Hepatic Angiosarcoma Associated with Chronic Schistosomiasis in a Patient with Decompensated Cirrhosis: A Rare Case Report

Abstract

Introduction

Hepatic angiosarcoma (HA) is a rare endothelial malignancy with poor prognosis. Chronic Schistosoma infection causes periportal fibrosis and portal hypertension, but has not been established as a risk factor for hepatic vascular tumors. We report a case of high-grade HA coexisting with chronic schistosomiasis in a patient presenting with acute decompensated cirrhosis.

Case Presentation

A 64-year-old Filipino woman with no known chemical exposures presented with progressive abdominal pain, jaundice, ascites, and hepatic encephalopathy. Doppler ultrasound initially suggested Budd-Chiari syndrome, later excluded on advanced imaging. MRI showed multifocal hepatic lesions, and CT demonstrated multiple intra-abdominal hematomas without active bleeding. Liver biopsy confirmed poorly differentiated HA (CD31+/CD34+) and revealed calcified Schistosoma eggs. Her course was complicated by hemorrhagic shock after biopsy and paracentesis, spontaneous bacterial peritonitis, acute kidney injury, and worsening hepatic encephalopathy. She died despite aggressive supportive care.

Conclusion

This case illustrates the diagnostic challenges and rapid progression of HA, especially in coagulopathic patients where biopsy carries significant risk. The coexistence of chronic schistosomiasis raises the possibility of parasitic inflammation contributing to hepatic vascular tumorigenesis. HA should be considered among unexplained hepatic masses with atypical imaging and clinical features.

Introduction

Hepatic angiosarcoma (HA) is a rare endothelial malignancy accounting for less than 1% of primary malignant liver tumors, with an estimated 200 cases diagnosed annually worldwide [1]. Prognosis is poor, with median survival of only a few months and a 1-year survival rate of 12.8% [2]. Risk factors include exposure to vinyl chloride monomer, arsenic, thorium dioxide, and ionizing radiation, although approximately 75% of cases have no identifiable cause [1, 2]. Currently, there is no evidence to suggest an association between angiosarcoma and parasitic infections such as schistosomiasis.

Schistosomiasis, caused by trematode blood flukes of the genus Schistosoma, affects over 200 million people worldwide. Chronic infection can result in periportal fibrosis, portal hypertension, and cirrhosis [3]. Long-standing inflammation is known to increase hepatocellular carcinoma risk, but only one previous case report has described HA coinciding with schistosomiasis [4].

HA may mimic other hepatic malignancies such as hepatocellular carcinoma, cholangiocarcinoma, or even benign vascular lesions like hemangiomas [5]. Given its rarity, nonspecific presentation, and overlapping imaging features, diagnosis often requires a high index of suspicion and histopathological confirmation.

This case report has been prepared in accordance with the CARE reporting guidelines. The completed CARE Checklist is provided in the online supplementary material (for all online suppl. material, see https://doi.org/10.1159/000549935).

Case Report

A 64-year-old woman of Filipino origin presented with 1.5 months of worsening epigastric pain and fatigue. Two weeks later, she re-presented with persistent abdominal pain, new-onset jaundice, abdominal distention, lower limb swelling, pruritus, dark urine, nausea, and poor oral intake. She declined admission at that time. Two months later, she returned with further deterioration, including altered mental status and reduced responsiveness.

She had no significant past medical history, no known exposure to hepatotoxic chemicals, and no family history of liver disease. Social history was notable for occasional alcohol consumption and no use of tobacco, illicit drugs, or herbal supplements. On examination, she was icteric with tense abdominal distention and shifting dullness consistent with ascites, as well as bilateral symmetric lower limb edema.

Differential Diagnosis

Initial differentials included decompensated cirrhosis from any chronic liver disease, Budd-Chiari syndrome, hepatocellular carcinoma, metastatic liver disease, viral hepatitis, drug-induced liver injury, and autoimmune hepatitis.

Laboratory Results

Imaging

Initial abdominal ultrasound with Doppler demonstrated features of cirrhosis, portal hypertension, mild ascites, and reduced hepatic vein flow, raising suspicion for Budd-Chiari syndrome. Subsequent MRI showed a multinodular cirrhotic liver with multifocal lesions suspicious for malignancy (shown in Fig. 1). Abdominal CT was performed to further characterize the lesions and assess for possible intervention. This revealed multiple intra-abdominal hematomas (largest 10 cm) without active extravasation (shown in Fig. 2), explaining her progressive anemia and hemodynamic instability.

Fig. 1.

MRI abdomen (axial view) demonstrating a multinodular cirrhotic liver with irregular contour and multiple variable-sized lesions scattered throughout the parenchyma, findings highly suspicious for malignancy.

Fig. 2.

Abdominal CT (axial), both arterial (L) and venous (R) phase, showing features of hepatic cirrhosis with multiple focal variable enhancing lesions + multiple collaterals in the splenorenal region. Presence of large (11.2 × 10.5 × 10 cm) hematoma traversing from liver hilum/caudate lobe to the splenic hilum.

Histopathology

Given the uncertain diagnosis and progressive course, image-guided liver biopsy was performed after careful risk assessment. Microscopy demonstrated a poorly differentiated malignant neoplasm of spindle cells forming irregular vascular channels. Immunohistochemistry revealed diffuse CD31 and CD34 positivity, confirming HA. Multiple calcified Schistosoma eggs were also present, indicating chronic schistosomiasis (shown in Fig. 3, 4).

