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以腹水为表现的肝血管肉瘤:一种罕见但致命疾病的病例报告

Hepatic Angiosarcoma Presenting as Ascites: Case Report of a Rare yet Lethal Disease.

作者信息

Garg Archit, Bassi Mehak, Pitchumoni Capecomorin, Broder Arkady

机构信息

Internal Medicine, Saint Peter's University Hospital, New Brunswick, NJ, USA.

Gastroenterology, Saint Peter's University Hospital, New Brunswick, NJ, USA.

出版信息

Case Rep Gastroenterol. 2025 Jul 2;19(1):488-495. doi: 10.1159/000546375. eCollection 2025 Jan-Dec.

Abstract

INTRODUCTION

Angiosarcomas, constituting less than 1% of all sarcomas, are rare soft tissue tumors originating from the endothelial cells. Hepatic angiosarcoma (HAS) is a rare and aggressive primary hepatic malignancy accounting for only 0.5%-2% of all liver tumors. The patients often endorse nonspecific symptoms like vague abdominal pain, nausea, vomiting, and jaundice making the diagnosis challenging. Most patients succumb to death within 6 months of diagnosis due to liver failure or hemorrhage from spontaneous rupture of HAS. Therapeutic guidelines remain undefined, and management often involves a multidisciplinary approach. Surgical resection is the only potentially curative option, which has been shown to be most beneficial when HAS is limited to one lobe. Hepatic artery embolization is used in the case of rupture of HAS. Chemotherapy can be used for palliative care in cases of advanced tumors. We present a fatal case of metastatic HAS to underscore diagnostic pitfalls and therapeutic challenges.

CASE DESCRIPTION

A 56-year-old male presented with 2 months of abdominal pain, distension, fatigue, and weight loss. Imaging revealed multifocal hypodense liver and splenic lesions. Laboratory findings included severe anemia (Hb 6.1 g/dL), thrombocytopenia (63 × 10/mm), and elevated liver enzymes. Ascitic fluid analysis demonstrated exudative, bloody ascites (SAAG <1.1) without malignant cytology. Liver biopsy confirmed HAS, showing atypical spindle cells infiltrating vascular channels, positive for CD34 and factor VIII. Despite transfusions, paracentesis, and palliative care, the patient developed disseminated intravascular coagulation and died 2 weeks post-diagnosis.

CONCLUSION

HAS is a rapidly fatal malignancy often diagnosed at advanced stages due to nonspecific symptoms and lack of definitive risk factors in most cases. Multidisciplinary collaboration is essential for symptom management, though treatment options remain limited, and prognosis is poor. Therefore, it becomes imperative for clinicians to keep in mind the common presentation of a rare but lethal disease.

摘要

引言

血管肉瘤占所有肉瘤的比例不到1%,是起源于内皮细胞的罕见软组织肿瘤。肝血管肉瘤(HAS)是一种罕见且侵袭性强的原发性肝脏恶性肿瘤,仅占所有肝脏肿瘤的0.5%-2%。患者常出现如腹部隐痛、恶心、呕吐和黄疸等非特异性症状,这使得诊断具有挑战性。大多数患者在诊断后6个月内死于肝衰竭或HAS自发性破裂出血。治疗指南尚不明确,治疗通常采用多学科方法。手术切除是唯一可能治愈的选择,当HAS局限于一个肝叶时,手术切除已被证明是最有益的。对于HAS破裂的情况,可采用肝动脉栓塞治疗。对于晚期肿瘤,化疗可用于姑息治疗。我们报告一例转移性HAS的致命病例,以强调诊断陷阱和治疗挑战。

病例描述

一名56岁男性出现腹痛、腹胀、乏力和体重减轻2个月。影像学检查显示肝脏和脾脏多发低密度病变。实验室检查结果包括严重贫血(血红蛋白6.1 g/dL)、血小板减少(63×10/mm)和肝酶升高。腹水分析显示为渗出性血性腹水(血清腹水白蛋白梯度<1.1),未发现恶性细胞。肝脏活检确诊为HAS,显示非典型梭形细胞浸润血管腔,CD34和因子VIII呈阳性。尽管进行了输血、腹腔穿刺和姑息治疗,患者仍发生了弥散性血管内凝血,并在诊断后2周死亡。

结论

HAS是一种迅速致命的恶性肿瘤,由于大多数情况下症状不特异且缺乏明确的危险因素,往往在晚期才被诊断出来。多学科协作对于症状管理至关重要,尽管治疗选择仍然有限,且预后较差。因此,临床医生必须牢记这种罕见但致命疾病的常见表现。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c2af/12215201/a124c396b58e/crg-2025-0019-0001-546375_F01.jpg

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