Liver PEComa 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.

Systematic Review of Literature Published by Spanish Authors about Liver PEComa

Systematic Review of Literature Published by Spanish Authors about Liver PEComa

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.

Liver PEComa. An extremely rare case report with complicated post-operative course

Background: Perivascular epithelioid cell tumor (PEComa) is a rare type of mesenchymal tumor with heterogeneous clinical behavior. Liver PEComas are very rare. Methods: The case of a 47-year-old man with hepatic PEComa and a complicated postoperative course is reported. Results: The patient had a history of systemic lupus erythematosus (SLE) and developed epigastric pain. An abdominal CT showed a large (7 cm) heterogeneous mass on segments I/IV, in direct contact with the inferior vena cava and portal vein. Percutaneous liver biopsy showed PEComa and the patient underwent left hepatectomy. On post-op day 7, he became pyrexial, with significant elevation in transaminases and inflammatory markers. Imaging (CT/MRI) showed a hypodense area in segment VIII close to resection margin. Blood cultures were taken and broad spectrum iv antibiotics were administered. As he continue to be pyrexial with a clinical picture of sepsis he underwent a second laparotomy, where necrosis of segment VIII was found and it was resected. As he became again pyrexial (on day 7), a new CT abdo was done revealing concentric hepatic artery stenosis with hypodense areas on segment VI with sphenoid shape. Acute exacerbation of SLE with right hepatic artery partial thrombosis or vasculitis was considered to be the diagnosis. He was commenced on steroids and anticoagulants with good clinical response. Conclusion: Management of hepatic PEComas can be very challenging especially in patients with significant co-morbidities.

Liver pecoma: perivascular epithelioid tumor. a case report.

Background: Perivascular epithelioid cell tumors (PEComa) are an unusual mesenchymal tumors family, including the classic angiomyolipoma (AML), lypmphangioleiomyomatosis and clear-cell tumors, that can arise from different locations of the body [1–4]. We present a rare case of a Liver PEComa. Methods: A 58-year-old female with no background of cirrhosis or any other liver disease, who in an abdominal ultrasound, presented an exophytic heterogenic/solid mass of 5x6 cm in the left lateral section. Computed Tomography found a hypodense mass of 6.2x4.9x6.2 cm with fat density areas, quickly enhanced in the arterial phase and becoming less enhanced on venous and delayed phases of imaging, suggesting a PEComa as diagnosis. Laparoscopic Left Lateral Sectionectomy was performed. Pathology findings were a mix of three type of components: Adipose, vascular and perivascular epithelioid cells, HMB-45+, with conclusive findings of an angiomyolipoma. The patient was discharged at the second postoperative day, with no complications so far. Results: Liver PEComas were before considered as benign tumors, with surgical treatment in selected cases. The publications in the medical literature of some cases that confirm a malign transformation of AML have made the surgical indications for this pathology a controversial issue, recommending it in exophytic, >5cm or fast growing tumors [1–3]. Conclusion: Nowadays, liver PEComas are considerate as rare tumors with risk of malignancy in some cases, making the surgical intervention the primary treatment.

Liver PEComa. An extremely rare case report with complicated post-operative course.

Liver PEComa. An extremely rare case report with complicated post-operative course.

Unresectable hepatic PEComa: a rare malignancy treated with stereotactic body radiation therapy (SBRT) followed by complete resection

BackgroundPerivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors occurring in various anatomic regions. Although diagnostic criteria and treatment management are not established, current treatment options consist of surgery and chemotherapy including mTOR inhibitors.Stereotactic body radiation therapy (SBRT) is a non-invasive ablative treatment which has shown excellent control rates for more common types of unresectable liver tumors and metastases. In this report we present a rare case of PEComa of the liver that was treated by stereotactic radiotherapy followed by resection. Staging and evaluation of treatment response was done by FDG-PET/CT. This case highlights the potential of SBRT as a neoadjuvant treatment even for rare liver malignancies. It is the first case of liver PEComa treated by SBRT and resection.Case presentationA 52-year-old woman presented at an external hospital with abdominal pressure and pain in the right upper abdominal quadrant. A CT scan showed a 700 cm3 liver lesion in segment IV. In repeated biopsy in July 2015 histopathological workup showed a pleomorphic epitheloid tumor with small to medium sized cells expressing vimentin and melan-A while being negative for cytokeratin establishing the diagnosis of PEComa of the liver.To achieve high, ablative doses a stereotactic body radiotherapy (SBRT) technique was chosen consisting of 60Gy (biologically effective dose 105Gy) in 8 fractions of 7.5Gy. Radiotherapy planning was based on MRI resulting in a planning target volume (PTV) of 1944 cm3. Treatment toxicity was limited to a slight elevation of transaminases (grade 1 and 3). A complete resection was performed 21 weeks after radiotherapy confirmed by negative surgical margins.At last follow-up 21 months after therapy, MRI showed neither local nor distant tumor recurrence. The patient was in stable condition (ECOG 1) and without late radiation toxicity.ConclusionsThis is the first documented case of liver PEComa treated by SBRT and resection. A favorable post-treatment course demonstrates that SBRT is a potential neoadjuvant treatment that is capable of reducing an inoperable rare liver tumor to a resectable lesion.

Our experience in the surgical treatment of liver PEComa

Our experience in the surgical treatment of liver PEComa

Growing Perivascular Epithelioid Cell Tumor of the Liver Studied With Contrast-Enhanced Ultrasonography and Magnetic Resonance Imaging

Perivascular epithelioid cell tumors (PEComas) are mesenchymal tumors with a usually benign biological behavior. 1,2 Perivascular epithelioid cell tumors constitute a family of mesenchymal neoplasms that include angiomyolipoma (AML), lymphangioleiomyomatosis, clear cell sugar tumors of the lung, and clear cell myomelanocytic tumors, arising in visceral and soft tissue; all show the presence of a particular cell type, known as a perivascular epithelioid cell. Angiomyolipoma of the liver belongs to this family, usually shows a prevalent component of large epithelioid cells, and is most frequently a slow-growing benign lesion. 1-4 We report a case of a rapidly growing liver PEComa in a patient with melanoma. Contrast-enhanced ultrasonography (CEUS), magnetic resonance imaging (MRI) with a hepatospecific contrast medium, and histologic examination were performed. During the follow-up, the dimensional increase and history of melanoma led to surgery, and a PEComa with no signs of malignancy was confirmed.