Background: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. The perivascular epithelioid cell (PEC) co-expresses melanocytic and muscle markers. Since no normal counterpart to the PEC has ever been identified in any normal tissue, the cell origin of these tumors is still uncertain. Although, several hypotheses have recently been advanced to explain the histogenesis of PEComa, it remains unclear.
Methods: The aim of this study was to discuss whether differential expression of stem cell-associated proteins could be used to aid in determining the histogenesis of PEComa. For this purpose, we detected the immunoexpression of 5 kinds of stem cell markers on PEComas, including CD29, CD44, CD133, ALDH1, and nestin. In addition to observed histopathologic morphology, we also performed PEComa relevant clinical diagnostic markers (HMB-45, SMA, melan-A, Desmin, Ki-67, S-100 and TFE3) to identify whether they belonged to PEComas.
Results: Our study included 13 PEComa samples, and we obtained positive immunoexpression results as follows: CD29 (13/13), CD44 (8/13), ALDH1 (10/13), nestin (1/13), and CD133 (0/13).
Conclusions: Since CD44 and CD29 are surface proteins associated with MSCs, these results suggest that PEComa might arise from MSCs. However, whether MSCs are the origin of PEComa needs to be further explored in the future.
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Arch Esp Urol
December 2024
Department of Urology, Kocaeli University Faculty of Medicine, 41001 İzmit, Turkey.
Background: Perivascular epithelioid-cell tumour (PEComa) is a rare mesenchymal tumour with low malignant potential. PEComa can be found in many organs throughout the body. In the urinary system, it can be found in the prostate, bladder, and kidney.
View Article and Find Full Text PDFClin J Gastroenterol
January 2025
Department of Gastroenterology and Hepatology, Osaka University Graduate School of Medicine, Suita, Japan.
A 55-year-old man with tuberous sclerosis complex (TSC) was diagnosed with left renal angiomyolipoma (AML), a group of perivascular epithelioid cell tumors called PEComas. He had received the mTOR inhibitor everolimus, which resulted in a complete response. However, a left renal mass relapsed in two years, followed by the occurrence of a hepatic mass five months later.
View Article and Find Full Text PDFFront Oncol
December 2024
Department of Obstetrics and Gynecology, The International Peace Maternity and Child Health Hospital, School of Medicine, Shanghai Jiao Tong University, Shanghai, China.
The distinction between a uterine leiomyosarcoma (uLMS) and a perivascular epithelioid cell neoplasm (PEComa) can be quite challenging. Here we report a 39-year-old woman who underwent a hysteroscopic myomectomy. An intraoperative frozen section pathological examination revealed that the mass was likely to be a mesenchymal malignancy.
View Article and Find Full Text PDFIn Vivo
December 2024
Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
Background/aim: Lymphangioleiomyomatosis (LAM) belongs to the perivascular epithelioid cell tumor (PEComa) family. The relationship between LAM and tuberous sclerosis complex (TSC) is of particular concern in a subset of women with clinically occult LAM involving the pelvic lymph nodes. This study aimed to investigate the clinicopathological features of incidental nodal LAM detected during the surgical staging of gynecological tumors.
View Article and Find Full Text PDFHistopathology
December 2024
Department of Pathology, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Republic of Korea.
Aims: The hepatic perivascular epithelioid cell tumour (PEComa), including angiomyolipoma, exhibits diverse morphology and clinical behaviour; however, its prognostic features remain undefined. This study aimed to investigate its histological features and prognostic factors.
Methods And Results: In total, 132 patients were included.
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