Next Article in Journal
Ozone Treatment in the Management of Chemotherapy-Induced Peripheral Neuropathy: A Review of Rationale and Research Directions
Previous Article in Journal
The Incidence, Mortality, and Survival Trends of Pancreatic Cancer in Kazakhstan: Data from the National Electronic Registry of Oncological Patients (2014–2023)
 
 
Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
Journals
Cancers
Volume 17
Issue 14
10.3390/cancers17142276
cancers-logo
Submit to this Journal Review for this Journal Propose a Special Issue
► Article Menu

Article Menu

share Share announcement Help format_quote Cite question_answer Discuss in SciProfiles
first_page
settings
Order Article Reprints
Font Type:
Arial Georgia Verdana
Font Size:
Aa Aa Aa
Line Spacing:
Column Width:
Background:
Systematic Review

Characteristics and Treatment of Primary Hepatic Perivascular Epithelioid Cell Tumor (PEComa) in Adults: A Systematic Review

by
Konstantinos Papantoniou
1,
Ioanna Aggeletopoulou
1,*,
Maria Kalafateli
2 and
Christos Triantos
1
1
Division of Gastroenterology, Department of Internal Medicine, University of Patras, 26504 Patras, Greece
2
Division of Digestive Diseases, Department of Metabolism, Digestion and Reproduction, Imperial College London, London W2 1NY, UK
*
Author to whom correspondence should be addressed.
Cancers 2025, 17(14), 2276; https://doi.org/10.3390/cancers17142276
Submission received: 5 June 2025 / Revised: 4 July 2025 / Accepted: 7 July 2025 / Published: 8 July 2025
(This article belongs to the Section Systematic Review or Meta-Analysis in Cancer Research)
Browse Figure
Review Reports Versions Notes

Simple Summary

Perivascular epithelioid cell tumors (PEComas) are a rare group of neoplasms characterized by their presence in areas surrounding blood vessels and the expression of special immunohistochemical markers. Reported cases regarding PEComas found in the liver are extremely rare, and specific guidelines regarding decision-making for these patients are currently lacking. The lack of distinctive clinical and radiological characteristics makes differential diagnosis with other tumors of the liver a challenge for clinicians. In this systematic review, we aim to characterize the clinical presentation, diagnostic approach, treatment, and outcomes of adult patients with hepatic PEComa. We hope that the information we provide will assist clinicians in their everyday practice and improve patient care, while also giving directions for future prospective studies on this subject.

Abstract

Background: Perivascular epithelioid cell tumors (PEComas) are a rare group of mesenchymal neoplasms with distinctive histological and immunohistochemical features. This systematic review aims to characterize the clinical presentation, diagnostic approach, treatment, and outcomes of adult patients with hepatic PEComa. Methods: We performed a systematic literature search for English-language articles regarding hepatic PEComas using the terms (perivascular epithelioid cell tumor) OR (PEComa) AND (liver) OR (hepatic), up to 25 May 2025. Results: A total of 145 studies encompassing 281 patients were included in the analysis. Most studies originated from Asia. The mean age at diagnosis was 46 years (IQR: 35.25–53.75) with a female predominance. The underlying comorbidities were uncommon among the reported cases, and more than half were asymptomatic at presentation. The tumor presented as a single liver lesion in almost 9 out of 10 patients. Surgical excision was the primary treatment, and diagnosis in 74% of patients was made with positive immunohistochemistry for markers such as HMB-45 and smooth muscle actin. A malignant phenotype was reported in 30 cases. The median follow-up duration was 24 months (IQR: 12–48); recurrence occurred in 17 patients, and disease-related mortality occurred in 8 patients. Conclusions: Primary hepatic PEComa is a rare liver tumor with mostly benign clinical behavior and non-specific presentation. Future studies are needed to support clinician decisions regarding this entity and improve patient care.

1. Introduction

Perivascular epithelioid cell tumors (PEComas) are a rare group of mesenchymal neoplasms that were originally described in 1992 [1]. The unique features of these tumors are mainly attributed to their composition. Perivascular epithelioid cells are a type of cell with distinctive histological and immunohistochemical characteristics. They are typically located in areas surrounding blood vessels, while they also express both melanocytic and smooth muscle markers [2]. Angiomyolipomas (AMLs), lymphangioleiomyomatosis, clear-cell “sugar” tumors, and tumors classified as PEComas not otherwise specified (PEComa—NOS) are classified as members of this group of neoplasms [3,4]. These tumors may arise in different locations including the female reproductive tract, skin, bones, lungs, gastrointestinal (GI) tract, and kidneys. Their prevalence appears to be higher in females [5]. Most PEComas are benign; however, malignant cases have also been reported [6,7].
The liver is a rare location for the development of PEComas. Some of these tumors, especially AMLs, may appear due to tuberous sclerosis complex (TSC), a genetic disorder characterized by the presence of tumors located in different body parts and a distinct set of neurodevelopmental syndromes known as TSC-associated neurological disorders [8]. However, many liver PEComas are sporadic. Surgical excision of these neoplasms has proven to be effective, but the lack of distinctive radiological and clinical features makes preoperative diagnosis challenging. As primary liver PEComas are rare, most knowledge regarding their clinical course and treatment is derived from single case reports and case series. The lack of specific guidelines regarding the clinical approach to these tumors only adds to the difficulties that clinicians face while treating these patients [9]. These neoplasms also often resemble other liver abnormalities, such as hepatocellular carcinoma (HCC) and focal nodular hyperplasia (FNH), and inappropriate treatment might be chosen [10].
A systematic search and identification of cases of primary hepatic PEComas could consolidate data from different reports and give a more comprehensive picture of clinical characteristics and established or emerging treatment modalities directed against these neoplasms. The aim of the present study was to systematically review reported case reports and case series of primary liver PEComas to determine patient baseline characteristics, clinical course, pathological, immunohistochemical, and radiological tumor features, the method and timing of diagnosis, and treatment types. These findings could help establish a standardized diagnostic and therapeutic approach for these patients.

2. Materials and Methods

2.1. Search Strategy

A systematic literature search in PubMed, Scopus, and Cochrane libraries was conducted on 25 May 2025 to retrieve all relevant articles. This article is a systematic review written in accordanceto the guidelines from the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) [11]. The following search terms were used: ((perivascular epithelioid cell tumor) OR (PEComa)) AND ((liver) OR (hepatic)).

2.2. Study Selection

EndNote version 21 was used to delete duplicate files. The titles and abstracts of the unique articles were screened first independently by two different authors (K.P. and I.A.). Full-text screening was then performed again by two independent authors (K.P. and I.A.), and all potentially eligible cases of primary liver PEComa were included. The references for each article were manually assessed to complete the research.

2.3. Eligibility Criteria

In this systematic review, we included case series and case reports of adult patients who were diagnosed with liver PEComa after the age of 18. Articles were included only if they were written in the English language and if they provided information regarding the patient’s initial symptoms and follow-up of at least 2 months after diagnosis. In order to reduce potential limitations in data interpretation, studies providing only aggregated data or mean values, without individual patient-level information, were not included. Cases that did not fulfill these criteria and other non-relevant article types, such as reviews and expert opinions, were excluded. Studies that focused only on specific tumor characteristics, such as radiological findings or immunohistochemistry, were also excluded. Regarding articles based on the same patient dataset, papers with the longest follow-up or the largest sample size were included. Disagreements between the authors regarding study eligibility were resolved by consensus. If no agreement could be reached, conflicts were resolved after discussion with a third senior author (C.T.).

2.4. Data Extraction and Synthesis

For each study, the following information were extracted based on a pre-specified form: first author, year of publication, continent, age, sex, comorbidities, diagnosis of tuberous scleroris, symptoms at presentation, concurrent primary PEComa in a different organ, liver tumor size, tumor nodules, tumor location on the liver, method of diagnosis, pre-surgical biopsy, PEComa cell type, malignancy, metastasis, surgery, arterial chemoembolization, adjuvant treatment, neoadjuvant treatment, immunohistochemical markers, follow-up duration, and relapse. We used the data from all available cases to create a secondary cohort. References were constructed via EndNote 21. Version 29.0.2.0 of IBM SPSS for Windows was used for statistical analysis.

3. Results

We identified a total of 987 records from the three databases, along with an additional 20 records through manual reference checks (Figure 1). After removing 137 duplicates, 870 articles remained for title and abstract screening. Subsequently, 390 full-text articles were evaluated for eligibility. Ultimately, 145 studies reporting on 281 patients with primary liver PEComa were included in the analysis. Individual study origins, comorbidities, imaging modalities, immunohistochemical profiles, and follow-up details are provided in Table S1.
In Table 1, we provide a comprehensive overview of patient demographics, clinical presentation, diagnostic workup, tumor characteristics, and treatment modalities for the primary hepatic PEComa cases included in the current study.
The majority of studies originated from Asia (97 studies, 66.6%), followed by Europe (35 studies, 24.1%), North America (8 studies, 5.5%), Africa (3 studies, 2.1%), South America (1 study, 0.7%), and Australia (1 study, 0.7%). Of the total, 48 studies (33.1%) were from China, and 24 (16.55%) were from Japan. Most studies were single case reports (121, 83.4%), while 24 (16.6%) were case series. The largest case series included was published by Jung et al. [12] and Jiang et al. [13], involving 23 and 19 patients, respectively.
The mean age of patients was 46 years (IQR: 35.25–53.75). The majority (208 patients, 74%) were female, while 73 (26%) were male. Most cases (197, 70.1%) had no significant medical history. Reported liver comorbidities included hepatitis B (23 patients, 8.2%), hepatitis C (5 patients, 1.8%), metabolic dysfunction-associated steatotic liver disease (MASLD) (3 patients, 1.1%), HCC (3 patients, 1.1%), hemangioma (3 patients, 1.1%), and cirrhosis (3 patients, 1.1%). Additionally, nine patients (3.2%) were diagnosed with tuberous sclerosis, and six (2.1%) were diagnosed with Li–Fraumeni syndrome. A history of extrahepatic malignancy was noted in 30 patients (10.7%).
The most commonly reported symptoms were abdominal pain (84 patients, 29.9%), abdominal discomfort (24 patients, 8.54%), fever (15 patients, 5.34%), weight loss (10 patients, 3.56%), fatigue (7 patients, 2.49%), vomiting (5 patients, 1.78%), and nausea (5 patients, 1.78%). Elevated alpha-fetoprotein (AFP) levels were noted only in three patients (1.1%). PEComa was detected incidentally in 157 patients (55.9%) who were asymptomatic. In 26 patients (9.3%), the tumor was discovered as a palpable mass. It was identified through abdominal ultrasound in 120 patients (43.7%), CT in 43 patients (13.7%), MRI in 11 patients (3.9%), and during unrelated abdominal surgery in 2 cases. Methods for initial tumor detection were not described for 79 patients.
During the diagnostic workup, 152 patients (54.1%) underwent ultrasound, 221 (78.6%) had CT scans, 156 (55.5%) underwent MRI, and 31 (11%) had PET-CT imaging. Tumor enhancement in the arterial phase after contrast administration with subsequent washout sign in the delayed or portal phases was observed in 93 cases (33.1%). Radiological diagnosis correctly identified PEComa in only 29 cases (10.3%), while differential diagnoses often included HCC, adenoma, focal nodular hyperplasia, and hemangioma.
Preoperative biopsy was performed in 59 patients (21%), with 44 (74.5%) receiving a confirmed PEComa diagnosis. The median tumor size was 5.15 cm (IQR: 3–9.3), with the largest tumor, measuring 30 × 25 cm, reported by Zhou et al. [14]. A single liver lesion was present in 249 patients (88.6%), and multiple nodules were present in 22 (7.8%) cases, while data were missing for 10 patients.
Tumor location was predominantly in the right hepatic lobe (149 cases, 53%), followed by the left lobe (101 cases, 33.9%) and the caudate lobe (12 cases, 4.3%). In 13 cases (4.6%), tumors extended across both the right and left lobes, with 1 case (0.4%) involving both the right and caudate lobes, and another (0.4%) involving the left and caudate lobes. Localization was not specified in four cases.
Immunohistochemical analysis was conducted in 268 cases (95.4%). Tumors were most frequently positive for HMB-45 (261/264, 98.9%), MELAN-A (124/132, 93.2%), smooth muscle actin (SMA) (179/200, 89.5%), and vimentin (62/79, 78.5%). They were commonly negative for S-100 (85/126, 67.5%), desmin (45/83, 54.2%), cytokeratins (123/125, 98.1%), epithelial membrane antigen (EMA) (26/28, 92.9%), and HepPar-1 (43/44, 97.7%).
Final diagnosis was established through radiology in 29 cases (10.3%), biopsy in 44 cases (15.7%), and post-surgical histopathology in 208 cases (74%). The tumors were classified as angiomyolipoma (AML) in 197 patients (70.1%), PEComa-NOS in 74 (26.3%), CCMMT of the falciform ligament or ligamentum teres in 7 (2.5%), and clear-cell sugar tumor in 3 (1.1%). Concurrent PEComas were found in the kidney (14 cases, 5%), lungs (1 case, 0.4%), and both kidneys and omentum (1 case, 0.4%).
Surgery was the primary treatment in 251 patients (89.3%), with 1 receiving neoadjuvant therapy and 3 undergoing adjuvant treatment postoperatively. An initial non-surgical approach was taken in 30 patients (10.7%), of whom 13 (43.3%) eventually required surgery due to tumor progression. Additional treatments included transarterial chemoembolization (TACE) in 11 patients (3.9%), transarterial embolization (TAE) in 3 (1.1%), radiofrequency ablation (RFA) in 3 (1.1%), sorafenib in 3 (1.1%), tamoxifen in 1 (0.4%), microwave ablation in 1 (0.4%), and liver transplantation in 1 patient (0.4%).
A malignant phenotype was reported in 30 cases (10.7%), with major vessel invasion observed in 8 (2.85%). One patient died before discharge following the initial hospitalization. Among those discharged, the median follow-up duration was 24 months (IQR: 12–48). Recurrence occurred in 17 patients (6%), and 7 patients (2.5%) died as a result of the disease.

