Hepatic epithelioid angiomyolipoma: magnetic resonance imaging characteristics

Li, Junhao; Zhang, Hongxia; Guo, Jiajun; Ahmad, Awais; Gao, Shanshan; Li, Jianke; Chen, Lingli; Ji, Yuan; Zeng, Mengsu; Wang, Mingliang

doi:10.1007/s00261-023-03818-z

Cited by 2 publications

(3 citation statements)

References 40 publications

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“…Hepatic AML primarily consists of fat, blood vessels, and PEC, and if PEC largely predominates, it can be defined as a type of PEComa called hepatic epithelioid AML (HEAML). A recent study conducted in 2023 identified an additional 113 cases of HEAML as a distinct subtype of PEComa ( 15 ). With the increasing clinical understanding of PEComa, it becomes evident that the existing cases may not precisely reflect the actual incidence of hepatic PEComa.…”

Section: Discussionmentioning

confidence: 99%

“…In our case, the hepatic lesion demonstrated distinct arterial phase enhancement followed by gradual clearance in the venous and delayed phases ( Figure 1 ). The presence of a rich arterial blood supply and relatively regular morphology of the liver mass should be distinguished from other liver masses, such as HCC, HA, and focal nodular hyperplasia (FNH) ( 15 ). While PEComa often contains fat, this particular hepatic PEComa is primarily composed of epithelioid cells, with no detectable fat component observed, which also indicates the subtype of HEAML.…”

Section: Discussionmentioning

confidence: 99%

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Hepatic perivascular epithelioid cell tumor resembling hepatic adenoma and hepatocellular carcinoma on preoperative imaging: a case report

Zhu,

Song,

Wang

et al. 2024

Front. Oncol.

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Perivascular epithelioid cell tumor (PEComa), an uncommon mesenchymal neoplasm, arises from specialized perivascular epithelioid cells exhibiting distinct features of smooth muscle and melanocytic differentiation with unpredictable behavior. PEComa tends to occur more commonly in the uterus and kidneys; its occurrence in the liver is exceedingly rare. We presented a case of a 29-year-old woman with hepatic PEComa and evaluated the tumor with MRI, integrated 18F-fluorodeoxyglucose (FDG), and 68Ga-fibroblast activation protein inhibitor (FAPI) PET/CT scans at presentation. The patient had a history of intermittent utilization of oral contraceptive drugs for several years. An abdominal ultrasound in a physical examination from an outside institution revealed a mass in the liver. A contrast-enhanced abdominal MRI revealed restricted diffusion on diffusion-weighted imaging (DWI) and rapid contrast enhancement and washout patterns in the hepatic lesion, suggesting hepatic adenoma (HA) or hepatocellular carcinoma (HCC). Further assessment was carried out using 18F-FDG and 68Ga-FAPI PET/CT scans. The hepatic lesion was non-FDG avid, whereas increased tracer uptake was observed on the 68Ga-FAPI PET/CT. Subsequently, laparoscopic partial resection of liver segment V was performed. Immunohistochemical analyses demonstrated positive staining for HMB45, Melan-A, and SMA while showing negative results for AFP, glypican-3, hepatocyte, and arginase-1. The results were indicative of a hepatic PEComa diagnosis based on these findings. We also review the current literature on the clinical characteristics, pathological features, and challenges in the diagnosis of hepatic PEComa.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Hepatic perivascular epithelioid cell tumor resembling hepatic adenoma and hepatocellular carcinoma on preoperative imaging: a case report

Zhu,

Song,

Wang

et al. 2024

Front. Oncol.

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“…Although these findings were mentioned in prior literature, they remain uncommon [ 15 ]. Such cystic foci are seldom seen in iCCA but have been reported in HCC [ 22 ] and hepatic epithelioid angiomyolipoma [ 24 ]. The emergence of these cystic foci might result from endothelial damage of the sinusoid due to increased intratumor pressure within encapsulation or hemorrhage inside the lesion [ 22 , 25 , 26 ].…”

Section: Discussionmentioning

confidence: 99%

The MR Imaging of Primary Intrahepatic Lymphoepithelioma-like Cholangiocarcinoma: A Diagnostic Challenge

Liu,

Guo,

et al. 2023

Diagnostics

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Purpose: To characterize the magnetic resonance imaging features of primary intrahepatic lymphoepithelioma-like cholangiocarcinoma (LELCC). Materials and Methods: Thirty-four patients with 38 histologically confirmed LELCCs were enrolled retrospectively from January 2014 to August 2022. We evaluated the clinical features, histologic findings, and imaging manifestations on dynamic enhanced MRI. Results: 74% (25/34) of the cases were associated with EBV infection. Moreover, patients infected with EBV exhibited a lower level of Ki-67 proliferation. The serum CA199 level was elevated in 10 patients. The median tumor diameter was 2.8 cm (range, 1.1–8.7 cm). Most tumors were well-defined with a smooth or lobulated margin and showed peripheral hyperintensity and central hypointensity on T2-weighted imaging (T2WI). T2 hyperintense foci were recognized in 8 patients. In the dynamic enhanced MRI, 21 tumors demonstrated Type A enhancement pattern (rim enhancement), 10 demonstrated Type B (rapid wash-in and wash-out), and seven demonstrated Type C (rapid wash-in without wash-out). Capsular enhancement in PVP or DP was found in 22 tumors. A few patients had satellite lesions, portal vein thrombosis, bile duct dilatation, and distal metastasis. Lymph node metastases were discovered pathologically in 11 patients. Conclusions: MRI findings of LELCC vary and are non-specific. While a majority of LELCCs exhibit typical features of intrahepatic cholangiocarcinoma (iCCA), unique findings like T2 hyperintense foci or capsular enhancement could suggest LELCC. EBV infection and elevated tumor markers can aid in differentiation. However, given the mimics of some cases of liver hypervascular lesions, histological examination remains essential for definitive diagnosis.

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Hepatic epithelioid angiomyolipoma: magnetic resonance imaging characteristics

Cited by 2 publications

References 40 publications

Hepatic perivascular epithelioid cell tumor resembling hepatic adenoma and hepatocellular carcinoma on preoperative imaging: a case report

Hepatic perivascular epithelioid cell tumor resembling hepatic adenoma and hepatocellular carcinoma on preoperative imaging: a case report

The MR Imaging of Primary Intrahepatic Lymphoepithelioma-like Cholangiocarcinoma: A Diagnostic Challenge

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