Hepatic Perivascular Epithelioid Cell Tumor Research Articles

Computed tomography imaging features of hepatic perivascular epithelioid cell tumor: A report of two cases and Review of the Literature

Background: Perivascular Epithelioid Cell tumor (PEComa) is an uncommon tumor of mesenchymal origin which can arise at a variety of visceral and soft tissue sites, most frequently in the uterus whereas particularly uncommon in liver,only 8% of PEComa cases. Here, we present two pathologically proven cases of PEComa of the liver, retrospectively discuss the clinical, imaging, histological features and review the literature. Case presentation: Case 1: A 54-year-old female patient with intermittent epigastric discomfort for 2 years and aggravated for 5 days.The laboratory examinations were normal. Computed Tomography (CT) showed the lesions were located on the right lobe. The discomfort had resolved after surgery and PEComa was diagnosed after pathological examination of the surgical specimen. The patient has been followedup for 5 years without recurrence. Case 2: A 30-year-old woman was found to have a mass of liver incidentally during regular physical examination after breast cancer surgery. On contrast enhancement CT, a well demarcated mass was found. After operation, pathological diagnosis was established as malignant PEComa of the right liver. Neither primary recurrence nor metastasis was found during the 2-year follow-up. Conclusion: The CT imaging manifestations of liver PEComa have certain characteristics. When the tumor density is not uniform or contains fat, and thickening vascular shadow appears around the lesion, combined with clinical and laboratory examination, suggestive diagnosis can be made.

Hepatic perivascular epithelioid cell tumor: a retrospective analysis of 36 cases.

Hepatic perivascular epithelioid cell tumor (PEComa) is a rare type of mesenchymal neoplasm and lacks systematic reports. The aim was to analyze the features of hepatic PEComa in order to provide our own experience for diagnosis and management from a single center. We retrospectively analyzed clinical data, imaging findings, pathology,treatments and prognosis of 36 patients with hepatic PEComa in the First Affiliated Hospital of Zhengzhou University from January 2016 to September 2023. 29 females and 7 males (median age, 47.8 years) were included in this study. The majority (26/36, 72.2%) of patients were diagnosed incidentally with non-specific symptoms. Abnormal enhancement of enlarged blood vessels (27/36,75%) can be observed on CT/MRI and only 7 patients (19.4%) were correctly diagnosed by imaging examinations. The positive immunohistochemical expressions were HMB-45(35/36,97.2%), Melan-A (34/35,97.1%), SMA (23/26,88.5%) and CD34(86.7%,26/30). Treatments include resection (24/36,67.7%), radiofrequency ablation (6/36,16.7%), transcatheter arterial chemoembolization(1/36,2.7%), conservative clinical follow-up(2/36,5.6%), and sirolimus-chemotherapy (3/36,8.3%). During the follow-up period (range, 2-81 months), except for one patient with a single intrahepatic recurrence and 3 malignant patients died in 6 months, the remaining patients had no signs of recurrence and metastasis. Hepatic PEComa has no specific clinical features and mainly depends on clinicopathological characteristics for accurate diagnosis. Resection is the best treatment for benign PEComa, but TACE and radiofrequency ablation can also be considered in case of contraindications for surgery.

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

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.

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

The objective of the study was to explore the CT and ultrasound features and clinical significance of perivascular epithelioid cell tumor (PEComa) of the liver. Eleven 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). CT 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. Some 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.

Analysis of the diagnosis and treatment of 24 cases of hepatic perivascular epithelioid cell tumor

