Primary perivascular epithelioid cell tumors of the liver: CT/MRI findings and clinical outcomes

• Published in: Abdominal imaging 2017-06, Vol.42 (6), p.1705-1712
• Main Authors: O’Malley, Martin E., Chawla, Tanya P., Lavelle, Lisa P., Cleary, Sean, Fischer, Sandra
• Format: Article
• Language: English
• Subjects: Adult; Age; Aged; Angiomyolipoma; Benign; Bleeding; Blood vessels; Calcification; Clinical outcomes; Complications; Computed tomography; Contrast Media; Correlation analysis; Female; Gastroenterology; Hemorrhage; Hepatocytes; Hepatology; Humans; Image Interpretation, Computer-Assisted; Imaging; Incidental Findings; Liver; Liver Neoplasms - diagnostic imaging; Liver Neoplasms - pathology; Magnetic Resonance Imaging; Male; Medicine; Medicine & Public Health; Men; Metastases; Middle Aged; Pathology; Patients; Perivascular Epithelioid Cell Neoplasms - diagnostic imaging; Perivascular Epithelioid Cell Neoplasms - pathology; Radiology; Retrospective Studies; Tomography, X-Ray Computed; Tumors
• ISSN: 2366-004X; 2366-0058
• DOI: 10.1007/s00261-017-1074-y

Objectives The purpose of our study was to describe the CT and MRI features of primary PEComas of the liver and to document the associated clinical outcomes. Methods Retrospective study included 20 patients with primary hepatic perivascular epithelioid cell tumors (PEComa) with pathology and clinical outcomes for correlation. Results Study group included 20 patients: 16 women, 4 men; mean age 53 (range 35–77) years. Initial pathology diagnoses were classic angiomyolipoma (AML) ( n  = 11), epithelioid AML ( n  = 7), and PEComa not otherwise specified ( n  = 2). Mean tumor size was 5.1 (range 1.3–15.0) cm. CT/MRI features included well-defined margins 20/20 (100%), arterial enhancement 18/19 (95%), subcapsular location 17/20 (85%), heterogeneous 16/20 (80%), dysmorphic vessels 14/20 (70%), fat 13/20 (65%), hemorrhage 4/20 (20%), cystic components 4/20 (20%), and calcification 1/20 (5%). At the time of discovery, 18 patients were asymptomatic and their tumors were incidentally detected on imaging, and 2 patients were symptomatic. Ultimately, 18 tumors were benign and 2 developed metastases. Conclusions On CT/MRI, most primary hepatic PEComas were well-defined, arterial enhancing, subcapsular, heterogeneous masses that often had dysmorphic vessels and contained fat. Most tumors were benign but complications included local symptoms, bleeding, and malignant change.