CT findings of primary hepatic angiosarcoma complicated with hepatic sinusoidal obstruction syndrome: a case description

Introduction

The 68-year-old female patient reported a six-month history of anorexia, and a two-week history of abdominal pain, abdominal distension, and edema of the lower limbs. Her laboratory test results were as follows: alpha-fetoprotein, 4.63 ng/mL; carcinoembryonic antigen, 1.05 ng/mL, carbohydrate tumor CA199, 1.77 U/mL; and ferritin, 454.2 1 ng/mL. Tests for hepatitis B surface antigen, hepatitis B surface antibody, hepatitis B E antigen, hepatitis B core antibody were all negative.

Multiple hypoechoic nodules were detected in the hilum of the liver, one of which was approximately 3.4 cm × 1.9 cm in size. The hilum of the lymph node shows a depressed appearance. Color doppler flow imaging showed no significant blood flow signal in the hilum. Abdominal computed tomography (CT) showed that the liver was enlarged and irregular in shape, with uneven edges and uneven density. Contrast-enhanced scans showed obviously uneven enhancement. The hepatic segment of the inferior vena cava and hepatic vein appeared slender, with partial visualization unclear; the portal cavity space and retroperitoneal lymph nodes were increased in number and enlarged. Abdominal and pelvic effusion was present, accompanied by abdominal mesangial swelling and turbidity. The scans showed a widespread and heterogeneous increase in bone density (Figure 1A-1F).

Figure 1 Abdomen CT findings. Non-contrast CT scan (A), arterial phase of enhanced CT scan (B), portal phase (C), and equilibrium phase (D), and enlarged lymph nodes in the portal area (as indicated by the white arrow) (E), as well as a coronal bone reconstruction window showing an extensive and inhomogeneous increase in all bones (as indicated by the white arrow) (F). CT, computed tomography.

Positron emission tomography (PET)/CT showed that the liver was enlarged with diffuse nodules, and the metabolism of fluorodeoxyglucose (FDG) was active. The hepatogastric ligament, hepatoduodenal ligament, and right septal angle lymph nodes were enlarged, and FDG metabolism was active. Multiple splenic nodules showed active FDG metabolism. Whole-body bone diffuse mixed bone destruction (mainly osteogenesis) was observed, and FDG metabolism was active but slightly uneven. Multiple vitreous nodules were observed in both lungs, and FDG metabolism was normal. A diagnosis of malignant neoplastic lesions was considered (Figure 2).

Figure 2 PET-CT with 18F-FDG whole-body scanning revealed diffusely distributed nodules in the liver, with active FDG metabolism (as indicated by the white arrow). The display zoom magnification is uniform at 0.38× for all subfigures within the panel. FDG, fluorodeoxyglucose; PET-CT, positron emission tomography-computed tomography.

CT-guided liver biopsy of a small tissue sample revealed a spindle cell lesion with hepatocyte edema in the residual liver tissue. The immunohistochemistry results were as follows: Vim (+), Desmin (–), CK (spindle lesion –), CD34 (focal +), S-100 (–), CD45 (lymphocyte +), CD56 (–), FLI-1 (+), ERG (+), CD31 (+), and Ki-67 (+, index about 20%) (Figure 3). A diagnosis of primary hepatic angiosarcoma (PHA) with multiple lymph node metastases in the abdominal cavity, multiple bone metastases in the spleen, lungs and whole body, and hepatic sinusoidal obstruction syndrome (HSOS) was confirmed. The patient died after 2 months.

Figure 3 Pathology results. (A) Hematoxylin and eosin staining results (×100); (B-E) Immunohistochemical staining results (CD31+, CD43+, EGR+, and FLI+).

All the procedures in this study were performed 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’s family for the publication of this article and the accompanying images. A copy of the written consent form is available for review by the editorial office of this journal.

Discussion

PHA is a very rare malignant mesenchymal tumor, originating from vascular endothelial cells. PHA accounts for 2% of all primary liver malignant tumors (1). PHA is more likely to occur in individuals aged 50–70 years, and is associated with high malignancy and poor prognosis (2). It is highly invasive and prone to extrahepatic metastasis. The common sites of metastasis include the lung, spleen, and bone, and bone metastases mainly present as osteolytic lesions (3). The patient in this case had developed multiple metastases throughout the body. The bone metastases primarily showed osteogenic bone destruction, which is extremely rare.

According to the morphology of the tumor, PHA can be divided into four types: giant type, multiple nodular type, diffuse microscopic nodular type, and mixed type (4). Some studies have reported that typical imaging findings include: (I) hemorrhage, necrosis, and cystic degeneration; (II) unclear or indistinct margins; and (III) hemangioma-like enhancement on contrast-enhanced scans (with marginal enhancement in the arterial phase, and progressive centripetal filling in the portal and delayed phases) (5). This female, who had a rare type of diffuse small nodules, only showed uneven enhancement of the liver parenchyma, without typical hemangioma-like enhancement pattern on scanning. In the hepatic arterial phase, small, immature arteries were observed, and the blood vessels were slender and rigid with blurred edges. In the venous phase, the edges of the hepatic vein appeared blurred, and the hepatic segment of the inferior vena cava appeared flat.

The female patient also had HSOS. The pathological basis of HSOS is the injury and shedding of hepatic sinusoidal endothelial cells, fiber deposition, and subsequent hepatic sinusoidal dilatation and congestion, liver cord compression, cholestasis, and hepatocyte ischemia, degeneration, and necrosis (6). The tumor in this patient had infiltrated most of the liver. PHA is a malignant epithelial cell that often grows along the existing vascular lumen, sinusoidal or cavernous lumen, and forms dysplastic blood vessels or sinusoids (7). PHA combined with HSOS has been reported in previous cases (1). Therefore, the authors speculate that diffuse micronodular PHA is more likely to be associated with HSOS than massive PHA.

Conclusions

PHA is characterized by a low incidence rate and a high mortality rate. It differs significantly from giant and multiple nodular PHA in terms of its imaging manifestations, and is difficult to diagnose in clinical practice. Moreover, when accompanied by HSOS, PHA often leads to coagulation dysfunction, contraindicating percutaneous liver biopsy. Transjugular liver biopsy can be used to diagnose PHA safely (1). The optimal treatment for PHA is still uncertain. At present, surgical resection is the optimal method, and liver transplantation is contraindicated for PHA. If patients do not receive treatment, most do not survive more than 2 years. Early diagnosis and treatment could improve the survival of patients (8). The imaging findings of this case may aid in the early diagnosis of diffuse micronodular PHA.

Acknowledgments

None.

Footnote

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-2025-1482/coif). The 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’s family 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.

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