PEComa—A Rare Liver Tumor

Krawczyk, Marek; Ziarkiewicz‐Wróblewska, Bogna; Wróblewski, Tadeusz; Podgórska, Joanna; Grzybowski, Jakub; Gierej, Beata; Krawczyk, Piotr; Nyckowski, P; Kornasiewicz, Oskar; Patkowski, Waldemar; Remiszewski, Piotr; Zając, Krzysztof; Grąt, Michał

doi:10.3390/jcm10081756

Cited by 9 publications

(16 citation statements)

References 26 publications

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“…Ultrasonography of the abdomen usually shows hyperechogenic cystic solid lesions with abundant blood supply. These tumors are hypodense to isodense on CT. MRI shows hypointense to isointense in comparison to skeletal muscle on T1-weighted imaging, and these tumors are heterogeneously hyperintense on T2weighted imaging (1,4). Due to abundant vascularization from hepatic artery branches, we can observe that the lesion is strongly and heterogeneously enhanced in ultrasonography involving intravenous contrast media.…”

Section: Insights Into Hepatic Pecomasmentioning

confidence: 92%

“…Additionally, we found epithelioid cells with abundant transparent to eosinophilic cytoplasm forming nests and trabeculae. In smaller neoplastic lesions, there are fewer atypical epithelioid cells and more adipose tissue (4,7,12,13). Immunohistochemically, PEComas are usually positive for melanocyte markers (HMB45, Melan-A, Mitf) and smooth muscle cell differentiation markers (SMA, Desmin), while epithelial cell markers (CK, EMA) and endocrine markers (S-100, Syn and CgA) are negative.…”

Section: Insights Into Hepatic Pecomasmentioning

confidence: 99%

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Insights into clinical diagnosis and treatment of malignant hepatic perivascular epithelioid cell tumor

Li¹,

Zhou

Wang

et al. 2022

IRDR

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PEComa, liver tumor, hepatectomy, mTOR inhibitors, TFE3 Perivascular epithelioid cell tumors (PEComas) are infrequent mesenchymal tumors. They are usually benign, and only a few are malignant. These tumors are more commonly found in middle-aged women. PEComas are mainly composed of differentiated perivascular epithelioid cells arranged radially around the vascular cavity, and they are usually positive for melanocyte markers and smooth muscle cell differentiation markers. Among the PEComas, hepatic PEComas generally have no obvious symptoms and no typical imaging manifestations. Malignant hepatic PEComas are even rarer. So, we explained our insights into clinical diagnosis and treatment of malignant hepatic PEComas, in order to help clinicians and pathologists to further understand PEComas.Perivascular epithelioid cell tumors (PEComas) are extremely infrequent (roughly equivalent to 1 case per 4 million population) mesenchymal tumors and typically diagnosed in females of an age range of 39-56 years old. PEComas are difficult to diagnose, as their morphology can be analogous to that of smooth muscle tumors (1,2). The 2016 World Health Organization (WHO) Classification of Soft Tissue Tumors defined PEComa as a mesenchymal tumor composed of unique cells that focal associate with blood vessel walls and typically express melanocytic and smooth-muscle markers (3).According to existing statistics, most PEComas are benign, and commonly occur in the uterus and gastrointestinal tract. Moreover, PEComas can also develop in organs such as the kidney, lung and liver. These tumors should be considered malignant only if any two or more of the following situations are found: a. tumor diameter > 5 cm; b. infiltrative growth; c. pronounced heteromorphic nuclei; d. number of mitotic figures ≥ 1/50 HPF; e. necrosis; and f. vascular invasion.

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Section: Insights Into Hepatic Pecomasmentioning

confidence: 92%

Section: Insights Into Hepatic Pecomasmentioning

confidence: 99%

Insights into clinical diagnosis and treatment of malignant hepatic perivascular epithelioid cell tumor

Li¹,

Zhou

Wang

et al. 2022

IRDR

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“…A few studies have explored some of the imaging features of PEComa and have found some atypical imaging features. In contrast-enhanced computed tomography (CECT) studies, the arterial phase showed intensive saturation of the vessels in the periphery of the tumor, and the portal and delayed phases showed a marked decrease in the amount of contrast ( 8 , 9 ). In MR studies in the T1 phase, a significant intensity was visible, while the T2 intensity decreased ( 9 , 10 ).…”

Section: Introductionmentioning

confidence: 99%

Specific imaging features indicate the clinical features of patients with hepatic perivascular epithelioid cell tumor by comparative analysis of CT and ultrasound imaging

Gao

Tang

et al. 2022

Front. Oncol.

