Bladder Perivascular Epithelioid Cell Tumor: Dynamic CT and MRI Presentation of 2 Cases With 2-year Follow-up and Review of the Literature

AbstractA 49-year-old woman consulted her general practitioner (GP) regarding epigastric pain that she had experienced for 2 months. Physical examination and laboratory results were unremarkable. An abdominal ultrasound indicated a solid pancreatic tumor, which was confirmed on subsequent CT and MRI. Endoscopic ultrasound (EUS) showed a well-defined heterogeneous, predominantly hypoechoic mass in the pancreatic body, so a neuroendocrine tumor (NET) was suspected. However, EUS-guided fine-needle aspiration (EUS-FNA) was performed and based on (immuno-)histochemical findings, the extremely rare diagnosis of a perivascular epithelioid cell tumor (PEComa) of the pancreas was made. Due to the malignant potential of pancreatic PEComas, laparoscopic left-sided pancreatectomy was performed. We present a case diagnosed by preoperative EUS-FNA highlighting the clinical and endosonographic features which help to distinguish it from its most important differential diagnosis, neuroendocrine tumors (NETs) of the pancreas.

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Malignant perivascular epithelioid cell tumor in children: description of a case and review of the literature

Journal of Pediatric Surgery ◽

10.1016/j.jpedsurg.2012.02.023 ◽

2012 ◽

Vol 47 (6) ◽

pp. e31-e40 ◽

Cited By ~ 9

Author(s):

Rita Alaggio ◽

Giovanni Cecchetto ◽

Guido Martignoni ◽

Gianni Bisogno ◽

Liang Cheng ◽

...

Keyword(s):

Epithelioid Cell ◽

Cell Tumor ◽

Review Of The Literature ◽

Perivascular Epithelioid Cell Tumor ◽

Perivascular Epithelioid Cell

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

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135116682452 ◽

2017 ◽

Vol 11 (4) ◽

pp. NP14-NP17 ◽

Cited By ~ 1

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.

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Primary cutaneous perivascular epithelioid cell tumor (PEComa): Five new cases and review of the literature

Journal of Cutaneous Pathology ◽

10.1111/cup.12972 ◽

2017 ◽

Vol 44 (8) ◽

pp. 713-721 ◽

Cited By ~ 7

Author(s):

Lauren N. Stuart ◽

Russell G. Tipton ◽

Michael R. DeWall ◽

Douglas C. Parker ◽

Christina D. Stelton ◽

...

Keyword(s):

Epithelioid Cell ◽

Cell Tumor ◽

Review Of The Literature ◽

Perivascular Epithelioid Cell Tumor ◽

Perivascular Epithelioid Cell

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

10.21203/rs.3.rs-733408/v1 ◽

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.

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