Objective: To investigate hepatic perivascular epithelioid cell tumor (PEComa) diagnosis and treatment plan. Methods: 24 cases diagnosed with PEComa clinical manifestations, serum alpha fetoprotein (AFP), carcinoembryonic antigen (CEA), carbohydrate antigen 199 (CA199), imaging findings, surgical methods, postoperative hospital stay, pathological results and prognosis were analyzed retrospectively from September 2015 to September 2020. Results: Majority of patients were females (79.2%), aged 41.5±11.4 years. Tumors were predominantly located in the right liver (50.0%). 76.7% of the cases were mostly clinically asymptomatic. AFP, CEA and CA199 indices were all negative. CT mostly showed low density tumor in the plain scan phase, enhanced in the enhancement phase, and enhanced and weakened in portal venous and equilibrium phase (66.7%). MRI manifestations of most tumors were hypointense on T1WI and hyperintense on T2WI (72.7%). B-ultrasound mostly showed hyperechoic mass in the tumor area with punctate vascular shadow (52.9%). Postoperative hospital stay was 9.0±2.4 days for laparoscopic surgery patients (n=13), 13.4±6.3 days for traditional laparotomy (hereinafter referred to as laparotomy, n=10), and 3 days for 1 patient with microwave ablation. All postoperative pathological results were positive for HMB45 and Melan-A. Follow-up results: 21 cases survived normally, with no tumor recurrence in the recent physical examination; two cases had tumor recurrence and they died two and three years after surgery, and one case was lost to follow-up. Conclusion: Hepatic PEComa more commonly occurs in middle-aged women, with no specific features for tumor markers and clinical manifestations. Some imaging findings are specific, so its features can be combined as a basis for diagnosis. Postoperative pathological examination results can confirm the diagnosis. Therefore, surgery remains the initial treatment plan. Microwave ablation and laparoscopic surgery are recommended as the preferred option because of shorter hospital stays and less trauma than open surgery.

Hepatic perivascular epithelioid cell tumor: Clinicopathological analysis of 26 cases with emphasis on disease management and prognosis.

BACKGROUNDPerivascular epithelioid cell tumor (PEComa) is an uncommon tumor of mesenchymal origin. Cases of PEComa in the liver are extremely rare.AIMTo analyze the clinicopathological features and treatment of hepatic PEComa and to evaluate the prognosis after different treatments.METHODSClinical and pathological data of 26 patients with hepatic PEComa were collected. All cases were analyzed by immunohistochemistry and clinical follow-up.RESULTSThis study included 17 females and 9 males, with a median age of 50 years. Lesions were located in the left hepatic lobe in 13 cases, in the right lobe in 11, and in the caudate lobe in 2. The median tumor diameter was 6.5 cm. Light microscopy revealed that the tumor cells were mainly composed of epithelioid cells. The cytoplasm contained heterogeneous eosinophilic granules. There were thick-walled blood vessels, around which tumor cells were radially arranged. Immunohistochemical analysis of pigment-derived and myogenic markers in PEComas revealed that 25 cases were HMB45 (+), 23 were Melan-A (+), and 22 SMA (+). TFE3 and Desmin were negative in all cases. All the fluorescence in situ hybridization samples were negative for TFE3 gene break-apart probe. Tumor tissues were collected by extended hepatic lobe resection or simple hepatic tumor resection as the main treatments. Median follow-up was 62.5 mo. None of the patients had metastasis or recurrence, and there were no deaths due to the disease.CONCLUSIONHepatic PEComa highly expresses melanin and smooth muscle markers, and generally exhibits an inert biological behavior. The prognosis after extended hepatic lobe resection and simple hepatic tumor resection is semblable.

Simultaneous diagnosis of liver PEComa in a family with known Li\u2013Fraumeni syndrome: a case report

BackgroundLi–Fraumeni syndrome (LFS) is an autosomal dominant hereditary disease. It is associated with the loss of function of the p53 protein and an increased risk of malignant tumor development at early age. The most frequently detected tumors include breast cancer, sarcomas, leukemia, brain tumors, and adrenocortical carcinomas. While sarcomas account for only 1% of solid tumors, they are more frequently detected in these families.Case presentationWe report a simultaneous diagnosis of hepatic perivascular epithelioid cell tumor (PEComa), a very rare subtype of sarcoma, in two siblings with a LFS.ConclusionsThe simultaneous diagnosis of PEComa in two siblings presented in this case allowed us to review the frequency of PEComa in this genetic syndrome previously reported, which was very little. Despite its rarity, PEComa must be considered in the differential diagnosis of new-onset liver lesions in patients who were previously diagnosed with LFS.

