Primary hepatic perivascular epithelioid tumor (PEComa)

Schaeffer, David F.; Poulin, Michael

doi:10.5604/16652681.1198822

Cited by 4 publications

(3 citation statements)

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“…The presence of washout in the portal and venous phases raises suspicion for hepatocellular carcinoma (HCC). 4,8,17,18 It can also restrict diffusion like HCC. 1,10 Hypervascular liver tumors such as focal nodular hyperplasia, hemangioma, and adenoma are included in the differential diagnosis.…”

Section: Discussionmentioning

confidence: 99%

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Very Rare Liver Tumor: PEComa Case Report with and a Review of Literature

Yazıcı,

Gündoğdu

2023

Indian J Radiol Imaging

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Perivascular epithelioid cell tumors (PEComas) are very rare mesenchymal tumors. In addition to being rare in general, they are even rarer in the liver. There are various subtypes and there is only one case of liver PEComa reported in the clear cell type in the literature to date. We present the second clear cell type liver PEComa in this case by reviewing the literature data. The hypodense, smooth-edged, ovoid lesion was detected on computed tomography (CT) for performing abdominal pain in a 41-year-old female patient. Magnetic resonance imaging (MRI) was then performed for lesion characterization. The lesion was hypointense on T1-weighted imaging (T1WI), hyperintense on T2-weighted imaging (T2WI). In dynamic phases, it showed marked enhancement on the arterial phase and capsular enhancement with central washout on the portal and late venous phases. The posterior branch of the right portal vein extended into the mass. The lesion was excised and the pathological result was epithelioid clear cell subtype of PEComa. Although the imaging findings are generally nonspecific and certain diagnosis is made histopathologically, radiologists should consider PEComa in the differential diagnosis in the presence of intensely enhanced lesion on the right lobe in female patients. Also, the “large vessel sign” may help in the diagnosis.

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Section: Discussionmentioning

confidence: 99%

“…They are usually discovered incidentally and benign in character. 4 In this study, we aimed to present an incidentally detected liver PEComa and its common radiological features by reviewing the literature data.…”

Section: Introductionmentioning

confidence: 99%

Very Rare Liver Tumor: PEComa Case Report with and a Review of Literature

Yazıcı,

Gündoğdu

2023

Indian J Radiol Imaging

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“…The following are available online at , Table S1: Published cases of primary liver PEComas. References [ 27 , 28 , 29 , 30 , 31 , 32 , 33 , 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 , 75 , 76 , 77 , 78 , 79 , ...…”

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Primary Liver Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review

Kvietkauskas,

Samuolyte,

Rackauskas

et al. 2024

Medicina

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A primary liver perivascular epithelioid cell tumor (PEComa) is an extremely rare entity. In this article, we present a case report with a review of the literature on the patients diagnosed with primary liver PEComa and an elaboration of diagnostic and treatment modalities. A systematic literature search was conducted using the terms “perivascular epithelioid cell tumor”, “PEComa”, “liver”, and “hepatic”. All articles describing patients diagnosed with primary liver PEComa were included. We identified a total of 224 patients of primary liver PEComa from 75 articles and a case from the present study with a significant preponderance of females (ratio 4:1) and with a mean age of 45.3 ± 12.1 years. Most of the patients (114 out of 224, 50.9%) were asymptomatic. A total of 183 (81.3%) patients underwent surgical hepatic resection at the time of diagnosis, while 19 (8.4%) underwent surveillance. Recurrence and metastases were detected in seven (3.1%) and six (2.7%) patients, respectively. In conclusion, surgical resection remains the cornerstone of therapy; however, the presence of nonspecific imaging features makes it difficult to reach a definite diagnosis preoperatively. Therefore, a multidisciplinary approach should be the gold standard in selecting the treatment modality.

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