Why the study?
Primary hepatic angiosarcoma and fat-poor angiomyolipoma are uncommon vascular liver tumors that require imaging and pathology to avoid misdiagnosis.
Case Report (n=1)
Highlights the importance of multimodality imaging and biopsy in diagnosing rare liver vascular tumors like fat-poor AML in patients with VHL syndrome.
Alerts clinicians to rare hepatic tumors in VHL; leaves open validation of imaging criteria in larger cohorts.
BACKGROUND: This article examines primary hepatic angiosarcoma (PHA) and fat-poor angiomyolipoma (AML), two uncommon vascular cancers. Clinical decisions in these situations are frequently aided by pathology reports and imaging techniques. Uncommon malignant tumors of the vascular endothelium include PHA. Another diagnosis that should not be overlooked when employing contrast-enhanced MR and contrast-enhanced computed tomography (CT) imaging techniques is fat-poor AML, one of the uncommon vascular tumors of the liver. In both conditions, biopsy is the primary means of diagnosis. CASE SUMMARY: In our article, besides the diagnosis of PHA, fat-poor AML, one of the other rare vascular tumors of the liver, is mentioned. In the case, a 50-year-old female patient with VHL Syndrome was admitted to our hospital with nonspecific lesions such as right upper quadrant pain, weight loss, and nausea. Abdominal ultrasonography (US) revealed a hypoechoic heterogeneous lesion with occasional faint contours. In computed tomography, it was observed as a hyperdense nodular lesion in segment 4. Magnetic resonance imaging (MRI) revealed that the lesion did not contain fat. In connection with the known history of VHL Syndrome, we first evaluated the possibility of AML. Thereupon, a histopathological sample was taken and the diagnosis was made as fat-poor AML with 5% fat content. CONCLUSION: In conclusion, PHA in our case report and fat-poor AML in our clinic are two uncommon liver vascular malignancies with comparable incidences. Important imaging techniques like contrast-enhanced US (CEUS), CECT, and CEMRI give us substantial advantages in both cases. However, a biopsy is used to provide the final diagnosis.
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Gülmez et al. (2023) conducted a case report in Fat-poor angiomyolipoma and VHL Syndrome (n=1). Imaging techniques (US, CT, MRI) and biopsy was evaluated on Diagnosis of fat-poor angiomyolipoma.
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