scholarly journals Role of Chemotherapy, VEGFR Inhibitors, and mTOR Inhibitors in Advanced Perivascular Epithelioid Cell Tumors (PEComas)

2019 ◽  
Vol 25 (17) ◽  
pp. 5295-5300 ◽  
Author(s):  
Roberta Sanfilippo ◽  
Robin L. Jones ◽  
Jean-Yves Blay ◽  
Axel Le Cesne ◽  
Salvatore Provenzano ◽  
...  
Keyword(s):  
Mtor Inhibitors ◽  
Epithelioid Cell ◽  
Perivascular Epithelioid Cell ◽  
Perivascular Epithelioid Cell Tumors

scholarly journals PEComa: A Perivascular Epithelioid Cell Tumor in the Liver—A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
Author(s):  
Faseeh Khaja ◽  
Allison Carilli ◽  
Said Baidas ◽  
Aravindhan Sriharan ◽  
Shanedelle Norford
Keyword(s):  
Malignant Tumors ◽  
Mtor Inhibitors ◽  
Epithelioid Cell ◽  
Soft Tissue Tumors ◽  
Cell Tumor ◽  
The Body ◽  
Benign Tumors ◽  
Review Of The Literature ◽  
Perivascular Epithelioid Cell ◽  
Perivascular Epithelioid Cell Tumors

Perivascular epithelioid cell tumors are soft tissue tumors that can occur in various locations in the body whose incidence is rising. Hepatic PEComas are quite rare and diagnosis involves positivity of Melan-A and HMB45 on immunohistochemistry. Usual treatment is surgery for benign tumors and chemotherapy including mTOR inhibitors for malignant tumors. Here we discuss the radiological and pathological diagnosis, evaluation, and management of a hepatic PEComa. We describe a 51-year-old patient who was diagnosed incidentally after unusual physical exam findings.

scholarly journals Rapidly enlarging malignant abdominal PEComa with hepatic metastasis: a promising initial response to sirolimus following surgical excision of primary tumor

2020 ◽  
Vol 2020 (3) ◽  
Author(s):  
Dustin J Uhlenhopp ◽  
Jacob West ◽  
Jonathan Heckart ◽  
Ruth Campbell ◽  
Abdelaziz Elhaddad
Keyword(s):  
Mtor Inhibitor ◽  
Mtor Inhibitors ◽  
Surgical Excision ◽  
Initial Response ◽  
Epithelioid Cell ◽  
Primary Lesion ◽  
Mesenchymal Tumors ◽  
Perivascular Epithelioid Cell ◽  
Metastatic Lesions ◽  
Perivascular Epithelioid Cell Tumors

Abstract Intra-abdominal perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Although no effective therapies have been agreed upon, mTOR inhibitors are currently being investigated as a potential therapy for this extremely rare tumor. We present a case of a 64-year-old male found to have a large intra-abdominal PEComa with multiple metastatic lesions in the liver. Patient underwent surgical resection of the primary lesion in the abdomen and sigmoid colon followed by adjuvant therapy with the mTOR inhibitor, sirolimus. Initial response was noted with a decrease in size and number of lesions found in the patient’s liver. After 8 months of therapy, restaging imaging showed disease progression in the liver lesions. Patient subsequently failed treatments with pazopanib, investigational therapy TAK-228 (Sapanisertib) and nivolumab and ipilimumab. Overall the patient died after 22 months of disease. PEComas generally follow a benign course. This case is a much rarer entity given the malignant features/outcome.

Primary perivascular epithelioid cell tumors of the liver: CT/MRI findings and clinical outcomes

2017 ◽  
Vol 42 (6) ◽  
pp. 1705-1712 ◽  
Author(s):  
Martin E. O’Malley ◽  
Tanya P. Chawla ◽  
Lisa P. Lavelle ◽  
Sean Cleary ◽  
Sandra Fischer
Keyword(s):  
Clinical Outcomes ◽  
Epithelioid Cell ◽  
Mri Findings ◽  
Liver Ct ◽  
Perivascular Epithelioid Cell ◽  
Perivascular Epithelioid Cell Tumors ◽  
Tumors Of The Liver
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