Perivascular epithelioid cell tumor (PEComas) are a group of ubiquitous neoplasms described in different organs that share distinctive morphologic, immunohistochemical, ultrastructural, and genetic features. They have been reported in several organs such as the uterus, lung, kidney, liver, small and large bowel, and prostate. To the best of our knowledge, only 8 cervical PEComa cases have been described. We report the case of a 43-yr-old woman who presented with abnormal uterine bleeding. Clinical diagnosis of a malignant cervical lesion followed an excision, histopathologically evaluated as PEComa. The hysterectomy specimen confirmed the diagnosis by strong HMB-45 positivity, weak S100 positivity, and focal, moderate cytoplasmic TTF-1 positivity, and negative melan A, SMA, desmin, vimentin, cytokeratins, CD1a and other markers. The patient was negative for tuberous sclerosis complex, did not receive additional therapy, and 3 yr later is disease free. Cervical PEComas are very rare tumors but have to be considered in the differential diagnosis of cervical lesions exhibiting unusual cytologic and immunohistochemical characteristics.
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http://dx.doi.org/10.1097/PGP.0000000000000436 | DOI Listing |
Am J Surg Pathol
January 2025
Departments of Pathology.
Proliferations of neoplastic perivascular epithelioid cells (PECs) may occur within the lung and extrathoracic sites. The term "PEComatosis" is applied to multiple or diffuse microscopic proliferations of neoplastic PECs. Pulmonary diffuse PEComatosis is extremely rare, with only one case documented in the literature to date.
View Article and Find Full Text PDFDiagn Pathol
January 2025
Department of Diagnostic Pathology, National Cancer Center Hospital, 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045, Japan.
Background: Perivascular epithelioid cell tumors (PEComas) rarely appear in the head and neck region. This case report describes two transcription factor E3 (TFE3)-rearranged PEComa cases, consisting of one in the orbit and one in the nasal cavity.
Case Presentation: Both cases demonstrated sheet-like or focal nested architecture and comprised epithelioid cells with abundant clear to eosinophilic cytoplasm and vascular stroma.
Cardiovasc Pathol
January 2025
Department of Pathology and Applied Neurobiology, Kyoto Prefectural University of Medicine, Japan. Electronic address:
A rare autopsy case of malignant transcription factor E3 (TFE3)-rearranged perivascular epithelioid cell tumor (PEComa)-like neoplasm is presented. An 84-year-old woman manifested multiple cerebral infarctions and repetitive embolic events in the supra mesenchymal artery (SMA), and the presence of a mobile mass in the heart's left ventricle was also revealed. Tumoral lesions were also found in a pelvic space and a right pleural cavity, and a biopsy was performed from one of the disseminated tumor masses in the right pleura.
View Article and Find Full Text PDFCureus
December 2024
Pathology and Laboratory Medicine, Saint Michael's Medical Center, Newark, USA.
Perivascular epithelioid cell tumors (PEComas) are a rare group of mesenchymal neoplasms composed of perivascular epithelioid cells. While commonly found in the kidney, uterus, and soft tissues, PEComas of the liver are exceedingly rare. We present a case of a PEComa incidentally discovered in a 73-year-old female patient undergoing evaluation for abdominal pain.
View Article and Find Full Text PDFArch 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.
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