4. Discussion

This is a systematic review of reported cases of primary liver PEComas. These neoplasms are more frequently observed in females and affect individuals across a wide age range, though they are more commonly diagnosed in middle-aged patients [15]. Cases have been reported globally, with contributions from various regions. Most articles included in our analysis originated from Asia. Our data are consistent with another systematic review on hepatic PEComas by Kvietkauskas et al., in which 61.3% of reported cases were from Asian centers [16]. Possible explanations include a genetic predisposition in these populations for hepatic PEComa development and more effective reporting mechanisms in these countries.
The pathophysiology of these tumors is not fully understood, and the normal cell of origin for PEComas has not been identified. Hypotheses include the abnormal differentiation of smooth muscle progenitor cells found in concurrent malignancies such as leiomyosarcomas [17] and the simultaneous expression of smooth muscle and melanocytic phenotypes by dysregulated multipotent stem cells of neural crest origin [18]. PEComas have also been observed in patients with genetic disorders. TSC is an autosomal dominant genetic condition characterized by the presence of tumors in many different body organs and neuropsychiatric disorders [19]. Mutations of the TSC1 and TSC2 genes can affect the mechanistic target of the rapamycin (mTOR) signaling pathway and thus disrupt the regulating mechanisms of cell growth and proliferation, leading to tumor development [20]. However, the dysregulation of the mTOR pathway has also been observed in sporadic hepatic PEComas without TSC evidence. This observation has made the mTOR pathway a suitable treatment target for PEComas even in patients without TSC [21]. Rearrangements of the transcription factor E3 (TFE3) gene are another genetic abnormality associated with these patients [22]. These cases often have different clinical and morphological characteristics compared to conventional PEComas. They are typically found in younger patients without a history of TSC, have a distinct alveolar morphology, exhibit strong TFE3 immunohistochemical reactivity, and are usually not reactive to smooth muscle markers [23]. These findings have led to the suggestion that these tumors might represent a clinical condition with different molecular characteristics [24]. In our study, some liver PEComas were found in patients with Li–Fraumeni syndrome. Li–Fraumeni syndrome is an autosomal dominant condition where mutations in the tumor suppressor protein P53 gene (TP53) contribute to cancer development [25]. TP53 germline mutations have been suggested as a driver of PEComa development in recent years, especially in patients where other notable gene mutations are not found. These tumors often exhibit an aggressive malignant behavior, and concurrent malignancies in many different body areas might occur in these cases [26,27]. Despite their association with PEComa development, these mutations have been found only in a minority of patients, making the pathogenesis of these neoplasms a subject for further investigation.
Most patients with liver PEComas do not have a history of prior liver disease. Many tumors are asymptomatic and are discovered incidentally during routine physical examinations or imaging. Symptoms, when present, are non-specific and may include abdominal pain or discomfort, nausea, vomiting, or fever [28]. Laboratory tests are also non-specific, and markers such as AFP are mainly useful in ruling out other diseases in the differential diagnosis. Liver PEComas typically present as single lesions, although a multinodular appearance does not exclude the diagnosis. They can occur in various liver locations, but they are more frequently found in the right lobe [29]. This may be because the right lobe receives the largest portion of portal vein flow and is thus more exposed to carcinogens and other harmful stimuli from the GI tract [30]. However, the fact that many PEComas occur in other liver regions suggests the possibility of additional developmental mechanisms.
The most common type of PEComa is AML. These tumors often contain a high concentration of mature adipose tissue and centrally located, thick-walled, feeding blood vessels. These features often make them identifiable through routine imaging studies. However, many cases lack these characteristics, complicating their differentiation from other liver tumors [31]. Various histologic subtypes of AML, such as conventional, inflammatory, and epithelioid AMLs (eAMLs), have been described. Conventional AMLs can exhibit lipomatous, myomatous, or angiomatous predominance and are generally benign. eAMLs, by contrast, are more frequently associated with malignant transformation, and closer follow-up is advised for these patients [32].
Hepatic PEComas lack specific radiological characteristics. On imaging, hepatic PEComas are often enhanced in the arterial phase after contrast administration and exhibit variable washout patterns [33]. However, these neoplasms can present with many different imaging features and are often misdiagnosed as different liver tumors. In the present study, PEComa enhancement was observed in the arterial phase in one out of three patients after contrast administration, with a subsequent washout sign in the delayed or portal phases. These observations often make their distinction from HCC very difficult, especially in patients with underlying liver disease and cirrhosis. However, low aFP levels and normal liver parenchyma should alert clinicians to the possibility of hepatic PEComa presence [34]. The well-defined margins and high vascularity of PEComas can also make radiologists confuse them for hepatic hemangiomas [35]. The use of contrast-enhanced imaging can help in these cases. Hemangiomas exhibit a notable peripheral enhancement in the arterial phase, which is usually followed by centripetal fill-in in later phases [36]. FNH is another important mimic of liver PEComas. Both these modalities appear more often in young women and show arterial enhancement after contrast administration. The presence of a large feeding artery and a central scar in CT and MRI imaging is typical of FNH and can make its identification clear [37].
In our study, only 10.3% of PEComas were correctly identified via radiology. Several studies have focused on describing PEComa characteristics across different imaging modalities [38,39]. The presence of fat can aid in their identification, particularly in AML cases. On ultrasound, PEComas typically appear hypoechoic or isoechoic relative to liver parenchyma, and Doppler imaging may reveal a rich vascular supply. While not always observed, the presence of the washout sign on contrast-enhanced ultrasound (CEUS), CT, or MRI can complicate the differential diagnosis with HCC [29]. Despite these challenges, PEComas should be considered in cases of solitary liver lesions in patients without cirrhosis and with negative AFP, even if the washout sign is present. Ma et al. recently proposed that the appearance of an early draining vein and a more significant reduction in T1 relaxation time during the hepatobiliary phase of gadolinium-enhanced abdominal MRI may help distinguish PEComas from HCC [40]. PET-CT scans can help rule out metastatic disease and assess treatment response, but their use is limited by availability and the variable fluorodeoxyglucose uptake of PEComas [41].
Most liver PEComas are diagnosed postoperatively through pathological analysis. As PEComas mimic HCC, they are often initially presumed to be malignant, leading to more extensive liver resections such as segmentectomy or lobectomy. Biopsies can pose risks, including bleeding and malignant spread through the needle tract, potentially causing tumor recurrence [42]. Consequently, many clinicians avoid preoperative biopsies in lesions suspected to be HCC, relying instead on imaging for diagnosis. Moreover, diagnosis of PEComa via biopsy is not always definitive, making its routine use controversial [27]. Nevertheless, ultrasound-guided biopsies can support accurate tumor identification through early immunohistochemical analysis, aid in preoperative planning, and should be considered when malignancy is uncertain [9]. Pathological analysis with immunohistochemistry remains the most reliable diagnostic method for PEComas. The characteristic epithelioid cells typically express melanocytic markers such as HMB-45 and MELAN-A, along with smooth muscle markers like SMA. Due to their mesenchymal origin, they may also express markers such as vimentin [43]. In the liver, additional markers, such as HepPar-1 and AFP for hepatocytes, and cytokeratins and EMA for cholangiocytes, can further assist in diagnosis [44]. Despite its utility, some PEComas may evade detection even after pathological examination [45], emphasizing the need for increased awareness among clinicians and pathologists regarding these tumors.
Surgery is the preferred treatment for PEComas. Complete tumor resection with clear margins can be curative, with low recurrence rates reported [46]. A conservative approach involving close observation with laboratory and imaging follow-up has also been employed in some cases of benign PEComas [47]. An initial non-surgical approach was taken in 30 patients in our study, of whom 13 (43.3%) eventually required surgery due to tumor progression. As most hepatic PEComas are benign, a non-surgical approach is an option for clinicians, especially in cases with small tumor size, lack of malignant characteristics, and contraindications to surgery. However, these patients must be closely monitored due to the possible enlargement and malignant transformation of PEComas [9]. In inoperable cases due to tumor size or advanced stage, neoadjuvant treatments should be considered. Stereotactic radiation therapy and chemotherapy have shown success in reducing tumor size and vascularity [48,49]. Liver transplantation is another option for selected patients, while TACE and mTOR inhibitors have also been used for tumor reduction prior to surgery [50]. mTOR inhibitors have been increasingly used for the treatment of PEComas in recent years, either as a first-line treatment in inoperable malignant cases or as adjuvant therapy after surgery [51]. The identification of increased mTOR pathway activity even in PEComa cases without TSC mutations has made this protein a reasonable therapeutic target. In a phase II trial of patients with malignant PEComas, the effect of the intravenous administration of nab-sirolimus, an mTOR inhibitor, was examined. Patients exhibited a rapid response to treatment with favorable disease control rates, while severe adverse events were not observed [52]. These agents have also had success as a neoadjuvant treatment in cases of hepatic PEComas, where their use contributed to tumor shrinkage and made surgical treatment of these patients possible [21,53]. Further studies are required to identify the optimal dose for liver PEComa treatment, with which patients can benefit the most from mTOR inhibition.
Most liver PEComas are benign, but malignant forms have been reported. Due to their malignant potential and the risks associated with tumor growth, close monitoring is recommended, as surgical intervention may ultimately be necessary [35]. Vascular invasion and complications such as Budd–Chiari syndrome and portal vein thrombosis are associated with poor prognosis [54]. Metastasis can occur in the liver or distant organs, even years after initial resection [55]. Additional treatments such as TACE, TAE, and targeted agents like sorafenib can be beneficial in these cases. mTOR inhibitors have shown promise in improving outcomes for patients with advanced or metastatic PEComas [56,57]. Early pathological recognition of malignant features can enhance patient management by identifying those requiring close surveillance, even after successful surgery. Folpe et al. proposed a classification system for PEComas based on the presence of two or more high-risk features observed during pathological analysis. These include tumor size larger than 5 cm, an infiltrative growth pattern, a high nuclear grade, high cellularity, necrosis, a mitotic rate higher than 1 mitosis/50 high-power fields, and vascular invasion. A large tumor or nuclear polymorphism alone categorizes the tumor as having uncertain malignant potential [58]. Many researchers have used these criteria for classification in recent years. More recently, Yoo et al. proposed specific risk factors for primary hepatic PEComas, which demonstrated good predictive value. Worrisome features included a tumor size ≥ 7 cm, infiltrative borders, a mitotic rate > 1/10 mm2, necrosis, vascular invasion, and a diagnosis of PEComa-NOS. Patients with three or more of these features were classified as high-risk, while those with none were considered low-risk [59]. Applying these criteria in future pathological assessments may help predict prognosis and guide treatment strategies.
Our study has certain limitations. Due to the large number of case reports and series, the data included were heterogeneous in terms of design, population, and interventions, precluding meta-analysis. Many articles were excluded because they were written in non-English languages or lacked follow-up data necessary to evaluate outcomes. Nonetheless, these excluded studies may have contained valuable information regarding the radiological and pathological characteristics of liver PEComas that were not utilized in this review. The significance of this systematic review lies in its comprehensive description of primary liver PEComa characteristics across a substantial number of adult patients from diverse geographic regions. The volume of cases included provides valuable insights that can assist clinical decision-making and support the development of formal guidelines for the evaluation and treatment of primary hepatic PEComas. This study also underscores the importance of physician awareness and highlights the value of a multidisciplinary approach involving various medical specialties to determine the most effective management strategies.

