extremely rare, with only 15 cases having been reported in the English-language literature worldwide. The patient was a 36-year-old woman who was seen at her local hospital for upper abdominal distension. A giant multilocular cystic tumor, which had almost entirely replaced the normal parenchyma of the right lobe of the liver, was diagnosed. She was referred to our hospital, where, with a diagnosis of biliary cystadenoma, the tumor was successfully removed by right hemihepatectomy. After an uneventful postoperative course, the patient was discharged from our hospital. On histological examination, the tumor consisted of numerous cystic lesions without epithelial lining cells; hepatocytes, bile duct, and vascular components, without either lobular structure or atypia, were observed in the pseudocyst wall, leading to a diagnosis of hepatic mesenchymal hamartoma. There have been a few previously reported cases of multifocal hepatic mesenchymal hamartoma reappearing in the remaining liver after hepatectomy, although these cases are considered to be extremely rare. Therefore, periodic follow-up will be necessary for the patient.

摘要

我们报告一例成人肝间叶性错构瘤;这种情况极为罕见,全球英文文献中仅报道过15例。患者为一名36岁女性,因上腹部胀满在当地医院就诊。诊断为一个巨大的多房性囊性肿瘤,几乎完全取代了肝右叶的正常实质。她被转诊至我院,在我院诊断为胆管囊腺瘤后,通过右半肝切除术成功切除肿瘤。术后过程顺利,患者从我院出院。组织学检查显示,肿瘤由众多无上皮衬里细胞的囊性病变组成;在假囊肿壁中观察到肝细胞、胆管和血管成分,无小叶结构或异型性,从而诊断为肝间叶性错构瘤。虽然之前有少数多灶性肝间叶性错构瘤在肝切除术后于剩余肝脏中复发的病例报道,但这些病例被认为极为罕见。因此,该患者需要定期随访。

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