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巨大间质性肝错构瘤累及肾上腺并引发呼吸衰竭:三个月大婴儿的黏液瘤之谜

Giant Mesenchymal Hepatic Hamartomas With Adrenal Involvement Precipitating Respiratory Failure: A Myxomatous Mystery in a Three-Month-Old.

作者信息

Yasback Ali, Ulhaque Abid, Chandra Tushar

机构信息

College of Allopathic Medicine, Nova Southeastern University Dr. Kiran C. Patel College of Allopathic Medicine, Fort Lauderdale, USA.

Radiology, HCA West Florida, Trinity Medical Center, Trinity, USA.

出版信息

Cureus. 2023 Apr 12;15(4):e37476. doi: 10.7759/cureus.37476. eCollection 2023 Apr.

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

胎盘间质性发育异常与肝脏间质性错构瘤并存是一种极其罕见的情况。我们报告一例35周妊娠出生的3个月大女性病例,该患儿有胎盘间质性发育异常病史,因多个腹部囊性病变增大出现非胆汁性、非血性呕吐及呼吸窘迫发作。该患者的表现较为独特,因为肝脏和肾上腺均存在实性和囊性病变。经过广泛的影像学检查和多次活检,活检组织的专家解读显示肝脏和肾上腺内均有肝脏间质性错构瘤。据我们所知,这是少数有记录的不可切除的肝脏间质性错构瘤累及肾上腺并成功接受全肝移植的病例之一。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/48d7/10176756/03f2fb954da8/cureus-0015-00000037476-i01.jpg

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