5. Conclusions

Primary hepatic PEComa is a rare mesenchymal liver tumor with mostly benign clinical behavior, but variable malignant potential. It presents more frequently in women and is a solid liver lesion without specific clinical or radiological characteristics in the majority of cases, leading to frequent misdiagnosis. Although many cases are associated with genetic disorders such as TSC and Li–Fraumeni syndrome, several cases are sporadic. Pathological analysis remains the gold standard for diagnosis, but the evolution of imaging studies and increased awareness of the disease by many medical specialties can contribute to earlier PEComa recognition and optimal treatment. The recognition of high-risk patients through the use of specific risk stratification criteria based on worrisome features can identify patients in need of close follow-up and guide clinical decision-making. Such criteria are not currently established, and future controlled studies are required for their development.
Although surgical resection remains the cornerstone of management, prospective studies examining optimal patient treatment and surveillance strategies are currently lacking. Moreover, the expanding use of mTOR inhibitors and other possible targeted therapies offers additional options for the treatment of these patients, especially in malignant or inoperable cases. Many liver PEComas exhibit different biological behavior, especially in cases of mutations such as TFE3 rearrangement. Future studies are needed to determine optimal treatment choices and surveillance intervals based on specific tumor and patient characteristics. The establishment of formal evidence-based guidelines regarding the treatment of liver PEComas can support clinician decisions regarding this entity and further improve patient care.

Supplementary Materials

The following supporting information can be downloaded at https://www.mdpi.com/article/10.3390/cancers17142276/s1: Table S1: Characteristics of patients included in the analysis. Data from references [12,13,14,15,16,19,28,29,33,38,45,47,49,50,54,55,60,61,62,63,64,65,66,67,68,69,70,71,72,73,74,75,76,77,78,79,80,81,82,83,84,85,86,87,88,89,90,91,92,93,94,95,96,97,98,99,100,101,102,103,104,105,106,107,108,109,110,111,112,113,114,115,116,117,118,119,120,121,122,123,124,125,126,127,128,129,130,131,132,133,134,135,136,137,138,139,140,141,142,143,144,145,146,147,148,149,150,151,152,153,154,155,156,157,158,159,160,161,162,163,164,165,166,167,168,169,170,171,172,173,174,175,176,177,178,179,180,181,182,183,184,185,186,187,188,189] are included in the Supplementary Materials.

Author Contributions

Conceptualization, M.K. and C.T.; methodology, K.P., M.K. and I.A.; software, I.A. and M.K.; validation, K.P. and I.A.; formal analysis, K.P. and M.K.; investigation, K.P. and I.A.; data curation, K.P. and I.A.; writing—original draft preparation, K.P. and I.A.; writing—review and editing, M.K.; visualization, C.T.; supervision, C.T.; project administration, C.T. All authors have read and agreed to the published version of the manuscript.

Funding

This research received no external funding.

Conflicts of Interest

The authors declare no conflicts of interest.

