Unique advantages of contrast-enhanced ultrasound in identifying extremely rare hepatic angiosarcoma with sinusoidal growth patterns

Introduction

Primary hepatic angiosarcoma (PHA) is an insidious disease with an extremely poor prognosis. It is difficult to capture PHA on conventional abdominal ultrasound. Moreover, multimodal imaging often lacks specific and consistent features (1). Contrast-enhanced ultrasound (CEUS) has the advantage of providing some additional information for differential diagnosis in clinical practice.

Case presentation

A 71-year-old male patient with a previous history of chronic hepatitis B with hepatic cirrhosis was admitted to our hospital with nonspecific symptoms, including dizziness and fatigue. Laboratory examination revealed normal alpha-fetoprotein levels, slightly increased liver enzymes and thrombosis-related indicators. A computed tomography (CT) scan revealed a slightly low-density nodule adjacent to the capsule of segment 8. On contrast-enhanced CT, the lesion measured 36 mm × 35 mm, which was larger than that observed on the non-contrast scan. Large blood vessels could be seen in the early stage of enhancement, and the delayed phase was consistent with the adjacent liver parenchyma enhancement. (Figure 1A). Magnetic resonance imaging (MRI) showed that in the S8 segment of the liver, there was a round nodule with a diameter of 11 mm, with long T1 and slightly longer T2 signal intensity. Diffusion was slightly restricted on diffusion-weighted imaging (DWI), the enhancement was not uniform, and the enhancement range was enlarged on contrast-enhanced scan. The nature of the lesion remained to be determined, and it is necessary to distinguish between inflammatory and neoplastic lesions (Figure 1B,1C). It showed heterogeneous enhancement in the arterial phase and hypointensity compared to normal liver parenchyma in the hepatobiliary phase, which made it difficult to distinguish between infectious and malignant lesions. However, no obvious liver space-occupying lesion was shown on conventional abdominal ultrasound. In the early arterial phase, a lesion about 20 mm × 11 mm in size was revealed in CEUS, showing slight hyperenhancement in the right anterior lobe of liver. It immediately became iso-enhancing with an unclear boundary. In late portal and delayed phases, slight hypoenhancement was observed, with the lesion size increasing to 31 mm × 27 mm until partial washout of the contrast agent at 1 minute 48 seconds (Figure 1D,1E). Based on CEUS Liver Imaging Reporting & Data System (LI-RADS) 2017, it was appropriately classified as LI-RADS category M (LR-M). Liver histopathology confirmed the presence of PHA with sinusoidal growth pattern (Figure 1F) (2). The patient underwent partial liver tissue resection and recovered well.

Figure 1 Multimodal imaging and pathological findings. Contrast-enhanced CT displayed a lesion measuring approximately 36 mm × 35 mm in size (A). The red arrow indicated slightly low-density nodules at the periphery of segment 8 in the CT scan. Contrast-enhanced MRI showed a circular nodule with long T1 and slightly longer T2 (B,C). The white arrow showed a round shape with a diameter of 11 mm in MRI. CEUS view showed the lesion measuring approximately 20 mm × 11 mm with an unclear boundary in arterial phase (D) and progressive hypoenhancement in the late portal and delayed phase (E). The white arrows are the morphological features of the nodules under CEUS. Liver histopathology stained with HE confirmed the presence of PHA with sinusoidal growth pattern (×4) (F). CEUS, contrast-enhanced ultrasound; CT, computed tomography; HE, hematoxylin and eosin; MRI, magnetic resonance imaging; PHA, primary hepatic angiosarcoma.

PHA progresses rapidly, with most cases being discovered at an advanced stage. Its median survival is <6 months without treatment (3), and most patients, even after surgery, die within one year of diagnosis (4). The multimodal imaging features of CT and MRI were nonspecific, which is a dilemma in PHA diagnosis. The diagnostic hurdle is radiological. PHA can present in various forms on cross-sectional imaging: as a single large mass, multiple nodules, or a diffusely infiltrative lesion. In previous studies, PHA has lacked consistent imaging characteristics on CEUS, probably presenting as earlier or hyperenhancement compared to the liver parenchyma during the early stage of arterial enhancement and rapid regression and hypoenhancement changes in both portal venous phase and delayed phase (5). However, this pattern is not universal. Some lesions exhibit peripheral nodular enhancement with progressive centripetal fill-in, similar to that of a hemangioma, whereas others show central enhancement, creating a diagnostic conundrum. Given the limitations of imaging, histopathological confirmation is essential. However, this introduces a second major dilemma: the significant risk of life-threatening hemorrhage. PHA is a fragile, blood-filled tumor, and percutaneous biopsy carries a documented risk of catastrophic bleeding. This often leads to clinical hesitation, particularly if a benign vascular lesion is still a possibility. Hence, in our case, CEUS provided further insights into the imaging features of PHA. Moreover, it has advantages in achieving better recognition of liver lesions that have proven difficult to visualize with conventional ultrasound. Most importantly, it serves as a valuable tool for guiding intraoperative needle localization to demonstrate its advantage in the perioperative period.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was provided by the patient for the publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Acknowledgments

None.

Footnote

Funding: This study was supported by the Hubei Provincial Natural Science Foundation of China (grant Nos. 2025AFD870 and 2022CFB212, to F.X.) and the National Nature Science Foundation of China (grant No. 82302227 to T.L. and No. 82171961 to J.W.).

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1484/coif). T.L. reports funding from the National Nature Science Foundation of China (grant No. 82302227). J.W. reports funding from the National Nature Science Foundation of China (grant No. 82171961). F.X. reports funding from the Hubei Provincial Natural Science Foundation of China (grant Nos. 2025AFD870 and 2022CFB212). The other authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Declaration of Helsinki and its subsequent amendments. Written informed consent was obtained from the patient for the publication of this article and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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