Hepatic perivascular epithelioid cell tumor: a report of 9 cases and literature review

回顾性分析2015年1月至2018年12月郑州大学第一附属医院病理确诊的9例肝血管周上皮细胞肿瘤(PEComa)患者，对其临床诊疗和预后进行分析。患者随访5.0～51.0个月，1例患者穿刺活检未进一步治疗，出院后6个月因肾脏肿瘤破裂死亡，另8例手术切除患者未发现肿瘤复发及转移。肝PEComa以女性多发，临床症状不典型，可伴腹痛、腹胀不适、食欲减退、发热、体重减轻。肝PEComa常单发，以肝右叶多见。术前完善影像学检查可协助诊断，CT平扫肿瘤呈均匀低密度或不均匀混杂密度，增强扫描动脉期表现为明显不均匀强化。肝PEComa以手术切除为主，良性首选手术治疗且预后良好，恶性需综合治疗。

Chills and fever as the first presentation of hepatic perivascular epithelioid cell tumor.

A 70-year-old female was admitted to the hospital with a history of chills and fever (with a high temperature of 37.5 °C) for 17 days. No obvious anomalies were detected on physical examination. Laboratory tests indicated white blood cells (6.96×10 9 /L), neutrophil percentage (65%), C-reactive protein, and procalcitonin (0.766 ng/mL) in the normal range. The liver function was normal, with Child-Pugh grade A, and the patient tested negative for hepatitis B virus infection.

A case report of hepatic perivascular epithelioid cell tumours (pecomas) and literature review

Perivascular epithelioidcelltumours (PEComas) belongs tothe family of mesenchymal neoplasms that can occur in many organs, but rarely found in liver. Preoperative diagnosis could be challenging due to unspecific and variable radiologic patterns, which can be clinically misdiagnosed with hepatocellular carcinoma and other benign tumors. This report aims to announce a case of hepatic PEComa with extramedullary hematopoiesis. A 44- year- old woman accidentally presented a nodular mass in the left hepatic segment on ultrasonography with normal liver function result. Abdominal enhanced CT Scan showed hypervascular lesion. Morphology exibits classical characteristics of PEComa with mature fat tissue predominance, thick-wall vessel; somewhat amount of extramedullary hematopoiesis was also recognized. Tumor cells are diffusely immunoreactive with HMB45. PEComa is a rare disease in liver, pathologists need to recognize three components of this tumor to avoid misdiagnosing with malignant conditions.

Hepatic perivascular epithelioid cell tumor in three patients

Perivascular epithelioid cell tumor (PEComa) is a rare, soft tissue tumor that can occur in various locations. The present report included three patients (one male and two females; age range, 25-51 years) with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is difficult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohistochemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues including smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant (double-positive for CD34 and Ki-67) and recurred 3 months after surgery. In addition, malignant hepatic PEComas were reviewed in the literature, indicating that the majority of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients (>50 years of age) with abdominal discomfort and pain, larger tumor size (>10 cm), or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohistochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.

Diagnosis and treatment of hepatic perivascular epithelioid cell tumor: A case report and literature review

Diagnosis and treatment of hepatic perivascular epithelioid cell tumor: A case report and literature review

Contrast-enhanced ct and angiographic findings in hepatic perivascular epithelioid cell tumor

We report a case of hepatic perivascular epithelioid cell tumor (PEComa) in a woman who was not a carrier of viral hepatitis and had a normal alpha-fetoprotein (AFP) level. CT scan showed a well-enhanced mass in the arterial-phase followed by early washout in the portal venous-phase in the lateral segment of the liver. Angiography revealed a hypervascular tumor in the liver with rapid washout of the contrast. If a hepatic tumor is found in a female patient with normal AFP level who is not a carrier of hepatitis and is free of alcoholic liver cirrhosis, tissue biopsy should precede treatment to avoid misdiagnosis of liver PEComa as hepatocellular carcinoma.

Hepatic perivascular epithelioid cell tumor:a report of one case

1临床资料患者女,28岁.因背部疼痛6个月,彩色多普勒超声检查发现肝脏占位性病变2周于2011年7月26日入院.体格检查:皮肤、巩膜未见黄染,腹平软,无压痛及肌紧张,肝、脾肋下未触及.实验室检查:血、尿常规和血生化检查无异常;AFP 500 U/L,CEA 490 ng/L,CA19-96590 U/L,ICG R153％. 关键词：血管周上皮样细胞瘤,肝脏;诊断;治疗

Hepatic Perivascular Epithelioid Cell Tumor: A Case Report and Review of the Literature

A perivascular epithelioid cell tumor (PEComa) is a rare type of mesenchymal tumor. Cases that arise from the liver are extremely rare. We report a case of a 41-years-old woman suffering from a hepatic PEComa with an emphasis on its imaging findings, primarily those of contrast-enhanced computer tomography (CECT) and sonography. We also conducted a literature review to evaluate imaging findings that could provide some information for preoperative diagnosis.