References

  1. Bonetti, F.; Pea, M.; Martignoni, G.; Zamboni, G. PEC and sugar. Am. J. Surg. Pathol. 1992, 16, 307–308. [Google Scholar] [CrossRef]
  2. Dong, B.N.; Zhan, H.; Luan, T.; Wang, J.S. Comprehensive Insights Into Renal Perivascular Epithelioid Cell Neoplasms: From Molecular Mechanisms to Clinical Practice. World J. Oncol. 2024, 15, 372–381. [Google Scholar] [CrossRef] [PubMed]
  3. Fadare, O.; Parkash, V.; Yilmaz, Y.; Mariappan, M.R.; Ma, L.; Hileeto, D.; Qumsiyeh, M.B.; Hui, P. Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal “PEComatosis”: A clinicopathological study with comparative genomic hybridization analysis. World J. Surg. Oncol. 2004, 2, 35. [Google Scholar] [CrossRef] [PubMed]
  4. Zhao, J.; Teng, H.; Zhao, R.; Ding, W.; Yu, K.; Zhu, L.; Zhang, J.; Han, Y. Malignant perivascular epithelioid cell tumor of the lung synchronous with a primary adenocarcinoma: One case report and review of the literature. BMC Cancer 2019, 19, 235. [Google Scholar] [CrossRef]
  5. Caliskan, S.; Akar, O.S.; Gun, S.; Kefeli, M. Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Uterus as Part of the Hereditary Cancer Syndrome: A Case Diagnosed with Multiple Malignancies. Turk. Patoloji. Derg. 2023, 39, 212–217. [Google Scholar] [CrossRef]
  6. Haiges, D.; Kurz, P.; Laaff, H.; Meiss, F.; Kutzner, H.; Technau-Hafsi, K. Malignant PEComa. J. Cutan. Pathol. 2018, 45, 84–89. [Google Scholar] [CrossRef]
  7. Dashraath, P.; Sidek, N.A.; Kalaichelvan, V.; Makmur, A.; Lim, D.G.S.; Low, J.J.H.; Ng, J.S.Y. Malignant perivascular epithelioid cell tumor (PEComa) of uterus. Ultrasound Obstet. Gynecol. 2022, 59, 826–828. [Google Scholar] [CrossRef]
  8. Algashaamy, K.; Montgomery, E.A.; Garcia-Buitrago, M. Liver mesenchymal neoplasms: Something old, something new. Pathology 2022, 54, 225–235. [Google Scholar] [CrossRef]
  9. Yan, S.; Lu, J.J.; Chen, L.; Cai, W.H.; Wu, J.Z. Hepatic perivascular epithelioid cell tumors: The importance of preoperative diagnosis. World J. Gastroenterol. 2024, 30, 1926–1933. [Google Scholar] [CrossRef]
  10. Khan, H.M.; Katz, S.C.; Libbey, N.P.; Somasundar, P.S. Hepatic PEComa: A potential pitfall in the evaluation of hepatic neoplasms. BMJ Case Rep. 2014, 2014, bcr2014204122. [Google Scholar] [CrossRef]
  11. Page, M.J.; McKenzie, J.E.; Bossuyt, P.M.; Boutron, I.; Hoffmann, T.C.; Mulrow, C.D.; Shamseer, L.; Tetzlaff, J.M.; Akl, E.A.; Brennan, S.E.; et al. The PRISMA 2020 statement: An updated guideline for reporting systematic reviews. BMJ 2021, 372, n71. [Google Scholar] [CrossRef] [PubMed]
  12. Jung, D.H.; Hwang, S.; Hong, S.M.; Kim, K.H.; Ahn, C.S.; Moon, D.B.; Alshahrani, A.A.; Lee, S.G. Clinico-pathological correlation of hepatic angiomyolipoma: A series of 23 resection cases. ANZ J. Surg. 2018, 88, E60–E65. [Google Scholar] [CrossRef] [PubMed]
  13. Jiang, T.; Hu, Y.; Wang, J.; Li, W. Fat-Poor Hepatic Angiomyolipoma in Noncirrhotic Livers: Imaging Features, Pathology, and Differential Diagnosis. J. Comput. Assist. Tomogr. 2024, 48, 727–733. [Google Scholar] [CrossRef] [PubMed]
  14. Zhou, Y.; Chen, F.; Jiang, W.; Meng, Q.; Wang, F. Hepatic epithelioid angiomyolipoma with an unusual pathologic appearance: Expanding the morphologic spectrum. Int. J. Clin. Exp. Pathol. 2014, 7, 6364–6369. [Google Scholar]
  15. Son, H.J.; Kang, D.W.; Kim, J.H.; Han, H.Y.; Lee, M.K. Hepatic perivascular epithelioid cell tumor (PEComa): A case report with a review of literatures. Clin. Mol. Hepatol. 2017, 23, 80–86. [Google Scholar] [CrossRef]
  16. Kvietkauskas, M.; Samuolyte, A.; Rackauskas, R.; Luksaite-Lukste, R.; Karaliute, G.; Maskoliunaite, V.; Valkiuniene, R.B.; Sokolovas, V.; Strupas, K. Primary Liver Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review. Medicina 2024, 60, 409. [Google Scholar] [CrossRef]
  17. Katsakhyan, L.; Shahi, M.; Eugene, H.C.; Nonogaki, H.; Gross, J.M.; Nucci, M.R.; Vang, R.; Xing, D. Uterine Leiomyosarcoma Associated With Perivascular Epithelioid Cell Tumor: A Phenomenon of Differentiation/Dedifferentiation and Evidence Suggesting Cell-of-Origin. Am. J. Surg. Pathol. 2024, 48, 761–772. [Google Scholar] [CrossRef]
  18. Nie, P.; Wu, J.; Wang, H.; Zhou, R.; Sun, L.; Chen, J.; Yang, G. Primary hepatic perivascular epithelioid cell tumors: Imaging findings with histopathological correlation. Cancer Imaging 2019, 19, 32. [Google Scholar] [CrossRef]
  19. Dymkowski, M.; Kalman, P.; Niecikowski, P.; Koperski, Ł.; Kosieradzki, M. Case report: Liver PEComa after kidney transplantation in recipient with tuberous sclerosis complex. Front. Oncol. 2024, 14, 1386569. [Google Scholar] [CrossRef]
  20. Henske, E.P.; Jóźwiak, S.; Kingswood, J.C.; Sampson, J.R.; Thiele, E.A. Tuberous sclerosis complex. Nat. Rev. Dis. Primers 2016, 2, 16035. [Google Scholar] [CrossRef]
  21. Dickson, M.A.; Schwartz, G.K.; Antonescu, C.R.; Kwiatkowski, D.J.; Malinowska, I.A. Extrarenal perivascular epithelioid cell tumors (PEComas) respond to mTOR inhibition: Clinical and molecular correlates. Int. J. Cancer 2013, 132, 1711–1717. [Google Scholar] [CrossRef] [PubMed]
  22. Izubuchi, Y.; Tanaka, T. PEComa—Its clinical features, histopathology, and current therapy. Jpn. J. Clin. Oncol. 2025, 55, 691–698. [Google Scholar] [CrossRef] [PubMed]
  23. Argani, P.; Aulmann, S.; Illei, P.B.; Netto, G.J.; Ro, J.; Cho, H.Y.; Dogan, S.; Ladanyi, M.; Martignoni, G.; Goldblum, J.R.; et al. A distinctive subset of PEComas harbors TFE3 gene fusions. Am. J. Surg. Pathol. 2010, 34, 1395–1406. [Google Scholar] [CrossRef] [PubMed]
  24. Lin, R.J.; Melamed, J.; Wu, J. PEComa with Transcription Factor E3 Overexpression: A Diagnostic and Therapeutic Challenge. Case Rep. Oncol. 2017, 10, 531–533. [Google Scholar] [CrossRef]
  25. Aedma, S.K.; Kasi, A. Li-Fraumeni Syndrome. In StatPearls; StatPearls Publishing: Treasure Island, FL, USA, 2025. [Google Scholar]
  26. Butz, H.; Lövey, J.; Szentkereszty, M.; Bozsik, A.; Tóth, E.; Patócs, A. Case Report: A Novel Pathomechanism in PEComa by the Loss of Heterozygosity of TP53. Front. Oncol. 2022, 12, 849004. [Google Scholar] [CrossRef]
  27. Jasim, S.; Tamboli, P.; Lee, S.-C.; Strong, L.C.; Elsayes, K.; Ayala-Ramirez, M.; Habra, M.A. Epithelioid Angiomyolipoma in a Patient With Li-Fraumeni Syndrome: Rare Pathologic Diagnosis. AACE Clin. Case Rep. 2016, 2, e251–e255. [Google Scholar] [CrossRef]
  28. Song, Z.; Xu, F.; Dai, C. Chills and fever as the first presentation of hepatic perivascular epithelioid cell tumor. Hepatobiliary Surg. Nutr. 2019, 8, 436–438. [Google Scholar] [CrossRef]
  29. Kou, Y.Q.; Yang, Y.P.; Ye, W.X.; Yuan, W.N.; Du, S.S.; Nie, B. Perivascular epithelioid cell tumors of the liver misdiagnosed as hepatocellular carcinoma: Three case reports. World J. Clin. Cases 2023, 11, 426–433. [Google Scholar] [CrossRef]
  30. Senne, M.; Sgourakis, G.; Molmenti, E.P.; Schroeder, T.; Beckebaum, S.; Nadalin, S.; Malagó, M.; Radtke, A. Portal and Hepatic Venous Territorial Mapping in Healthy Human Livers: Virtual Three-Dimensional Computed Tomography Size-Shape-Topography Study. Exp. Clin. Transpl. 2022, 20, 826–834. [Google Scholar] [CrossRef]
  31. Klompenhouwer, A.J.; Verver, D.; Janki, S.; Bramer, W.M.; Doukas, M.; Dwarkasing, R.S.; de Man, R.A.; IJzermans, J.N.M. Management of hepatic angiomyolipoma: A systematic review. Liver Int. 2017, 37, 1272–1280. [Google Scholar] [CrossRef]
  32. Nese, N.; Martignoni, G.; Fletcher, C.D.; Gupta, R.; Pan, C.C.; Kim, H.; Ro, J.Y.; Hwang, I.S.; Sato, K.; Bonetti, F.; et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: Detailed assessment of morphology and risk stratification. Am. J. Surg. Pathol. 2011, 35, 161–176. [Google Scholar] [CrossRef] [PubMed]
  33. Yang, W.; Sun, Q.; Shang, M.; Li, S.; Hu, X.; Hu, X. Multimodal imaging study of hepatic perivascular epithelioid cell tumors: A case report. Front. Med. 2023, 10, 1322048. [Google Scholar] [CrossRef]
  34. Ji, M.; Zhang, Y.; Liu, S.; Zhang, M.; Qiao, B. Hepatic perivascular epithelioid cell tumor: A retrospective analysis of 36 cases. Front. Oncol. 2024, 14, 1416254. [Google Scholar] [CrossRef]
  35. Yang, X.; Wang, Q.; Zhou, X.; Zhou, H.; Jia, W.; Hu, C.; Chu, J.; Kong, L. Retrospective analysis of hepatic perivascular epithelioid cell tumour (PEComa) in a single centre for clinical diagnosis and treatment clinical diagnosis and treatment of hepatic PEComa. Medicine 2022, 101, e29506. [Google Scholar]
  36. Kacała, A.; Dorochowicz, M.; Matus, I.; Puła, M.; Korbecki, A.; Sobański, M.; Jacków-Nowicka, J.; Patrzałek, D.; Janczak, D.; Guziński, M. Hepatic Hemangioma: Review of Imaging and Therapeutic Strategies. Medicina 2024, 60, 449. [Google Scholar] [CrossRef]
  37. LeGout, J.D.; Bolan, C.W.; Bowman, A.W.; Caserta, M.P.; Chen, F.K.; Cox, K.L.; Sanyal, R.; Toskich, B.B.; Lewis, J.T.; Alexander, L.F. Focal Nodular Hyperplasia and Focal Nodular Hyperplasia-like Lesions. Radiographics 2022, 42, 1043–1061. [Google Scholar] [CrossRef]
  38. Guo, Y.L.; Dang, Y.; Zhang, H.; Lian, J. Contrast-Enhanced Ultrasound of a Hepatic Perivascular Epithelioid Cell Tumor: A Case Report and Literature Review. Cureus 2025, 17, e81579. [Google Scholar] [CrossRef]
  39. Tan, Y.; Zhang, H.; Xiao, E.H. Perivascular epithelioid cell tumour: Dynamic CT, MRI and clinicopathological characteristics--analysis of 32 cases and review of the literature. Clin. Radiol. 2013, 68, 555–561. [Google Scholar] [CrossRef]
  40. Ma, R.; Feng, S.-T.; Meicheng, C.; Wang, J.; Dong, Z.; Zhou, X. Hepatic Pecoma versus Hepatocellular Carcinoma In The Noncirrhotic Liver on Gd-EOB-DTPA-Enhanced MRI: A Diagnostic Challenge. Curr. Med. Imaging 2024, 20, e15734056269369. [Google Scholar] [CrossRef]
  41. Sun, L.; Sun, X.; Li, Y.; Xing, L. The role of (18)F-FDG PET/CT imaging in patient with malignant PEComa treated with mTOR inhibitor. Onco Targets Ther. 2015, 8, 1967–1970. [Google Scholar] [CrossRef]
  42. Streba, C.T.; Streba, L.A.M.; Dumitrescu, D.; Georgescu, E.F. Risks and Benefits of Liver Biopsy in Focal Liver Disease. In Liver Biopsy—Indications, Procedures, Results; Tagaya, N., Ed.; IntechOpen: Rijeka, Croatia, 2012. [Google Scholar]
  43. Bao, L.; Shi, Y.; Zhong, J.; Zhao, M.; Wu, J.; Hai, L.; Xu, X.; Du, H.; Shi, Y. Histopathologic characteristics and immunotypes of perivascular epithelioid cell tumors (PEComa). Int. J. Clin. Exp. Pathol. 2019, 12, 4380–4389. [Google Scholar]
  44. Kumagai, A.; Kondo, F.; Sano, K.; Inoue, M.; Fujii, T.; Hashimoto, M.; Watanabe, M.; Soejima, Y.; Ishida, T.; Tokairin, T.; et al. Immunohistochemical study of hepatocyte, cholangiocyte and stem cell markers of hepatocellular carcinoma: The second report: Relationship with tumor size and cell differentiation. J. Hepatobiliary Pancreat. Sci. 2016, 23, 414–421. [Google Scholar] [CrossRef] [PubMed]
  45. Britt, A.; Mohyuddin, G.R.; Al-Rajabi, R. Maintenance of Stable Disease in Metastatic Perivascular Epithelioid Cell Tumor of the Liver With Single-Agent Sorafenib. Am. J. Ther. 2020, 29, e259–e261. [Google Scholar] [CrossRef] [PubMed]
  46. Wang, Z.S.; Xu, L.; Ma, L.; Song, M.Q.; Wu, L.Q.; Zhou, X. Hepatic falciform ligament clear cell myomelanocytic tumor: A case report and a comprehensive review of the literature on perivascular epithelioid cell tumors. BMC Cancer 2015, 15, 1004. [Google Scholar] [CrossRef]
  47. Chao, C.H.; Lin, C.Y.; Chan, S.C.; Chen, K.S. Concurrent hepatic and ruptured renal angiomyolipoma in tuberous sclerosis complex. Chang. Gung. Med. J. 2004, 27, 696–700. [Google Scholar]
  48. Liu, J.L.; Lin, Y.M.; Lin, M.C.; Yeh, K.T.; Hsu, J.C.; Chin, C.J. Perivascular epithelioid cell tumor (PEComa) of the uterus with aggressive behavior at presentation. Hematol. Oncol. Stem Cell Ther. 2009, 2, 426–430. [Google Scholar] [CrossRef]
  49. Kirste, S.; Kayser, G.; Zipfel, A.; Grosu, A.L.; Brunner, T. Unresectable hepatic PEComa: A rare malignancy treated with stereotactic body radiation therapy (SBRT) followed by complete resection. Radiat. Oncol. 2018, 13, 28. [Google Scholar] [CrossRef]
