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免疫功能正常患者的原发性肝脏平滑肌瘤。

Primary leiomyoma of the liver in an immunocompetent patient.

作者信息

Coletta Diego, Parrino Chiara, Nicosia Simone, Manzi Emy, Pattaro Giada, Oddi Andrea, D'Annibale Marco, Marino Mirella, Grazi Gian Luca

机构信息

Hepatopancreatobiliary Surgery, IRCCS - Regina Elena National Cancer Institute, Rome, Italy.

Emergency Department - Emergency and Trauma Surgery Unit, Umberto I University Hospital, Sapienza University of Rome, Rome, Italy.

出版信息

Intractable Rare Dis Res. 2020 Nov;9(4):251-255. doi: 10.5582/irdr.2020.03075.

Abstract

Primary leiomyoma of the liver (PLL) is a rare benign tumor occurring in immunosuppressed people. From 1926 less than fifty cases are reported in the scientific literature and about half are in immunocompetent patients. Etiology of this kind of lesion is not yet well known. We report a case of primary hepatic leiomyoma in a 60-year-old immunocompetent woman. The patient presented with lipothymia with unexpected vomiting. She underwent an ultrasound (US), and a computed tomography (CT) scan that revealed the presence of a single, solid lesion about 9 cm located between the S5 and S8 segment of the liver. It showed a well-defined, heterogeneous hypodensity with internal and peripheral enhancement and various central hypoattenuating areas and no wash-out in the portal and the late phases. Because of her symptoms and the risk of malignancy, the patient underwent a surgical liver resection. Histological diagnosis was primary leiomyoma of the liver. The patient had an uneventful recovery and was discharged after 7 days. At 30 months follow-up there were no symptoms and no evidence of disease. Leiomyoma of the liver is a rare benign neoplasm of which clinical symptoms are nonspecific and the exact radiological diagnosis still remains a challenge for radiologists. Etiology is still unclear and usually PLL represents an incidental diagnosis. Surgery plays a primary role not only in the treatment algorithm, but also in the diagnostic workout.

摘要

肝脏原发性平滑肌瘤(PLL)是一种发生于免疫抑制人群的罕见良性肿瘤。自1926年以来,科学文献报道的病例不足50例,其中约一半为免疫功能正常的患者。这类病变的病因尚不清楚。我们报告一例60岁免疫功能正常女性的原发性肝脏平滑肌瘤病例。患者表现为晕厥伴意外呕吐。她接受了超声(US)和计算机断层扫描(CT)检查,结果显示肝脏S5和S8段之间存在一个约9厘米的单个实性病变。它表现为边界清晰、密度不均匀的低密度影,内部和周边有强化,中央有多个低密度区,门静脉期和延迟期无廓清。由于患者的症状及恶性肿瘤风险,她接受了肝脏手术切除。组织学诊断为肝脏原发性平滑肌瘤。患者恢复顺利,7天后出院。随访30个月时,无症状且无疾病迹象。肝脏平滑肌瘤是一种罕见的良性肿瘤,其临床症状不具特异性,准确的影像学诊断对放射科医生来说仍是一项挑战。病因仍不清楚,PLL通常为偶然诊断。手术不仅在治疗方案中起主要作用,在诊断过程中也至关重要。

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