Abdominal perivascular epithelioid cell tumor (PEComa) without visible fat: a clinicopathologic and radiological analysis of 16 cases

• DOI: 10.1007/s11547-020-01241-8
• Publication Date: 2020-06-19T11:03:51Z
• Authors: Jiaxi Hu; Wenguang Liu; Simin Xie; Mengsi Li; Kangtao Wang; Wenzheng Li
• Keywords: Adult; Male; Perivascular Epithelioid Cell Neoplasms; Middle Aged; Magnetic Resonance Imaging; 3. Good health; 03 medical and health sciences; 0302 clinical medicine; Abdomen; Humans; Female; Tomography, X-Ray Computed; Aged; Retrospective Studies

To retrospectively review the clinicopathological features and computed tomography (CT) and magnetic resonance imaging (MRI) findings of abdominal perivascular epithelioid cell tumor without visible fat (PEComawvf).Sixteen patients with surgically and pathologically confirmed perivascular epithelioid cell tumor without visible fat were enrolled. Their clinicopathological data and imaging findings were retrospectively reviewed. The CT and MRI features, including location, size, shape, margin, density, calcification, cystic necrosis and enhancement pattern, were analyzed.There were 4 males and 12 females (median age, 46 years; range, 21-65 years) in this study. All 16 patients were diagnostic asymptomatic unenhanced CT or MRI and revealed a well-defined (n = 13), oval (n = 10), mass with heterogeneous (n = 6) or homogeneous density/signal intensity (n = 7), calcification and hemorrhage was no found in any cases. On enhanced CT/MRI, markedly enhancement patterns (n = 14) were observed. The "peripheral enhancement" sign was observed in 13 cases. One in 16 cases recurrence was discovered during the follow-up period.Dynamic CT, MRI and pathology of PEComawvf had some characteristics of non-aggressive pattern of performance, and MRI would provide beneficial detection of microscopic fat. Enhanced imaging showed PEComawvf is characterized by a "peripheral enhancement" with a marked enhancement pattern. Knowing these characteristics could contribute to improving the understanding abdominal PEComawvf and related palliative care.