  50. Wang, W.T.; Li, Z.Q.; Zhang, G.H.; Guo, Y.; Teng, M.J. Liver transplantation for recurrent posthepatectomy malignant hepatic angiomyolipoma: A case report. World J. Gastroenterol. 2015, 21, 3755–3758. [Google Scholar] [CrossRef]
  51. Liu, C.H.; Chao, W.T.; Lin, S.C.; Lau, H.Y.; Wu, H.H.; Wang, P.H. Malignant perivascular epithelioid cell tumor in the female genital tract: Preferred reporting items for systematic reviews and meta-analyses. Medicine 2019, 98, e14072. [Google Scholar] [CrossRef]
  52. Wagner, A.J.; Ravi, V.; Riedel, R.F.; Ganjoo, K.; Van Tine, B.A.; Chugh, R.; Cranmer, L.; Gordon, E.M.; Hornick, J.L.; Du, H.; et al. nab-Sirolimus for Patients With Malignant Perivascular Epithelioid Cell Tumors. J. Clin. Oncol. 2021, 39, 3660–3670. [Google Scholar] [CrossRef]
  53. Bergamo, F.; Maruzzo, M.; Basso, U.; Montesco, M.C.; Zagonel, V.; Gringeri, E.; Cillo, U. Neoadjuvant sirolimus for a large hepatic perivascular epithelioid cell tumor (PEComa). World J. Surg. Oncol. 2014, 12, 46. [Google Scholar] [CrossRef] [PubMed]
  54. Abhirup, B.; Kaushal, K.; Sanket, M.; Ganesh, N. Malignant hepatic perivascular epithelioid cell tumor (PEComa)—Case report and a brief review. J. Egypt. Natl. Cancer Inst. 2015, 27, 239–242. [Google Scholar] [CrossRef]
  55. Fukuda, Y.; Omiya, H.; Takami, K.; Mori, K.; Kodama, Y.; Mano, M.; Nomura, Y.; Akiba, J.; Yano, H.; Nakashima, O.; et al. Malignant hepatic epithelioid angiomyolipoma with recurrence in the lung 7 years after hepatectomy: A case report and literature review. Surg. Case Rep. 2016, 2, 31. [Google Scholar] [CrossRef] [PubMed]
  56. Gennatas, C.; Michalaki, V.; Kairi, P.V.; Kondi-Paphiti, A.; Voros, D. Successful treatment with the mTOR inhibitor everolimus in a patient with perivascular epithelioid cell tumor. World J. Surg. Oncol. 2012, 10, 181. [Google Scholar] [CrossRef]
  57. Świtaj, T.; Sobiborowicz, A.; Teterycz, P.; Klimczak, A.; Makuła, D.; Wągrodzki, M.; Szumera-Ciećkiewicz, A.; Rutkowski, P.; Czarnecka, A.M. Efficacy of Sirolimus Treatment in PEComa–10 Years of Practice Perspective. J. Clin. Med. 2021, 10, 3705. [Google Scholar]
  58. Folpe, A.L.; Mentzel, T.; Lehr, H.A.; Fisher, C.; Balzer, B.L.; Weiss, S.W. Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: A clinicopathologic study of 26 cases and review of the literature. Am. J. Surg. Pathol. 2005, 29, 1558–1575. [Google Scholar] [CrossRef]
  59. Yoo, Y.; Kim, J.; Song, I.H. Risk prediction criteria for the primary hepatic perivascular epithelioid cell tumour family, including angiomyolipoma: Analysis of 132 cases with a literature review. Histopathology 2025, 86, 979–992. [Google Scholar] [CrossRef]
  60. Kudo, M.; Okuno, T.; Tomita, S.; Kajiwara, T.; Shirane, H.; Usuki, N.; Todo, A. Hepatic angiomyolipoma pre-operatively diagnosed by imaging. J. Gastroenterol. Hepatol. 1993, 8, 483–488. [Google Scholar] [CrossRef]
  61. Yamada, N.; Shinzawa, H.; Makino, N.; Matsuhashi, T.; Itasaka, S.; Takahashi, T.; Fuyama, S. Small angiomyolipoma of the liver diagnosed by fine-needle aspiration biopsy under ultrasound guidance. J. Gastroenterol. Hepatol. 1993, 8, 495–498. [Google Scholar] [CrossRef]
  62. Carmody, E.; Yeung, E.; McLoughlin, M. Angiomyolipomas of the liver in tuberous sclerosis. Abdom. Imaging 1994, 19, 537–539. [Google Scholar] [CrossRef]
  63. Kimura, N.; Kubota, M.; Nagura, H. A hepatic tumor associated with bilateral renal angiomyolipomas: A variant of angiomyolipoma? Pathol. Int. 1994, 44, 540–547. [Google Scholar] [CrossRef] [PubMed]
  64. Peh, W.C.; Ngan, H.; Fan, S.T.; Ng, I.O. Case report: Variable imaging appearances of angiomyolipomas of the liver. Br. J. Radiol. 1995, 68, 540–544. [Google Scholar] [CrossRef] [PubMed]
  65. Hoffman, A.L.; Emre, S.; Verham, R.P.; Petrovic, L.M.; Eguchi, S.; Silverman, J.L.; Geller, S.A.; Schwartz, M.E.; Miller, C.M.; Makowka, L. Hepatic angiomyolipoma: Two case reports of caudate-based lesions and review of the literature. Liver Transplant. Surg. 1997, 3, 46–53. [Google Scholar] [CrossRef]
  66. Sawai, H.; Manabe, T.; Yamanaka, Y.; Kurahashi, S.; Kamiya, A. Angiomyolipoma of the liver: Case report and collective review of cases diagnosed from fine needle aspiration biopsy specimens. J. Hepatobiliary Pancreat. Surg. 1998, 5, 333–338. [Google Scholar] [CrossRef]
  67. Röcken, C.; Schneider-Stock, R.; Buhtz, P.; Manger, T.; Roessner, A. Hepatic angiomyolipoma in a 26-year-old Caucasian woman with a history of tibial osteosarcoma. Pathol. Res. Pract. 1999, 195, 765–772. [Google Scholar] [CrossRef]
  68. Sajima, S.; Kinoshita, H.; Okuda, K.; Saito, N.; Hashino, K.; Sugimoto, R.; Eriguchi, N.; Aoyagi, S. Angiomyolipoma of the liver--a case report and review of 48 cases reported in Japan. Kurume Med. J. 1999, 46, 127–131. [Google Scholar] [CrossRef]
  69. Dalle, I.; Sciot, R.; de Vos, R.; Aerts, R.; van Damme, B.; Desmet, V.; Roskams, T. Malignant angiomyolipoma of the liver: A hitherto unreported variant. Histopathology 2000, 36, 443–450. [Google Scholar] [CrossRef]
  70. Yamasaki, S.; Tanaka, S.; Fujii, H.; Matsumoto, T.; Okuda, C.; Watanabe, G.; Suda, K. Monotypic epithelioid angiomyolipoma of the liver. Histopathology 2000, 36, 451–456. [Google Scholar] [CrossRef]
  71. Ji, Y.; Zhu, X.; Xu, J.; Zhou, J.; Tan, Y.; Wang, J.; Fan, J.; Zhou, Y. Hepatic angiomyolipoma: A clinicopathologic study of 10 cases. Chin. Med. J. 2001, 114, 280–285. [Google Scholar]
  72. Tang, L.H.; Hui, P.; Garcia-Tsao, G.; Salem, R.R.; Jain, D. Multiple angiomyolipomata of the liver: A case report. Mod. Pathol. 2002, 15, 167–171. [Google Scholar] [CrossRef]
  73. Rimola, J.; Martín, J.; Puig, J.; Darnell, A.; Gil, D. Hepatic angiomyolipoma: Progressive changes in size and tumor composition. Abdom. Imaging 2003, 28, 665–667. [Google Scholar] [CrossRef] [PubMed]
  74. Akatsu, T.; Sakamoto, M.; Shimazu, M.; Kitajima, M. Pedunculated angiomyolipoma of the liver with a predominant pelioid pattern. Virchows Arch. 2004, 444, 467–469. [Google Scholar] [CrossRef] [PubMed]
  75. De Bruecker, Y.; Ballaux, F.; Allewaert, S.; Vanbeckevoort, D.; Bielen, D.; Roskams, T.; Aerts, R.; Roex, L.; Simoens, M. A solitary hepatic lesion: MRI-pathological correlation of an hepatic angiomyolipoma (2004:4b). Eur. Radiol. 2004, 14, 1324–1326. [Google Scholar] [CrossRef] [PubMed]
  76. Kojima, M.; Nakamura, S.; Ohno, Y.; Sugihara, S.; Sakata, N.; Masawa, N. Hepatic angiomyolipoma resembling an inflammatory pseudotumor of the liver. A case report. Pathol. Res. Pract. 2004, 200, 713–716. [Google Scholar] [CrossRef]
  77. Lin, K.J.; Eng, H.L.; Lu, S.N.; Chiu, K.W.; Kuo, F.Y. Hepatic angiomyolipoma: Report of two cases with emphasis on smear cytomorphology and the use of cell block with immunohistochemical stains. Diagn. Cytopathol. 2004, 31, 263–266. [Google Scholar] [CrossRef]
  78. Romano, F.; Franciosi, C.; Bovo, G.; Cesana, G.C.; Isella, G.; Colombo, G.; Uggeri, F. Case report of a hepatic angiomyolipoma. Tumori J. 2004, 90, 139–143. [Google Scholar] [CrossRef]
  79. Saito, M.; Tsukamoto, T.; Takahashi, T.; Sai, K.; Fujii, H.; Nagashima, K. Multifocal angiomyolipoma affecting the liver and lung without tuberous sclerosis. J. Clin. Pathol. 2004, 57, 221–224. [Google Scholar] [CrossRef]
  80. Sebastian, S.; Tuite, D.; Torreggiani, W.; Crotty, P.; Buckley, M. Angiomyolipoma of the liver causing Budd-Chiari syndrome. J. Gastroenterol. Hepatol. 2004, 19, 722–723. [Google Scholar] [CrossRef]
  81. Tryggvason, G.; Blöndal, S.; Goldin, R.D.; Albrechtsen, J.; Björnsson, J.; Jónasson, J.G. Epithelioid angiomyolipoma of the liver: Case report and review of the literature. Apmis 2004, 112, 612–616. [Google Scholar] [CrossRef]
  82. Takamura, K.; Miyake, H.; Fujii, M.; Nishi, M.; Tashiro, S.; Shimada, M. Multiple hepatic angiomyolipomas with a solitary omental angiomyolipoma. J. Med. Investig. 2005, 52, 218–222. [Google Scholar] [CrossRef]
  83. Yen, Y.H.; Wang, J.H.; Lu, S.N.; Changchien, C.S. Contrast-enhanced ultrasonography in hepatic angiomyolipoma. J. Ultrasound Med. 2005, 24, 855–859. [Google Scholar] [CrossRef] [PubMed]
  84. Flor, N.; Sardanelli, F.; Serantoni, S.; Brovelli, F.; Cornalba, G.P. Low-fat angiomyolipoma of the liver studied with contrast-enhanced ultrasound and multidetector computed tomography. Acta Radiol. 2006, 47, 543–546. [Google Scholar] [CrossRef]
  85. Parfitt, J.R.; Bella, A.J.; Izawa, J.I.; Wehrli, B.M. Malignant neoplasm of perivascular epithelioid cells of the liver. Arch. Pathol. Lab. Med. 2006, 130, 1219–1222. [Google Scholar] [CrossRef] [PubMed]
  86. Wang, S.N.; Tsai, K.B.; Lee, K.T. Hepatic angiomyolipoma with trace amounts of fat: A case report and literature review. J. Clin. Pathol. 2006, 59, 1196–1199. [Google Scholar] [CrossRef] [PubMed]
  87. Fang, S.H.; Zhou, L.N.; Jin, M.; Hu, J.B. Perivascular epithelioid cell tumor of the liver: A report of two cases and review of the literature. World J. Gastroenterol. 2007, 13, 5537–5539. [Google Scholar] [CrossRef]
  88. Larbcharoensub, N.; Karnsombut, P.; Jatchavala, J.; Wasutit, Y.; Nitiyanant, P. Primary hepatic clear cell myomelanocytic tumor. Case report and review of the literature. Apmis 2007, 115, 1454–1459. [Google Scholar] [CrossRef]
  89. Yang, C.Y.; Ho, M.C.; Jeng, Y.M.; Hu, R.H.; Wu, Y.M.; Lee, P.H. Management of hepatic angiomyolipoma. J. Gastrointest. Surg. 2007, 11, 452–457. [Google Scholar] [CrossRef]
  90. Della Vigna, P.; Preda, L.; Monfardini, L.; Gorone, M.S.; Maffini, F.A.; Bellomi, M. Growing perivascular epithelioid cell tumor of the liver studied with contrast-enhanced ultrasonography and magnetic resonance imaging. J. Ultrasound Med. 2008, 27, 1781–1785. [Google Scholar] [CrossRef]
  91. Deng, Y.F.; Lin, Q.; Zhang, S.H.; Ling, Y.M.; He, J.K.; Chen, X.F. Malignant angiomyolipoma in the liver: A case report with pathological and molecular analysis. Pathol. Res. Pract. 2008, 204, 911–918. [Google Scholar] [CrossRef]
  92. Lenci, I.; Angelico, M.; Tisone, G.; Orlacchio, A.; Palmieri, G.; Pinci, M.; Bombardieri, R.; Curatolo, P. Massive hepatic angiomyolipoma in a young woman with tuberous sclerosis complex: Significant clinical improvement during tamoxifen treatment. J. Hepatol. 2008, 48, 1026–1029. [Google Scholar] [CrossRef]
  93. Nguyen, T.T.; Gorman, B.; Shields, D.; Goodman, Z. Malignant hepatic angiomyolipoma: Report of a case and review of literature. Am. J. Surg. Pathol. 2008, 32, 793–798. [Google Scholar] [CrossRef] [PubMed]
  94. Paiva, C.E.; Moraes Neto, F.A.; Agaimy, A.; Custodio Domingues, M.A.; Rogatto, S.R. Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor. World J. Gastroenterol. 2008, 14, 800–802. [Google Scholar] [CrossRef] [PubMed]
  95. Yang, B.; Chen, W.H.; Shi, P.Z.; Xiang, J.J.; Xu, R.J.; Liu, J.H. Coincidence of hepatocelluar carcinoma and hepatic angiomyolipomas in tuberous sclerosis complex: A case report. World J. Gastroenterol. 2008, 14, 812–814. [Google Scholar] [CrossRef] [PubMed]
  96. Zimmermann, A.; von der Brelie, C.; Berger, B.; Kappeler, A.; Candinas, D. Primary perivascular epithelioid cell tumor of the liver not related to hepatic ligaments: Hepatic PEComa as an emerging entity. Histol. Histopathol. 2008, 23, 1185–1193. [Google Scholar] [CrossRef]
  97. Akitake, R.; Kimura, H.; Sekoguchi, S.; Nakamura, H.; Seno, H.; Chiba, T.; Fujimoto, S. Perivascular epithelioid cell tumor (PEComa) of the liver diagnosed by contrast-enhanced ultrasonography. Intern. Med. 2009, 48, 2083–2086. [Google Scholar] [CrossRef]
  98. Chen, P.; Yuan, T.; Liu, H. Hepatic angiomyolipoma mimicking hepatic clear cell carcinoma. J. Int. Med. Res. 2009, 37, 257–263. [Google Scholar] [CrossRef]
  99. Priola, A.M.; Priola, S.M.; Cataldi, A.; Marci, V.; Fava, C. Acute abdomen as an unusual presentation of hepatic PEComa. A case report. Tumori 2009, 95, 123–128. [Google Scholar] [CrossRef]
  100. Strzelczyk, J.M.; Durczynski, A.; Szymanski, D.; Jablkowski, M.; Dworniak, D.; Sporny, S. Primary perivascular epithelioid cell tumor (PEComa) of the liver: Report of a case. Surg. Today 2009, 39, 916–921. [Google Scholar] [CrossRef]
