Cardiac Perivascular Epithelioid Cell Tumor: Report of a Child and Review of the Literature

2017 ◽  
Vol 11 (4) ◽  
pp. NP14-NP17 ◽  
Author(s):  
Sanjay Rao ◽  
P. Pavithra ◽  
Sunil Bhat ◽  
Colin John ◽  
Ashley J. D’Cruz
Keyword(s):  
Treatment Options ◽  
Contemporary Literature ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Cardiac Tumors ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Mass Size ◽  
Midline Sternotomy

Cardiac tumors presenting as mediastinal masses in childhood are rare. This report describes the case of a 6-year-old girl who presented to us after a failed attempt at resection of a mass arising from the left atrial appendage. Computed tomography–guided biopsy suggested a perivascular epithelioid cell tumor (PEComa). Sirolimus was started in order to reduce the mass size. She subsequently underwent resection of the mass by midline sternotomy approach and cardiopulmonary bypass. Recovery was uneventful and the child is doing well at two-year follow-up. Contemporary literature is reviewed. Only seven cases, including two children, with mediastinal PEComa have been reported. Pathology and treatment options are discussed.

scholarly journals Renal Perivascular Epithelioid Cell Tumor with Lymph Nodes Metastasis: A Case Report and Literature Review

2021 ◽  
Author(s):  
Lingkai Cai ◽  
Qiang Cao ◽  
Hongjin Hua ◽  
Qikai Wu ◽  
Baorui Yuan ◽  
...  
Keyword(s):  
Lymph Nodes ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Epithelioid Angiomyolipoma ◽  
Case Presentation ◽  
Perivascular Epithelioid Cell Tumor ◽  
Lymph Nodes Metastasis ◽  
Perivascular Epithelioid Cell ◽  
Renal Hilum

Abstract Background: Perivascular epithelioid cell tumor (PEComa) is mesenchymal tumor that originated from perivascular epithelioid cells. Angiomyolipoma (AML) is a common benign PEComa, composed of blood vessels, smooth muscle and mature adipose tissue. Epithelioid angiomyolipoma (EAML) is a rare subtype of AML that has the potential to be malignant.Case presentation: The patient was a 42-year-old woman admitted to the hospital for her left low back swelling. The computed tomography angiography (CTA) revealed a 6.3*5.5*6.7cm cystic-solid tumor in the intermediate kidney. Then we performed a left nephrectomy. Postoperative pathology showed that the tumor was angiomyolipoma (PEComa) with necrotic formation and was 6cm*6cm*5.5cm in size. Additionally, lymph nodes involved (4/17) were observed in the left renal hilum. Immunohistochemistry staining indicated that tumor cells focally expressed MelanA and HMB45. No evidence of disease progression at the six-month follow-up after surgery.Conclusions: Lymph nodes involvement in renal PEComa was rare and was regarded as a type of metastasis. Lymph nodes metastasis might indicate a poor prognosis.

Malignant perivascular epithelioid cell tumor (PEComa) arising in the broad ligament

2004 ◽  
Vol 14 (5) ◽  
pp. 1036-1039 ◽  
Author(s):  
D. Fink ◽  
D. E. Marsden ◽  
L. Edwards ◽  
C. Camaris ◽  
N. F. Hacker
Keyword(s):  
Broad Ligament ◽  
Total Abdominal Hysterectomy ◽  
Abdominal Hysterectomy ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Mesenchymal Neoplasm ◽  
Eosinophilic Cytoplasm

Malignant perivascular epithelioid cell tumor (PEComa) is an extremely rare mesenchymal neoplasm mostly composed of HMB-45-positive epithelioid cells with clear-to-eosinophilic cytoplasm, a propensity for perivascular distribution and a coexpression of smooth muscle markers. The uterus seems to be one of the most prevalent sites of involvement, although only 14 cases of uterine PEComa have been described. We report the case of a 51-year-old woman with a PEComa arising in the broad ligament. She was treated with total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and pelvic radiation, and remains without evidence of disease 15 months after diagnosis. This is, to the best of our knowledge, the first report of a malignant PEComa arising in the broad ligament. To correctly diagnose PEComa, an extensive immunohistochemical panel is essential. As PEComas can behave in an aggressive manner, careful follow-up is warranted.

scholarly journals Malignant Perivascular Epithelioid Cardiac Sarcomas: A Case Report and a Review of the Literature

