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.

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.

Transcription Factor E3 Protein–Positive Perivascular Epithelioid Cell Tumor of the Appendix Presenting as Acute Appendicitis: A Case Report and Review of the Literature

2013 ◽  
Vol 137 (3) ◽  
pp. 434-437 ◽  
Author(s):  
Kristina A. Matkowskyj ◽  
M. Sambasiva Rao ◽  
Kirtee Raparia
Keyword(s):  
Transcription Factor ◽  
Acute Appendicitis ◽  
Neuron Specific Enolase ◽  
Epithelioid Cell ◽  
Renal Angiomyolipoma ◽  
Mesenchymal Tumors ◽  
Perivascular Epithelioid Cell ◽  
Eosinophilic Cytoplasm ◽  
Immunohistochemical Studies ◽  
Perivascular Epithelioid Cell Tumors

Perivascular epithelioid cell tumors (PEComas) are a group of mesenchymal tumors that coexpress melanocytic and smooth muscle markers; their exact origin remains unknown. This group includes renal angiomyolipoma, clear cell sugar tumor, and lymphangioleiomyomatosis, although the term perivascular epithelioid cell tumors is currently used for lesions that exhibit a similar morphologic and immunohistochemical profile throughout the human body. Recently, a distinct subset of PEComas has been shown to harbor transcription factor E3 gene (TFE3) fusions. We report, for the first time, a unique case of TFE3-positive PEComa presenting as acute appendicitis in a 24-year-old woman. Microscopically, the tumor was composed of benign-appearing epithelioid cells with clear and eosinophilic cytoplasm, and arranged in nested and alveolar patterns. Immunohistochemical studies showed diffuse strong positivity for neuron-specific enolase, TFE3, and progesterone receptor and focal strong positivity for human melanoma black–45 (HMB-45) and melanocyte differentiation antigen (Melan-A) in the tumor cells. Although rare, PEComa should be included in the differential diagnosis of mesenchymal tumors of the appendix.

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

Clinicopathological features and treatment of perivascular epithelioid cell tumor.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. e23538-e23538
Author(s):  
Ying Lin ◽  
Xin Liu ◽  
Xiaowei Zhang ◽  
Yong Chen ◽  
Chunmeng Wang ◽  
...  
Keyword(s):  
Sample Size ◽  
Mtor Inhibitor ◽  
Radical Surgery ◽  
Mtor Inhibitors ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Clinicopathological Features ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Malignant Pecoma

e23538 Background: Perivascular epithelioid cell tumor (PEComa) is a rare form of mesenchymal neoplasms. No clinical trial of large sample size regarding medical treatment of PEComa has been reported so far. Previous retrospective studies with small sample size have showed efficacy of mTOR inhibitors as treatment of PEComa. This study aimed to analyze the clinicopathological features of PEComa, and the efficacy of mTOR inhibitor in advanced PEComa. Methods: Medical information of patients diagnosed with PEComa and treated in Fudan University Shanghai Cancer Center were collected. Survival analysis was performed by Kaplan-Meier method. Radiological response was assessed according to RECIST version1.1. Results: A total of 17 patients were treated in our center during June 2007 to June 2020. PEComa mostly occurs in middle-aged women. The most common primary sites are pelvis, lung and abdomen. Relapse usually occurs within 2 years after radical surgery. Radical surgery is the main treatment for PEComa of limited stage, and remains the main option for those with limited recurrent lesions. Of the 13 patients with advanced malignant PEComa in our cohorts, the objective response rate of mTOR inhibitors in advanced malignant PEComa was 45.5%, and median progression-free survival was 27.7 months (95% CI 4.7-50.5 months). 2 patients discontinued mTOR inhibitor treatment due to pneumonitis combined with dyspnea and fever respectively. Conclusions: For advanced malignant PEComa, mTOR inhibitors are effective. Lung toxicity of mTOR inhibitor should be monitored.

scholarly journals Predicting the Behavior of Perivascular Epithelioid Cell Tumors of the Uterine Corpus

2017 ◽  
Vol 141 (3) ◽  
pp. 463-469 ◽  
Author(s):  
Andres Martin Acosta ◽  
Brian P. Adley
Keyword(s):  
Surgical Excision ◽  
Reproductive Organs ◽  
Epithelioid Cell ◽  
World Health ◽  
Clinical Behavior ◽  
Uterine Corpus ◽  
Perivascular Epithelioid Cell ◽  
Complete Surgical Excision ◽  
Aggressive Clinical Behavior ◽  
Perivascular Epithelioid Cell Tumors

Perivascular epithelioid cell tumors (PEComas) are rare neoplasms that share phenotypic features with angiomyolipomas, clear cell sugar tumors, and lymphangioleiomyomatosis. They presumably represent the neoplastic counterpart of a yet-unidentified perivascular epithelioid cell that expresses smooth muscle and melanocytic immunomarkers. The uterus is the second most common site of origin for perivascular epithelioid cell tumors, after the retroperitoneum. Although most uterine perivascular epithelioid cell tumors are clinically benign and can be cured by a complete surgical excision, there is a subset characterized by both local and distant dissemination. Unfortunately, no single histopathologic or immunohistochemical parameter can accurately predict the clinical behavior of these tumors, which is why the 2012 World Health Organization classification of tumors of the female reproductive organs suggests the use of several criteria to predict the risk of aggressive clinical behavior. Here we review those perivascular epithelioid cell tumors of the uterine corpus with aggressive clinical behavior reported in the literature, and we discuss their most relevant clinical and histopathologic features.

scholarly journals Ultrasound Features of a Uterine Perivascular Epithelioid Cell Tumor (PEComa): Case Report and Literature Review

Diagnostics ◽  
2020 ◽  
Vol 10 (8) ◽  
pp. 553
Author(s):  
Luca Giannella ◽  
Giovanni Delli Carpini ◽  
Nina Montik ◽  
Valeria Verdecchia ◽  
Francesca Puccio ◽  
...  
Keyword(s):  
Color Doppler ◽  
Laparoscopic Hysterectomy ◽  
Smooth Muscle Tumor ◽  
Epithelioid Cell ◽  
Uterine Wall ◽  
Imaging Features ◽  
Rare Occurrence ◽  
Mesenchymal Tumors ◽  
Perivascular Epithelioid Cell ◽  
Ultrasound Evaluation

Background: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. One of the most frequent localizations of PEComas is the female genitourinary tract, and the uterus is the most involved site after the kidney. Correct preoperative diagnosis is rarely achieved due to the presence of nonspecific imaging features. We report a case of a uterine PEComa with particular reference to ultrasound’s role in characterizing this rare occurrence. Case presentation: a 45-year-old White woman came to our observation for cyclic abdominopelvic pain and chronic constipation. The pre-surgical ultrasound examination showed a heterogeneous tumor that was 4 cm in size, localized on the right anterolateral uterine wall. The mass had well-delimited borders and a central hypoechoic portion. The use of color Doppler showed a rich, irregular vasculature in the center with low impedance. The preoperative diagnostic hypothesis was of a smooth muscle tumor of uncertain malignant potential. After careful counseling, a surgical approach was decided upon, including a total laparoscopic hysterectomy with bilateral salpingectomy. The histological and phenotypical features were consistent with a uterine PEComa. At the last follow-up, two years after surgery, the patient is alive and well. Conclusions: Uterine PEComa is a rare occurrence that should be included in the differential diagnosis of uterine wall tumors. It can appear as a small uterine mass with heterogeneous echogenicity and a rich vascular pattern during an ultrasound evaluation. This diagnostic suspicion may assist in better surgical planning.

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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