Hepatic perivascular epithelioid cell tumor: Five case reports and literature review

Perivascular epithelioid cell tumor (PEComa) is a rare tumor. Here, we present data regarding clinical presentations, diagnoses, management, and prognosis of five cases of hepatic PEComa between January 2002 and December 2008. Ultrasonography showed hyperechoic masses in all patients. Precontrast computed tomography (CT) showed that all lesions scanned were heterogeneous in density and were heterogeneously enhanced in arterial phase images. In two cases, magnetic resonance imaging showed hypointensity on T1-weighted images and hyperintensity on T2-weighted images. In enhanced scanning, lesions showed asymmetrical enhancement during arterial phase imaging. All tumors were composed of varying proportions of smooth muscle, adipose tissue, and thick-walled blood vessels, and showed positive immunohistochemical staining for Human Melanoma Black-45. All patients underwent hepatectomy, and there was no evidence of recurrence or metastasis during the follow-up period.

Hepatic perivascular epithelioid cell tumor (PEComa): dynamic CT, MRI, ultrasonography, and pathologic features—analysis of 7 cases and review of the literature

To evaluate the dynamic CT, MRI, ultrasonography, and pathologic features of hepatic perivascular epithelioid cell tumor (PEComa), improving the understanding and diagnosis of the tumor. A retrospective analysis of CT, MRI, ultrasonography, and pathologic features of 7 hepatic PEComas diagnosed by pathology during 1st January 2005 to 1st September 2011 in our hospital. The performance of dynamic CT, MRI, and ultrasonography revealed that lesions were regular masses with well-defined borders, the maximum diameters were 2.5-8.5 cm (mean = 4 cm), density was homogeneous, contrast-enhanced CT and MRI showed the lesions were significantly and heterogeneously enhanced on arterial phase, less enhanced on portal venous phase, and slightly hypodense on delayed phase. One patient had multiple hepatic lesions and had delayed enhancement. There were no backgrounds of hepatitis and cirrhosis, enlarged lymph nodes, or distant metastases. Pathology showed the gross appearance of the tumor was smooth. Tumor cells were round or polygonal, with clear boundaries and clear membranes, and had abundant translucent cytoplasm. Nuclei were round, with medium size. Tumor cells were epithelial-like cells and arranged in dense sheets. Immunohistochemistry showed that most of them were positive in HMB45 and MelanA, S-100, SMA, while negative in CgA, Syn, CK, CD117, CD10, and CD34. Dynamic CT, MRI, ultrasonography, and pathology of PEComa had some characteristics of benign tumor's performance. Enhanced scan showed PEComa quickly enhanced on arterial phase and enhanced less on portal venous phase. Knowing these characteristics could help to improve the understanding and diagnosis of hepatic PEComa.

Perivascular epithelioid cell tumor of the liver coexisting with a gastrointestinal stromal tumor

Approximately 10% of patients with gastrointestinal stromal tumors (GIST) develop other neoplasms, either synchronously or metachronously. In this report we describe coexistence of a gastrointestinal stromal tumor and a hepatic perivascular epithelioid cell tumor (PEComa) in a 51-year-old woman with no evidence of tuberous sclerosis. A subcapsular hepatic nodule (0.8 cm in diameter) was found during surgery for symptomatic gastric neoplasm (15 cm in diameter) arising from the lesser curvature. Both tumors revealed histomorphological and immunohistochemical features confirming a diagnosis of a small incidental hepatic PEComa and a high risky extramural gastric GIST, respectively. The patient remained disease-free 25 mo after surgery with no evidence of tumor recurrence or new neoplasms. To our knowledge, this is the first report of PEComa in a patient with GIST. Hepatic lesions detected synchronously or metachronously in patients with GISTs may represent histogenetically distinct lesions and should be sampled to confirm or exclude metastatic GISTs.