  101. Wang, Y.J.; Wang, Y.C.; Chien, C.C.; Chen, C.J.; Yang, R.N.; Fang, C.L. Diagnosis of hepatic angiomyolipomata using CT: Report of three cases and review of the literature. Clin. Radiol. 2009, 64, 329–334. [Google Scholar] [CrossRef]
  102. Kamimura, K.; Oosaki, A.; Sugahara, S.; Mori, S.; Moroda, T.; Satoh, O.; Morita, T.; Kimura, K.; Kamura, T.; Nomoto, M.; et al. Malignant potential of hepatic angiomyolipoma: Case report and literature review. Clin. J. Gastroenterol. 2010, 3, 104–110. [Google Scholar] [CrossRef]
  103. Shi, H.; Cao, D.; Wei, L.; Sun, L.; Guo, A. Inflammatory angiomyolipomas of the liver: A clinicopathologic and immunohistochemical analysis of 5 cases. Ann. Diagn. Pathol. 2010, 14, 240–246. [Google Scholar] [CrossRef] [PubMed]
  104. Zeng, J.P.; Dong, J.H.; Zhang, W.Z.; Wang, J.; Pang, X.P. Hepatic angiomyolipoma: A clinical experience in diagnosis and treatment. Dig. Dis. Sci. 2010, 55, 3235–3240. [Google Scholar] [CrossRef] [PubMed]
  105. Ahn, J.H.; Hur, B. Primary Perivascular Epithelioid Cell Tumor (PEComa) of the Liver: A Case Report and Review of the Literature. J. Pathol. Transl. Med. 2011, 45, S93–S97. [Google Scholar] [CrossRef]
  106. Mima, K.; Beppu, T.; Chikamoto, A.; Ishiko, T.; Horino, K.; Hayashi, N.; Watanabe, M.; Takamori, H.; Okabe, K.; Yamanaka, T.; et al. Laparoscopy-assisted resection of an undiagnosed liver tumor and ascending colon cancer via mini median laparotomy: Report of a case. Surg. Today 2011, 41, 1633–1638. [Google Scholar] [CrossRef]
  107. Selvaggi, F.; Risio, D.; Claudi, R.; Cianci, R.; Angelucci, D.; Pulcini, D.; D’Aulerio, A.; Legnini, M.; Cotellese, R.; Innocenti, P. Malignant PEComa: A case report with emphasis on clinical and morphological criteria. BMC Surg. 2011, 11, 3. [Google Scholar] [CrossRef]
  108. Tani, A.; Yoshida, H.; Mamada, Y.; Taniai, N.; Mineta, S.; Yoshioka, M.; Kawano, Y.; Ueda, J.; Naito, Z.; Uchida, E. Hepatic angiomyolipoma with a giant hemangioma. J. Nippon. Med. Sch. 2011, 78, 317–321. [Google Scholar] [CrossRef]
  109. Vagefi, P.A.; Eilers, H.; Hiniker, A.; Freise, C.E. Liver transplantation for giant hepatic angiomyolipoma. Liver Transplant. 2011, 17, 985–986. [Google Scholar] [CrossRef]
  110. Agaimy, A.; Vassos, N.; Croner, R.S.; Strobel, D.; Lell, M. Hepatic angiomyolipoma: A series of six cases with emphasis on pathological-radiological correlations and unusual variants diagnosed by core needle biopsy. Int. J. Clin. Exp. Pathol. 2012, 5, 512–521. [Google Scholar]
  111. Costa, S.; Tente, D.; Costa, A.; Maciel, J. Sporadic exophytic hepatic angiomyolipoma. BMJ Case Rep. 2012, 2012, 007224. [Google Scholar] [CrossRef]
  112. Durczyński, A.; Hogendorf, P.; Szymański, D.; Sporny, S.; Strzelczyk, J. Synchronous occurrence of multiple focal nodular hyperplasia and huge hepatic perivascular epithelioid cells tumor (PEComa) in young woman after oral contraceptive use--is there a common pathogenesis? Pol. Przegl. Chir. 2012, 84, 457–460. [Google Scholar]
  113. Liu, Y.; Wang, J.; Lin, X.Y.; Xu, H.T.; Qiu, X.S.; Wang, E.H. Inflammatory angiomyolipoma of the liver: A rare hepatic tumor. Diagn. Pathol. 2012, 7, 122. [Google Scholar] [CrossRef] [PubMed]
  114. Tan, Y.; Xiao, E.H. Hepatic perivascular epithelioid cell tumor (PEComa): Dynamic CT, MRI, ultrasonography, and pathologic features--analysis of 7 cases and review of the literature. Abdom. Imaging 2012, 37, 781–787. [Google Scholar] [CrossRef]
  115. Agaimy, A.; Märkl, B. Inflammatory angiomyolipoma of the liver: An unusual case suggesting relationship to IgG4-related pseudotumor. Int. J. Clin. Exp. Pathol. 2013, 6, 771–779. [Google Scholar]
  116. Cheung, T.T.; Trendell-Smith, N.; Poon, R.T. Primary perivascular epithelioid cell tumour (PEComa) of the liver. BMJ Case Rep. 2013, 2013, 008706. [Google Scholar] [CrossRef]
  117. Jafari, A.; Fischer, H.P.; von Websky, M.; Hong, G.S.; Kalff, J.C.; Manekeller, S. Primary perivascular epitheloid cell tumour (PEComa) of the liver: Case report and review of the literature. Z. Gastroenterol. 2013, 51, 1096–1100. [Google Scholar] [CrossRef] [PubMed]
  118. Patra, S.; Vij, M.; Kota, V.; Kancherla, R.; Rela, M. Pigmented perivascular epithelioid cell tumor of the liver: Report of a rare case with brief review of literature. J. Cancer Res. Ther. 2013, 9, 305–307. [Google Scholar] [CrossRef] [PubMed]
  119. Shen, H.Q.; Chen, D.F.; Sun, X.H.; Li, X.; Xu, J.; Hu, X.B.; Li, M.Q.; Wu, T.; Zhang, R.Y.; Li, K.Z. MRI diagnosis of perivascular epithelioid cell tumor (PEComa) of the liver. Rom. J. Morphol. Embryol. 2013, 54, 643–647. [Google Scholar]
  120. Shi, H.; Bai, Y.; Guo, A. Four cases of primary malignant perivascular epithelioid cell tumour of the liver. Pathology 2013, 45, 614–616. [Google Scholar] [CrossRef]
  121. Tay Sh, Y.; Lao, W.T.; Chen Ch, L.; Chan, W.P. Contrast-enhanced ct and angiographic findings in hepatic perivascular epithelioid cell tumor. J. Belg. Soc. Radiol. 2013, 96, 308–310. [Google Scholar] [CrossRef]
  122. Yang, L.; Xu, Z.; Dong, R.; Fan, J.; Du, Y.; Zhang, Y.; Wang, X.; Cheng, X.; Guo, J. Is surgery necessary for patients with hepatic angiomyolipoma? Retrospective analysis from eight Chinese cases. J. Gastroenterol. Hepatol. 2013, 28, 1648–1653. [Google Scholar] [CrossRef]
  123. Yu, D.; Tang, S. Hepatic perivascular epithelioid cell tumor: A case report and review of the literature. Intern. Med. 2013, 52, 1333–1336. [Google Scholar] [CrossRef] [PubMed]
  124. Zhao, L.J.; Yang, Y.J.; Wu, H.; Huang, S.M.; Liu, K. Perivascular epithelioid cell tumor of the liver: A case report and literature review. Eur. Rev. Med. Pharmacol. Sci. 2013, 17, 1665–1668. [Google Scholar] [PubMed]
  125. Fang, S.; Zhou, L.; Jin, M.; Hu, J. Perivascular epithelioid cell tumour of the liver. Liver Int. 2007, 27, 1293–1294. [Google Scholar] [CrossRef] [PubMed]
  126. Ameurtesse, H.; Chbani, L.; Bennani, A.; Toughrai, I.; Beggui, N.; Kamaoui, I.; Elfatemi, H.; Harmouch, T.; Amarti, A. Primary perivascular epithelioid cell tumor of the liver: New case report and literature review. Diagn. Pathol. 2014, 9, 149. [Google Scholar] [CrossRef]
  127. Barbier, L.; Torrents, J.; Hardwigsen, J. Hepatic angiomyolipoma: What management? Acta Chir. Belg. 2014, 114, 139–142. [Google Scholar]
  128. Kechaou, I.; Cherif, E.; Ben Hassine, L.; Khalfallah, N. Liver involvement in tuberous sclerosis. BMJ Case Rep. 2014, 2014, 201650. [Google Scholar] [CrossRef]
  129. Kumasaka, S.; Arisaka, Y.; Tokue, A.; Higuchi, T.; Nakajima, T.; Tsushima, Y. A case of multiple hepatic angiomyolipomas with high (18) F-fluorodeoxyglucose uptake. BMC Med. Imaging 2014, 14, 17. [Google Scholar] [CrossRef]
  130. Liu, D.; Shi, D.; Xu, Y.; Cao, L. Management of perivascular epithelioid cell tumor of the liver: A case report and review of the literature. Oncol. Lett. 2014, 7, 148–152. [Google Scholar] [CrossRef]
  131. Solarana Ortíz, J.A.; Placencia Gilart, J.E.; Rodríguez Diéguez, M.; Miranda Moles, Z.; Pullés Labadié, M.; Lau Cuza, J.C.; Corpas Fuster, S. Primary tumour of the round ligament of the liver: A case presentation. Pathologica 2014, 106, 26–28. [Google Scholar]
  132. Sun, K.; Zhao, M.; Yao, H.; Wang, L.; Wei, J. Premelanosome-negative inflammatory angiomyolipoma of liver with expression of cathepsin K and TFE3. Int. J. Clin. Exp. Pathol. 2014, 7, 8170–8175. [Google Scholar]
  133. Tan, Y.; Zhang, H.; Wang, X.C. Clear cell myomelanocytic tumor of the falciform ligament/ligamentum teres. Indian J. Pathol. Microbiol. 2014, 57, 453–455. [Google Scholar] [CrossRef] [PubMed]
  134. Wang, C.P.; Li, H.Y.; Wang, H.; Guo, X.D.; Liu, C.C.; Liu, S.H.; Gao, X.D.; Qu, J.H.; Liu, Z.; Chang, X.J.; et al. Hepatic angiomyolipoma mimicking hepatocellular carcinoma: Magnetic resonance imaging and clinical pathological characteristics in 9 cases. Medicine 2014, 93, e194. [Google Scholar] [CrossRef] [PubMed]
  135. Yamaguchi, M.; Ueda, Y.; Suzuki, T. Hepatic angiomyolipoma growing to cause epigastric discomfort: A case report. Clin. J. Gastroenterol. 2014, 7, 365–369. [Google Scholar] [CrossRef] [PubMed]
  136. Ge, X.W.; Zeng, H.Y.; Su-Jie, A.; Du, M.; Ji, Y.; Tan, Y.S.; Hou, Y.Y.; Xu, J.F. Hepatocellular carcinoma with concomitant hepatic angiomyolipoma and cavernous hemangioma in one patient. World J. Gastroenterol. 2015, 21, 3414–3419. [Google Scholar] [CrossRef]
  137. Maebayashi, T.; Abe, K.; Aizawa, T.; Sakaguchi, M.; Ishibashi, N.; Abe, O.; Takayama, T.; Nakayama, H.; Matsuoka, S.; Nirei, K.; et al. Improving recognition of hepatic perivascular epithelioid cell tumor: Case report and literature review. World J. Gastroenterol. 2015, 21, 5432–5441. [Google Scholar] [CrossRef]
  138. Neofytou, K.; Famularo, S.; Khan, A.Z. PEComa in a Young Patient with Known Li-Fraumeni Syndrome. Case Rep. Med. 2015, 2015, 906981. [Google Scholar] [CrossRef]
  139. Zhou, G.; Hu, W.; Bao, H.; Zhang, Q. A rare case of xanthogranulomatous pyelonepheritis with hepatic angiomyolipoma. Int. J. Clin. Exp. Pathol. 2015, 8, 11819–11822. [Google Scholar]
  140. Hao, B.B.; Rao, J.H.; Fan, Y.; Zhang, C.Y.; Dai, X.Z.; Li, X.; Leng, Y.; Zhang, F. Hepatic perivascular epithelioid cell tumor in three patients. Hepatobiliary Pancreat. Dis. Int. 2016, 15, 660–664. [Google Scholar] [CrossRef]
  141. Kiriyama, Y.; Tsukamoto, T.; Mizoguchi, Y.; Ishihara, S.; Horiguchi, A.; Tokoro, T.; Kato, Y.; Sugioka, A.; Kuroda, M. Intrahepatic peribiliary perivascular epithelioid cell tumor (PEComa) associated with heterotopic pancreas: A case report. Diagn. Pathol. 2016, 11, 81. [Google Scholar] [CrossRef]
  142. Lan, Y.Z.; Hua, X.E. Hepatic multiple perivascular epithelioid cell neoplasm: A case report and literature review. Mol. Clin. Oncol. 2016, 4, 619–621. [Google Scholar] [CrossRef]
  143. Tang, D.; Wang, J.; Tian, Y.; Li, Q.; Yan, H.; Wang, B.; Xiong, L.; Li, Q. Hepatic perivascular epithelioid cell tumor: Case report and brief literature review. Medicine 2016, 95, e5572. [Google Scholar] [CrossRef] [PubMed]
  144. Cardoso, H.; Silva, M.; Vilas-Boas, F.; Cunha, R.; Lopes, J.; Maia, J.C.; Macedo, G. Hepatic perivascular epithelioid tumor (PEComa). A case report. Clin. Res. Hepatol. Gastroenterol. 2017, 41, e43–e46. [Google Scholar] [CrossRef]
  145. Damaskos, C.; Garmpis, N.; Garmpi, A.; Nonni, A.; Sakellariou, S.; Margonis, G.A.; Spartalis, E.; Schizas, D.; Andreatos, N.; Magkouti, E.; et al. Angiomyolipoma of the Liver: A Rare Benign Tumor Treated with a Laparoscopic Approach for the First Time. In Vivo 2017, 31, 1169–1173. [Google Scholar] [CrossRef] [PubMed]
  146. Guan, H.; Zou, Y.; Lv, Y.; Wang, C. Hepatic perivascular epithelioid cell tumor treated by transarterial embolization plus radiofrequency ablation: A case report and literature review. Medicine 2017, 96, e6969. [Google Scholar] [CrossRef] [PubMed]
  147. Hekimoglu, K.; Haberal, M. Liver Perivascular Epithelioid Cell Tumor with an Unusual Location: Diagnostic Characteristics with Multidetector Computed Tomography and Magnetic Resonance Imaging. J. Clin. Imaging Sci. 2017, 7, 36. [Google Scholar] [CrossRef]
  148. Kubo, H.; Yamazaki, H.; Okada, T.; Takahashi, Y.; Nishi, Y.; Yokomori, H. Primary hepatic angiomyolipoma: Immunohistochemistry and electron microscopic observations: A case report. J. Med. Case. Rep. 2017, 11, 76. [Google Scholar] [CrossRef]
  149. Miyata, T.; Yamashita, Y.; Yamao, T.; Umezaki, N.; Tsukamoto, M.; Kitano, Y.; Yamamura, K.; Arima, K.; Kaida, T.; Nakagawa, S.; et al. Hepatobiliary and Pancreatic: Hepatocellular carcinoma developed with angiomyolipoma. J. Gastroenterol. Hepatol. 2017, 32, 547. [Google Scholar] [CrossRef]
  150. Kirnap, M.; Ozgun, G.; Moray, G.; Haberal, M. Perivascular epithelioid cell tumor outgrowth from the liver. Int. J. Surg. Case Rep. 2018, 53, 295–298. [Google Scholar] [CrossRef]
