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

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.

Keywords: Primary hepatic angiosarcoma, Dynamic contrast-enhanced ultrasonography, Quantitative analysis, Multimodal imaging, Case report

Core Tip: The proportion of the patients with primary hepatic angiosarcoma (PHA) is low, and infiltrating PHA is extremely rarer. The kind of the patients with infiltrating PHA are highly prone to misdiagnosis and missed diagnosis, which leads to the poor prognosis. The contrast-enhanced ultrasonography (CEUS) pattern of the patient in this case report is different from that of previously reported PHA patients. We analyzed the lesions by dynamic CEUS (DCE-US), and their manifestation mimicked the imaging pattern of hepatocellular carcinoma known as "quick wash-in and quick wash-out". DCE-US may be helpful for the diagnosis of infiltrating PHA.