2015 ◽  
Vol 2015 ◽  
pp. 1-5
Author(s):  
Candice Baldeo ◽  
Abdul wahab Hritani ◽  
Robert Ali ◽  
Sana Chaudhry ◽  
Fawad N. Khawaja
Keyword(s):  
Gold Standard ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Cardiac Tumors ◽  
Optimal Management ◽  
Review Of The Literature ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Complete Surgical Resection ◽  
Primary Cardiac Sarcoma

Cardiac tumors, either benign or malignant, are difficult to diagnose due to their rarity, variety, and nonspecific presentation. Since primary cardiac sarcoma remains an unusual diagnosis, the literature on its presentation, diagnosis, and optimal management remains scarce. To our knowledge the following case of cardiac perivascular epithelioid cell tumor is the fourth reported case found in the literature. Although complete surgical resection remains the gold standard for cardiac sarcomas, our case demonstrates that not all of them can be completely resected.

Perivascular Epithelioid Cell Tumor of the Urinary Bladder: A Systematic Review

2019 ◽  
Vol 28 (4) ◽  
pp. 393-400 ◽  
Author(s):  
Ameer Hamza ◽  
Charles C. Guo
Keyword(s):  
Urinary Bladder ◽  
Clinical Presentation ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Small Subset ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Common Clinical Presentation ◽  
Immunohistochemical Studies

Perivascular epithelioid cell tumor (PEComa) of the urinary bladder is a rare neoplasm showing distinct melanocytic and smooth muscle differentiation. We aimed to review the clinicopathologic features of bladder PEComa using all the available cases in the literature, along with 2 new cases from our database. The patients included 15 females and 15 males with a mean age of 39.2 ± 15.3 years. Painless hematuria was the most common clinical presentation. The tumors were usually well circumscribed with a mean tumor size of 4.4 ± 2.7 cm. Bladder PEComas demonstrated nests, trabeculae, or sheets of epithelioid cells with intermixed spindled cells and numerous thin-walled vessels. Immunohistochemical studies showed that the tumors were positive for HMB45 (27/27), cathepsin (4/4), SMA (20/22), and caldesmon (3/3) and were negative for pan cytokeratin (0/18) and EMA (0/4). Molecular studies revealed that PEComa was associated with the TFE3 (n = 3) and EWSR1 (n = 1) gene rearrangements. Treatment included partial cystectomy (n = 18), transurethral resection (n = 8), and radical cystectomy (n = 4). Twenty patients had no evidence of disease during a mean follow-up time of 19.4 ± 17.2 months. Two patients had recurrence, and 1 patient died of metastatic disease. In conclusion, bladder PEComas demonstrate distinct morphologic and immunohistochemical features. Although most tumors follow a benign course, a small subset may develop metastasis and cause death.

LONG-TERM FOLLOW-UP OF MEDIASTINAL PERIVASCULAR EPITHELIOID CELL TUMOR

CHEST Journal ◽  
2018 ◽  
Vol 154 (4) ◽  
pp. 625A
Author(s):  
RIJU DASGUPTA ◽  
CHELSEA LEIPOLD ◽  
PATRICK ELWOOD ◽  
SIVARAMAN SIVASWAMI ◽  
ASOK DASGUPTA ◽  
...  
Keyword(s):  
Epithelioid Cell ◽  
Cell Tumor ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Long Term Follow Up

scholarly journals Liver Perivascular Epithelioid Cell Tumor with an Unusual Location: Diagnostic Characteristics with Multidetector Computed Tomography and Magnetic Resonance Imaging

2017 ◽  
Vol 7 ◽  
pp. 36 ◽  
Author(s):  
Koray Hekimoglu ◽  
Murat Haberal
Keyword(s):  
Poor Prognosis ◽  
Epithelioid Cell ◽  
Cell Tumor ◽  
Rare Tumor ◽  
Resonance Imaging ◽  
Perivascular Epithelioid Cell Tumor ◽  
Perivascular Epithelioid Cell ◽  
Diagnostic Characteristics ◽  
Curative Hepatectomy

Primary perivascular epithelioid cell tumor (PEComa) of the liver is a very rare tumor that originates from mesenchyma. Gastrointestinal tract with perivascular distribution is the most common anatomic sites of these tumors. Only few cases of hepatic PEComa have been described so far. Malignant PEComas exhibit aggressive behavior with poor prognosis, making early diagnosis crucial. Hereby, we report a 79-year-old female with unusually located mass in the liver. A partial curative hepatectomy has been done, and PEComa was diagnosed histopathologically. No evidence of recurrence was observed during the 6-month follow-up.

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