  151. Ma, Y.; Huang, P.; Gao, H.; Zhai, W. Hepatic perivascular epithelioid cell tumor (PEComa): Analyses of 13 cases and review of the literature. Int. J. Clin. Exp. Pathol. 2018, 11, 2759–2767. [Google Scholar]
  152. Nell, R.J.; Wong, D.D.; van Vliet, C.; Parry, J.; Fermoyle, S. Inflammatory angiomyolipoma of the liver: A diagnostic pitfall. Pathology 2018, 50, 352–355. [Google Scholar] [CrossRef]
  153. Voulgari, P.V.; Tatsi, V.; Milionis, H.J.; Goussia, A.; Xydis, V.; Glantzounis, G.K. Liver perivascular epithelioid cell tumor in a patient with systemic lupus erythematosus. Int. J. Surg. Case Rep. 2018, 53, 193–195. [Google Scholar] [CrossRef] [PubMed]
  154. Galera López, M.D.M.; Márquez Rodas, I.; Agra Pujol, C.; García Pérez, Á.; Velasco Sánchez, E.; Álvarez Álvarez, R. Simultaneous diagnosis of liver PEComa in a family with known Li-Fraumeni syndrome: A case report. Clin. Sarcoma. Res. 2020, 10, 24. [Google Scholar] [CrossRef] [PubMed]
  155. Xu, G.; Xu, H.; Yang, H.; Du, S.; Mao, Y. Primary hepatic perivascular epithelioid cell neoplasm (PEComa) with fever in a 53-year-old man. Postgrad. Med. J. 2020, 96, 505–506. [Google Scholar] [CrossRef] [PubMed]
  156. Attard, A.; Piscopo, N.; Schembri, J.; Buhagiar, T.; Cortis, K.; Ellul, P. A Rare Case of PEComa of the Liver. GE Port. J. Gastroenterol. 2021, 28, 217–221. [Google Scholar] [CrossRef]
  157. Ergün, S.; Akinci, O.; Çomunoğlu, N.; Kocael, A. Clear-Cell Myomelanocytic Tumor of Ligamentum Teres Hepatis. Cerrahpaşa Med. J. 2021, 45, 57–59. [Google Scholar] [CrossRef]
  158. He, F.; Xia, Y.; Ling, X. Diagnosis and Individualized Treatment of Three Primary Malignant Tumors: A Case Report. Breast Cancer (Dove. Med. Press) 2021, 13, 519–527. [Google Scholar] [CrossRef]
  159. Huang, Z.; Xin, J.Y.; Li, K.Y. Ultrasound contrast agent Sonazoid for the diagnosis of hepatic epithelioid angiomyolipoma: A case report. BMC Gastroenterol. 2021, 21, 487. [Google Scholar] [CrossRef]
  160. Li, C.; Xu, J.Y.; Liu, Y. Sonazoid-enhanced ultrasonography and pathologic characters of CD68 positive cell in primary hepatic perivascular epithelioid cell tumors: A case report and literature review. Open Med. (Wars) 2021, 16, 737–741. [Google Scholar] [CrossRef]
  161. Li, Y.F.; Wang, L.; Xie, Y.J. Hepatic perivascular epithelioid cell tumor: A case report. World J. Clin. Cases 2022, 10, 4273–4279. [Google Scholar] [CrossRef]
  162. Wang, S.; Xia, H.; Liu, X.; Liu, Y.; Lou, C. Hepatic epithelioid angiomyolipoma mimicking hepatocellular carcinoma on MR and (18)F-FDG PET/CT imaging: A case report and literature review. Hell J. Nucl. Med. 2022, 25, 205–209. [Google Scholar]
  163. Zhang, X.; Chen, J.; Huang, B.; Wang, L. Case report: Hepatic epithelioid angiomyolipoma with elevated alpha-fetoprotein and a history of breast cancer. Front. Surg. 2022, 9, 991228. [Google Scholar] [CrossRef]
  164. Zhang, X.; Zhong, X.; Lin, X.; Li, X.; Tian, H.; Chang, B.; Wang, Y.; Tong, J.; Wang, N.; Li, D.; et al. Tuberous Sclerosis Complex With Multiple Organ Tumors: Case Report and Literature Review. Front. Oncol. 2022, 12, 916016. [Google Scholar] [CrossRef]
  165. Zhu, J.; Wang, G.; Sun, G.; Xie, B.; Xiao, W.; Li, Y. Primary hepatic epithelioid angiomyolipoma: A small case series. ANZ J. Surg. 2022, 92, 1803–1808. [Google Scholar] [CrossRef] [PubMed]
  166. Cai, X.; Sun, S.; Deng, Y.; Liu, J.; Pan, S. Hepatic epithelioid angiomyolipoma is scattered and unsuitable for surgery: A case report. J. Int. Med. Res. 2023, 51, 3000605231154657. [Google Scholar] [CrossRef]
  167. Harwal, R.; Joseph Rosemary, L.J.; Raju, P.; Chidambaranathan, S.; Bharathi Vidya Jayanthi, J.; Obla Lakshmanamoorthy, N.B. Hepatic Perivascular Epithelioid Cell Tumor Mimicking Hepatocellular Carcinoma. ACG Case Rep. J. 2023, 10, e00962. [Google Scholar] [CrossRef]
  168. Ji, J.; Zhang, Y.; Yang, C. Hepatic caudate epithelioid angiomyolipoma mimicking hydatid cyst. Asian J. Surg. 2023, 46, 1700–1701. [Google Scholar] [CrossRef]
  169. Li, Z.; Su, D.; Zhou, S.; Wang, Y.; Chen, Y. Comparison of 18 F-FDG and 68 Ga-FAPI PET/CT in a Patient With Hepatic Perivascular Epithelioid Cell Neoplasm. Clin. Nucl. Med. 2023, 48, 1124–1126. [Google Scholar] [CrossRef]
  170. Marinho, B.M.; Canha, A.G.; Silva, D.S.; Rodrigues, A.P. Hepatic angiomyolipoma, misdiagnosed as hepatocellular carcinoma. J. Surg. Case Rep. 2023, 2023, rjad556. [Google Scholar] [CrossRef]
  171. Matrood, S.; Görg, C.; Safai Zadeh, E.; Alhyari, A. Hepatic perivascular epithelioid cell tumor (PEComa): Contrast-enhanced ultrasound (CEUS) characteristics—A case report and literature review. Clin. J. Gastroenterol. 2023, 16, 444–449. [Google Scholar] [CrossRef]
  172. Mochizuki, K.; Aoki, T.; Kusano, T.; Tomioka, K.; Tashiro, Y.; Koizumi, T.; Matsuda, K.; Enami, Y.; Yamochi, T.; Murakami, M. Laparoscopic Resection of a Hepatic Epithelioid Angiomyolipoma Revealed by Indocyanine Green Fluorescence Imaging. Am. Surg. 2023, 89, 2061–2063. [Google Scholar] [CrossRef]
  173. Wannasai, K.; Charoenchue, P.; Lapisatepun, W.; Wongsuriyathai, T.; Kongkarnka, S. Primary hepatic perivascular epithelioid cell tumor: A case report and review of literature. Hum. Pathol. Rep. 2023, 31, 300699. [Google Scholar] [CrossRef]
  174. Costa, C.J.; Nguyen, M.T.T.; Ibrahim, E.; Potashinsky, A. Perivascular Endothelial Carcinomas: An Uncommon Hepatic Tumor Requiring Resection. Ann. Intern. Med. Clin. Cases 2024, 3, 230. [Google Scholar] [CrossRef]
  175. Lee, D.H.; Lee, J.Y. A comprehensive study on the feasibility and diagnostic potential of fluctuation imaging in liver tumor assessment. Sci. Rep. 2024, 14, 30662. [Google Scholar] [CrossRef]
  176. Liu, X.L.; Cui, J.; Tian, H. Hepatic perivascular epithelioid cell neoplasm in a 58-year-old woman. Pol. Arch. Intern. Med. 2024, 134, 16615. [Google Scholar] [CrossRef]
  177. Tababi, R.; Medhioub, M.; Soussi, I.; Noomen, I.; Kallel, Y.; Yakoubi, M.; Mohamed, A.B.; Mahmoudi, M.; Gharbi, G.; Bouassida, M.; et al. Hepatic Angiomyolipoma: A Case Report and Literature Review. J. Investig. Med. High Impact Case Rep. 2024, 12, 23247096241306542. [Google Scholar] [CrossRef]
  178. Takada, R.; Takahashi, M.; Hayashi, T.; Higashihara, T.; Morita, Y.; Inoue, D.; Okada, H.; Araki, J. Laparoscopic resection of liver PEComa associated with Li-Fraumeni syndrome: A case report. Biomed. Rep. 2024, 21, 154. [Google Scholar] [CrossRef]
  179. Tan, L.L.Y.; Lee, V.T.W.; Lim, T.K.H. A case of liver angiomyolipoma accompanied with multiple focal nodular hyperplasia. Pathology 2024, 56, 755–758. [Google Scholar] [CrossRef]
  180. Vijayanirmala, P.; Yadav, R.; Goyal, S.; Barwad, A.; Bhowmik, S.; Malik, R.; Pal, S.; Sharma, R.; Sakhuja, P.; Das, P. Hepatic and perihepatic PEComas: A study describing a series of five rare cases. Indian J. Pathol. Microbiol. 2024, 67, 355–361. [Google Scholar] [CrossRef]
  181. Yang, H.T.; Wang, F.R.; He, N.; She, Y.H.; Du, Y.Y.; Shi, W.G.; Yang, J.; Chen, G.; Zhang, S.Z.; Cui, F.; et al. Massive simultaneous hepatic and renal perivascular epithelioid cell tumor benefitted from surgery and everolimus treatment: A case report. World J. Gastrointest. Surg. 2024, 16, 3334–3342. [Google Scholar] [CrossRef]
  182. Yazıcı, C.; Gündoğdu, E. Very Rare Liver Tumor: PEComa Case Report with and a Review of Literature. Indian J. Radiol. Imaging 2024, 34, 172–176. [Google Scholar] [CrossRef]
  183. Zaidi, A.; Chatterjee, D.; Bhargav, V.; Gupta, V.; Das, A. Clear cell myomelanocytic tumor of ligamentum teres. Autops. Case Rep. 2024, 14, e2024503. [Google Scholar] [CrossRef] [PubMed]
  184. Zhu, D.; Song, S.; Wang, D.; Kuang, D.; Cheng, S.; Zhou, J.; Zou, S. Hepatic perivascular epithelioid cell tumor resembling hepatic adenoma and hepatocellular carcinoma on preoperative imaging: A case report. Front. Oncol. 2024, 14, 1292313. [Google Scholar] [CrossRef]
  185. Abe, N.; Yamazaki, F.; Tsujikawa, H.; Kasuga, R.; Taniki, N.; Shimada, H. Case review of perivascular epithelioid cell tumor occurring in patients with Li-Fraumeni syndrome. Fam. Cancer 2025, 24, 18. [Google Scholar] [CrossRef] [PubMed]
  186. Boccatonda, A.; Marcellini, M.M.; Ruggeri, E.; Felicani, C.; Brighenti, A.; Loiacono, R.; Ercolani, G.; Serra, C. Ceus features of liver pecoma: A case report and literature review. J. Ultrasound 2025, 28, 261–268. [Google Scholar] [CrossRef]
  187. Paula, D.; Amaral, M.J.; Madeira, J.; Simões, J.; Lázaro, A.; Silva, N.; Tralhão, J.G. Rare Encounter: A Case Report of Hepatic Perivascular Epithelioid Cell Tumor—An Uncommon Mesenchymal Tumor in the Liver. Case Rep. Gastroenterol. 2025, 19, 43–51. [Google Scholar] [CrossRef]
  188. Saadoun, J.E.; Traversari, E.; Meillat, H.; Guiramand, J. Perivascular epithelioid cell tumour (PEComa) of the ligamentum teres hepatis, a rare and mobile tumour presentation. ANZ J. Surg. 2025, 95, 832–833. [Google Scholar] [CrossRef]
  189. Yang, Y.; Lee, J.; Woo, C.G.; Lee, O.J.; Son, S.M. Epithelioid angiomyolipoma of the liver in a patient with Li-Fraumeni syndrome: A case report. Diagn. Pathol. 2024, 19, 16. [Google Scholar] [CrossRef]
Cancers 17 02276 g001
Figure 1. Flow chart of study selection.
Figure 1. Flow chart of study selection.
Cancers 17 02276 g001
Table 1. Patient demographics, medical history, and tumor features in reported cases of primary liver PEComas.
Table 1. Patient demographics, medical history, and tumor features in reported cases of primary liver PEComas.
ParameterValue
Study origin
Asia97 studies (66.6%)
Europe35 studies (24.1%)
North America8 studies (5.5%)
South America3 studies (2.1%)
Africa1 study (0.7%)
Australia1 study (0.7%)
Age46 years (IQR: 35.25–53.75)
Females208 patients (74%)
Comorbidities
Hepatitis B23 patients (8.2%)
Hepatitis C5 patients (1.8%)
History of extrahepatic malignancy30 patients (10.7%)
TSC9 patients (3.2%)
Li–Fraumeni syndrome6 patients (2.1%)
Presentation
Asymptomatic157 patients (55.9%)
Abdominal pain 84 patients (29.9%)
Abdominal discomfort 24 patients (8.5%)
Fever 15 patients (5.3%)
Weight loss 10 patients (3.6%)
Fatigue 7 patients (2.5%)
Vomiting 5 patients (1.8%)
Nausea 5 patients (1.8%).
Diagnostic workup
U/S152 patients (54.1%)
CT221 patients (78.6%)
MRI156 patients (55.5%)
PET-CT31 patients (11%)
Tumor size5.15 cm (IQR: 3–9.3)
Tumor location
Right lobe149 cases (53%)
Left lobe101 cases (34%)
Caudate lobe12 cases (4.3%)
Tumor nodules
Single liver lesion249 patients (88.6%)
Multiple nodules22 patients (7.8%)
Positive immunohistochemical markers
HMB-45261/264 (98.9%)
MELAN-A124/132 (93.2%)
SMA179/200 (89.5%)
Vimentin62/79 (78.5%)
Negative immunohistochemical markers
S-10085/126 (67.5%)
Desmin45/83 (54.2%)
Cytokeratins123/125 (98.1%)
EMA26/28 (92.9%)
HepPar-143/44 (97.7%)
Final diagnosis
Radiology29 cases (10.3%)
Biopsy44 cases (15.7%)
Post-surgical analysis208 cases (74%)
PEComa type
AML197 patients (70.1%)
PEComa-NOS74 patients (26.3%)
CCMMT of the falciform ligament7 patients (2.5%)
Clear-cell sugar tumor3 cases (1.1%)
Malignancy30 cases (10.7%)
Surgery251 patients (89.3%)
Follow-up duration (months)24 months (IQR: 12–48)
Recurrence17 patients (6%)
Death due to PEComa8 patients (2.85%)
Abbreviations: IQR, Interquartile Range; TSC, Tuberous Sclerosis Complex; U/S, Ultrasound; CT, Computed Tomography; MRI, Magnetic Resonance Imaging; PET-CT, Positron Emission Tomography–Computed Tomography; HMB-45, Human Melanoma Black-45; MELAN-A, Melanoma Antigen Recognized by T cells A; SMA, Smooth Muscle Actin; S-100, S-100 protein; EMA, Epithelial Membrane Antigen; HepPar-1, Hepatocyte Paraffin 1; AML, Angiomyolipoma; PEComa, Perivascular Epithelioid Cell Tumor; PEComa-NOS, Perivascular Epithelioid Cell Tumor—Not Otherwise Specified; CCMMT, Clear-Cell Myomelanocytic Tumor.
Disclaimer/Publisher’s Note: The statements, opinions and data contained in all publications are solely those of the individual author(s) and contributor(s) and not of MDPI and/or the editor(s). MDPI and/or the editor(s) disclaim responsibility for any injury to people or property resulting from any ideas, methods, instructions or products referred to in the content.

