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心脏血管肉瘤的多发性肝转移

Multiple hepatic metastases of cardiac angiosarcoma.

作者信息

Cunha-Silva Marlone, Mazo Daniel Ferraz de Campos, Gomes Igor Logetto Caetité, Imbrizi Marcello Rabello, Sevá-Pereira Tiago, Vaz Nayana Fonseca, Lourenço Mariana Sandoval, Eloy da Costa Larissa Bastos, Escanhoela Cecilia Amelia Fazzio, Almeida Jazon Romilson de Souza

机构信息

University of Campinas (Unicamp), School of Medical Sciences, Department of Gastroenterology (Gastrocentro). Campinas, SP, Brazil.

University of Campinas (Unicamp), School of Medical Sciences, Department of Internal Medicine. Campinas, SP, Brazil.

出版信息

Autops Case Rep. 2018 Oct 9;8(4):e2018048. doi: 10.4322/acr.2018.048. eCollection 2018 Oct-Dec.

Abstract

The differential diagnosis of hepatic focal lesions is challenging because the etiology can be inflammatory, infectious, and even neoplastic. A rare cause of metastatic liver nodules is cardiac angiosarcoma. We report a case of this tumor, which was diagnosed only after autopsy. A 26-year-old Caucasian man was admitted for progressive dyspnea and cough over the past 3 weeks. Physical examination showed only hypophonetic heart sounds. Laboratory analysis demonstrated anemia and elevated inflammatory markers, despite normal biochemical parameters and liver function. Transthoracic echocardiography revealed massive pericardial effusion. Abdomen computed tomography (CT) showed multiple hepatic nodules, the largest of which measured 3 cm, but the percutaneous biopsy revealed only lobular necrosis and perisinusoidal fibrosis without granulomas or neoplastic cells. During hospitalization, the patient had fever and night sweats with weight loss, and empiric treatment for extrapulmonary tuberculosis associated with corticosteroids was initiated. The outpatient follow-up revealed complete improvement of the pericardial effusion, but maintenance of the liver lesions. After 2 months of hospital discharge, the patient was readmitted with hemorrhagic shock due to bleeding liver lesions, which were evidenced by CT. Embolization of the right hepatic artery was performed, but the patient soon died. The autopsy revealed a primary cardiac angiosarcoma with multiple hepatic metastases, rupture of the Glisson's capsule and laceration of the liver. The case shows how important and difficult the diagnosis of focal liver lesions is, since it may result in an unexpected fatal outcome.

摘要

肝脏局灶性病变的鉴别诊断具有挑战性,因为其病因可能是炎症性、感染性甚至是肿瘤性的。转移性肝结节的一种罕见病因是心脏血管肉瘤。我们报告一例这种肿瘤,该病例仅在尸检后才得以诊断。一名26岁的白种男性因过去3周内进行性呼吸困难和咳嗽入院。体格检查仅发现心音减弱。实验室分析显示贫血和炎症标志物升高,尽管生化参数和肝功能正常。经胸超声心动图显示大量心包积液。腹部计算机断层扫描(CT)显示多个肝结节,最大的结节直径为3厘米,但经皮活检仅显示小叶坏死和窦周纤维化,无肉芽肿或肿瘤细胞。住院期间,患者出现发热、盗汗和体重减轻,遂开始对与皮质类固醇相关的肺外结核进行经验性治疗。门诊随访显示心包积液完全改善,但肝脏病变持续存在。出院2个月后,患者因肝脏病变出血导致失血性休克再次入院,CT证实了这一点。对右肝动脉进行了栓塞,但患者很快死亡。尸检显示原发性心脏血管肉瘤伴多发肝转移、肝门管区包膜破裂和肝脏撕裂伤。该病例表明肝脏局灶性病变的诊断是多么重要和困难,因为它可能导致意想不到的致命后果。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/32e8/6360823/d5cb55a6b911/autopsy-08-04e2018048-g01.jpg

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