Fig. 3.

H&E stained sections (×20) showing liver parenchyma with angiosarcoma (black arrowhead) and calcified Schistosoma eggs (encircled) (L), and demonstrating a malignant, mitotically active spindle cell neoplasm with vasoformative differentiation (R).

Fig. 4.

Photomicrographs (×2) showing CD31 (L) and CD34 (R) immunostain strongly and diffusely positive on tumor cells.

Clinical Course and Outcome

Following biopsy and therapeutic paracentesis, the patient developed worsening anemia and hemodynamic instability consistent with intra-abdominal bleeding. Table 1 shows ascitic fluid obtained during paracentesis that demonstrated features consistent with spontaneous bacterial peritonitis, with WBC 1,200 × 10⁶/L (78% neutrophils), protein 32 g/L, albumin 19 g/L, and glucose 5.2 mmol/L; fluid culture grew Escherichia coli. She was initiated on intravenous piperacillin-tazobactam. Despite antimicrobial therapy and transfusion support, she progressed to hemorrhagic shock, respiratory failure, and worsening hepatic encephalopathy. Surgical intervention was deemed futile given her critical condition and poor prognosis. She was admitted to the intensive care unit with hepatic encephalopathy several days later and subsequently developed cardiopulmonary arrest and died. The adverse event was considered likely related to post-procedural hemorrhage in the context of coagulopathy and fragile tumor vasculature.

Table 1.

Key laboratory results and ascitic fluid analysis

Test category	Parameter	Result/findings
Hematology	Hemoglobin	13.0 g/dL
	WBC (peripheral)	8.5 ×109/L
	Platelets	130 ×109/L
Coagulation	INR	1.69 → 2.7 (peak)
Liver function	Total bilirubin	24.0 mg/dL → 52.3 mg/dL (peak)
	AST	145 U/L
	ALT	67 U/L
	Albumin (serum)	1.4 g/dL
Renal function	Creatinine	1.98 mg/dL
Viral hepatitis panel	Hepatitis A, B, C, E	Negative
Ascitic fluid	WBC	1,200 ×106/L
	RBC	300 ×106/L
	Polymorphs	83%
	Neutrophils	78%
	Albumin (fluid)	19 g/L
	Protein (fluid)	32 g/L
	Glucose (fluid)	5.2 mmol/L
	Culture	Escherichia coli (positive)
Interpretation	​	Consistent with spontaneous bacterial peritonitis (SBP) due to elevated neutrophils and positive culture

Discussion

This case highlights several diagnostic and therapeutic challenges in HA. Clinically, HA presents with vague symptoms such as abdominal pain, fatigue, and weight loss, and often mimics other hepatic lesions [3]. Imaging findings are heterogeneous; Doppler may show altered hepatic vein flow (as in this case), prompting consideration of Budd-Chiari syndrome, but absence of thrombosis on further imaging helps exclude it.

Histopathology remains the gold standard, with tumor cells typically expressing endothelial markers such as CD31, CD34, and factor VIII-related antigen [3]. Prognosis is extremely poor, and most patients are ineligible for surgical resection due to multifocal disease. Chemotherapy and radiotherapy have limited efficacy.

Schistosomiasis causes chronic granulomatous inflammation around deposited eggs, leading to periportal fibrosis, portal hypertension, and cirrhosis. The persistent inflammatory state generates reactive oxygen species and may induce mutations in tumor suppressor genes (e.g., p53) and promote vascular endothelial growth factor-mediated angiogenesis [4, 6]. Although no causal relationship between schistosomiasis and HA has been established, the coexistence of both conditions in this and a previously reported case [6] suggests a possible pathogenic link worthy of further study.

Conclusion

This case describes the rare coexistence of HA and chronic schistosomiasis in a patient with decompensated cirrhosis. HA should be considered in the differential diagnosis of unexplained hepatic masses, especially when imaging and clinical presentation are atypical. Early biopsy is essential for diagnosis, though procedural risks are significant in coagulopathic patients. While schistosomiasis is not an established risk factor for HA, cases like this prompt further investigation into the potential role of chronic parasitic inflammation in hepatic vascular tumorigenesis.

Statement of Ethics

Ethical approval was not required for this case report in accordance with institutional policies. The Sheikh Shakhbout Medical City (SSMC) Research Ethics Committee, Abu Dhabi, UAE, reviewed the case and confirmed that it was exempt from formal ethics approval. Written informed consent for publication of the patient’s clinical details and accompanying images was obtained from the patient’s next-of-kin.

Conflict of Interest Statement

The authors have no conflicts of interest to declare.

Funding Sources

This study was not supported by any sponsor or funder.

Author Contributions

T.S. conceived the case report, coordinated clinical data collection, and supervised the project. C.K. performed the literature review, drafted the initial manuscript, and contributed to data interpretation. N.S.B.G. assisted with data acquisition, imaging review, and critical revision of the manuscript. S.H.A.M. contributed to clinical management of the patient, verified the clinical details, and provided substantial manuscript revisions. All authors reviewed and approved the final manuscript.

Funding Statement

This study was not supported by any sponsor or funder.

Data Availability Statement

All data generated or analyzed during this study are included in this article and its online supplementary files. Further inquiries can be directed to the corresponding author.