Share and Cite

MDPI and ACS Style

Papantoniou, K.; Aggeletopoulou, I.; Kalafateli, M.; Triantos, C. Characteristics and Treatment of Primary Hepatic Perivascular Epithelioid Cell Tumor (PEComa) in Adults: A Systematic Review. Cancers 2025, 17, 2276. https://doi.org/10.3390/cancers17142276

AMA Style

Papantoniou K, Aggeletopoulou I, Kalafateli M, Triantos C. Characteristics and Treatment of Primary Hepatic Perivascular Epithelioid Cell Tumor (PEComa) in Adults: A Systematic Review. Cancers. 2025; 17(14):2276. https://doi.org/10.3390/cancers17142276

Chicago/Turabian Style

Papantoniou, Konstantinos, Ioanna Aggeletopoulou, Maria Kalafateli, and Christos Triantos. 2025. "Characteristics and Treatment of Primary Hepatic Perivascular Epithelioid Cell Tumor (PEComa) in Adults: A Systematic Review" Cancers 17, no. 14: 2276. https://doi.org/10.3390/cancers17142276

APA Style

Papantoniou, K., Aggeletopoulou, I., Kalafateli, M., & Triantos, C. (2025). Characteristics and Treatment of Primary Hepatic Perivascular Epithelioid Cell Tumor (PEComa) in Adults: A Systematic Review. Cancers, 17(14), 2276. https://doi.org/10.3390/cancers17142276

Note that from the first issue of 2016, this journal uses article numbers instead of page numbers. See further details here.

Article Metrics

Back to TopTop