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ObjectiveThe objective of the study was to explore the CT and ultrasound features and clinical significance of perivascular epithelioid cell tumor (PEComa) of the liver.MethodsEleven hepatic PEComa patients treated in our hospital were retrospectively analyzed based on the characteristics of the imaging results of the patients, including conventional ultrasound, CDFI, contrast-enhanced ultrasound (CEUS), and contrast-enhanced CT (CECT).ResultsCT scans showed that all lesions were hypodense. Ultrasonography showed that lesions were either hyperechoic (4/11, 36.36%), hypoechoic (4/11, 36.36%), isoechoic (1/11, 9.09%), or heterogeneously echoic (2/11, 18.18%). CDFI showed that most of the lesions had an abundant blood supply (9/11, 81.82%). Whether on CT scan or ultrasonography, the margins of the lesions were dominated by clear margins. Ultrasonography revealed more features: hyperechoic patterns around lesions (3/11, 27.27%) and lateral shadow (5/11, 45.45%). The CDFI showed that large blood vessels were observed around the lesions (9/11, 81.82%). CECT shows two enhancement patterns: “fast in and fast out (FIFO)” (8/11, 72.72%) and “fast in and slow out (FISO)” (3/11, 27.27%). CEUS shows that all lesions had the enhancement pattern of “FISO,” which was different from CECT. All lesions displayed rapid enhancement during HAP in CEUS during 7–20 s. Four patients (36.36%) washed out at 60–180 s, another four (36.36%) washed out at 180–300 s, and the remaining three patients (27.27%) showed no signs of washout even at 360 s.ConclusionSome imaging features, such as clear margins, peripheral hyperechoic around the lesion, lateral shadow, the large blood vessels around lesions, and the “FISO” enhancement pattern, may indicate expansive growth of the tumor and be helpful in the diagnosis of PEComa. Ultrasound images may provide more details for clinical reference.

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“…PEComa family tumors are rare (up to 1 case per 4 million population) and usually occur sporadically. Radical resection may be the curative treatment of most PEComa cases [2,3]. Nevertheless, selected cases show malignant behavior with infiltrative growth, local recurrences after surgical resection, and/or metastatic spread [2,4].…”

Section: Introductionmentioning

confidence: 99%

“…Due to the low incidence of PEComa in the general population, reports on the use of sirolimus in this population of patients are limited. Most case series that have been published report on the efficacy of surgery in PEComa patients [3,28,29]. The largest case series from Royal Marsden Hospital covered ten consecutive patients treated with sirolimus or temsirolimus between 2007 and 2013 [30].…”

Section: Introductionmentioning

confidence: 99%

Efficacy of Sirolimus Treatment in PEComa–10 Years of Practice Perspective

Świtaj

Sobiborowicz

Teterycz

et al. 2021

JCM

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Perivascular epithelioid cell tumors (PEComa) represent a family of rare mesenchymal tumors resultant from deregulation in mTOR pathway activity. The aim of this study is to evaluate the long-term efficacy of targeted PEComa treatment. We reviewed all consecutive patients with PEComa who started systemic treatment with sirolimus in our reference sarcoma center between January 2011 and August 2020. Histopathology of PEComa was reviewed and confirmed in all cases by a designated sarcoma pathologist. Any surviving progression-free patients were censored at the last follow-up (31 March 2021). Survival curves were calculated according to Kaplan–Meier method and compared with the log-rank test or a Cox proportional hazard model. Fifteen (12 females and 3 males) consecutive PEComa patients were treated. The median age of patients treated systemically was 50 years. Median progression-free survival (PFS) was 4.9 months (95% CI: 3.8-NA) for first-line chemotherapy and was not reached (95% CI: 42.0-NA) for sirolimus as first-line therapy. There was one objective response (OR) in the chemotherapy group. The OR rate reached 73% (11/15 cases) for sirolimus regardless of the treatment line. All patients archived disease control. Three patients died due to disease progression after 55, 32, and 32 months since metastatic disease diagnosis. After a median follow-up of 55.7 (range: 3.2–220) months, the 5 yr OS was 65% (CI 95% 39–100). Our study is the largest single-institution report on PEComa systemic targeted therapy and fills the gap in the field of advanced PEComa care since the FDA/EMEA approval of sirolimus.

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PEComa—A Rare Liver Tumor

Cited by 9 publications

References 26 publications

Insights into clinical diagnosis and treatment of malignant hepatic perivascular epithelioid cell tumor

Insights into clinical diagnosis and treatment of malignant hepatic perivascular epithelioid cell tumor

Specific imaging features indicate the clinical features of patients with hepatic perivascular epithelioid cell tumor by comparative analysis of CT and ultrasound imaging

Efficacy of Sirolimus Treatment in PEComa–10 Years of Practice Perspective

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