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罕见的浸润性原发性肝血管肉瘤:一例报告并文献复习

Rare infiltrative primary hepatic angiosarcoma: A case report and review of literature.

作者信息

Lin Xiao-Jing, Luo Hong-Chang

机构信息

Department of Medical Ultrasound, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology, Wuhan 430030, Hubei Province, China.

出版信息

World J Gastrointest Oncol. 2024 Jul 15;16(7):3341-3349. doi: 10.4251/wjgo.v16.i7.3341.

Abstract

BACKGROUND

The most primary sites of angiosarcoma are the skin, breast gland, and soft tissues. Primary hepatic angiosarcoma (PHA) is a rare malignant tumor of mesothelial tissue originating from the liver. PHA often presents with multiple intrahepatic foci or metastasis at the time of presentation due to its nonspecific clinical presentation and highly aggressive nature. There are no established or effective treatment guidelines for PHA, so early detection and early treatment are of great value for patient survival. Unfortunately, there is a paucity of literature on the imaging features of PHA, making the diagnosis and treatment of this disease a considerable challenge.

CASE SUMMARY

In this case report, we present a 59-year-old man who initially presented with abdominal pain and radiating pain in the right shoulder. Magnetic resonance imaging and positron emission tomography-computed tomography revealed multiple intrahepatic nodules that needed to be differentiated from tumors of vascular epithelial origin and tumors with progressive enhancement features, and signs of tumor metastasis were assessed. The patient was then subjected to contrast-enhanced ultrasonography (CEUS) to further clarify the extent of tumor infiltration and the state of microcirculatory perfusion. The manifestations observed on CEUS were similar to the classical characteristic presentation of hepatocellular carcinoma, called "quick wash-in and quick wash-out". In addition, CEUS showed that the lesion exhibited gradual infiltration and growth along the liver pedicle structures with no invading blood vessels. Finally, based on pathological and immunohistochemical tests and the above imaging manifestations, it was confirmed that the patient had infiltrating PHA, which is a rare pathological type of PHA. The patient underwent transcatheter arterial chemoembolization and chemotherapy. Four months after the onset of symptoms, the follow-up radiological examination revealed poor treatment efficacy and rapid deterioration.

CONCLUSION

This case report complements the imaging modalities of a rare infiltrative PHA, in which CEUS and quantitative analysis are found to offer substantial advantages in characterizing the microcirculatory perfusion of the lesion, providing clinicians with diagnostic information at the earliest opportunity to make a diagnosis and develop a treatment strategy to prolong the patient survival.

摘要

背景

血管肉瘤最主要的发病部位是皮肤、乳腺和软组织。原发性肝血管肉瘤(PHA)是一种起源于肝脏的间皮组织的罕见恶性肿瘤。由于其临床表现不具特异性且侵袭性强,PHA在发病时常常表现为肝内多发病灶或已有转移。目前尚无针对PHA的确立的或有效的治疗指南,因此早期发现和早期治疗对患者生存具有重要价值。不幸的是,关于PHA影像学特征的文献匮乏,这使得该疾病的诊断和治疗面临相当大的挑战。

病例摘要

在本病例报告中,我们介绍了一名59岁男性,其最初表现为腹痛及右肩部放射痛。磁共振成像和正电子发射断层扫描-计算机断层扫描显示肝内有多个结节,需要与血管上皮来源的肿瘤以及具有渐进性强化特征的肿瘤相鉴别,并评估肿瘤转移迹象。随后对该患者进行了超声造影(CEUS),以进一步明确肿瘤浸润范围和微循环灌注状态。CEUS观察到的表现类似于肝细胞癌的经典特征性表现,即“快进快出”。此外,CEUS显示病灶沿肝蒂结构呈渐进性浸润生长,无血管侵犯。最后,基于病理和免疫组化检查以及上述影像学表现,确诊该患者患有浸润性PHA,这是一种罕见的PHA病理类型。该患者接受了经动脉化疗栓塞和化疗。症状出现四个月后的随访影像学检查显示治疗效果不佳且病情迅速恶化。

结论

本病例报告补充了罕见的浸润性PHA的影像学检查方法,其中发现CEUS和定量分析在表征病灶微循环灌注方面具有显著优势,能为临床医生尽早提供诊断信息以做出诊断并制定治疗策略,从而延长患者生存期。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6689/11271798/bc5529e33414/WJGO-16-3341-g001.jpg

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