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肝脏间叶性错构瘤的长期随访——单中心研究。

Long-term follow up of mesenchymal hamartoma of liver--single center study.

机构信息

Department of Pediatric Surgery, Institute of Medical Sciences, Banaras Hindu University, Varanasi-221 005, UP, India.

出版信息

Saudi J Gastroenterol. 2011 Jan-Feb;17(1):20-2. doi: 10.4103/1319-3767.74449.

Abstract

BACKGROUND/AIM: Mesenchymal hamartoma of liver (MHL) is a rare liver tumor of childhood. About 200 cases have been reported till now. Most of the work on MHL is limited to case reports and there are not many long term follow-up studies. We present our 20 years of experience with this uncommon entity. This study aims to highlight clinical features, diagnosis and treatment of MHL.

MATERIALS AND METHODS

All patients with a diagnosis of MHL in last 20 years were included in this retrospective study. The patients were evaluated clinically, radiologically and pathologically.

RESULTS

The total number of patients with a diagnosis of MHL was nine. Mean age of the patients was 19.89 ± 2.75 months. Right lobe was involved in eight patients. The prominent clinical features were distension of abdomen and anorexia. Surgical options used were hepatic lobectomy, wedge resection and enucleation. Histopathology of the specimens showed cysts of variable size with normal hepatocytes, bile ducts and connective tissue stroma. Overall mortality was one (11.11%).

CONCLUSION

MHL is a benign tumor that can present with various clinical features. It should be differentiated carefully from other liver masses especially malignant ones. The diagnosis can be made with the help of radiology and histopathology. Adequate resection is curative in most of the cases and long-term follow up is satisfactory.

摘要

背景/目的:肝间叶性错构瘤(MHL)是一种罕见的儿童肝脏肿瘤。到目前为止,大约已经报告了 200 例病例。大多数关于 MHL 的研究都仅限于病例报告,并且没有很多长期随访研究。我们介绍了我们在这种罕见实体上的 20 年经验。本研究旨在强调 MHL 的临床特征、诊断和治疗。

材料和方法

在过去的 20 年中,所有诊断为 MHL 的患者均被纳入本回顾性研究。患者接受了临床、放射学和病理学评估。

结果

诊断为 MHL 的患者总数为 9 例。患者的平均年龄为 19.89 ± 2.75 个月。8 例患者的右叶受累。突出的临床特征是腹部膨隆和食欲不振。使用的手术方案是肝叶切除术、楔形切除术和肝切除术。标本的组织病理学显示大小不一的囊肿,其中包含正常肝细胞、胆管和结缔组织基质。总死亡率为 1 例(11.11%)。

结论

MHL 是一种良性肿瘤,可表现出各种临床特征。它应与其他肝脏肿块(特别是恶性肿瘤)仔细区分。借助影像学和组织病理学可以做出诊断。大多数情况下,充分切除是可治愈的,并且长期随访结果令人满意。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6928/3099075/21a5d54ac66b/SJG-17-20-g001.jpg

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