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.
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http://dx.doi.org/10.1155/2015/258140 | DOI Listing |
SAGE Open Med Case Rep
January 2025
Department of Hepatobiliary Pancreatic and Splenic Surgery Ward Ⅰ, The Affiliated ChuZhou Hospital of Anhui Medical University (The First People's Hospital of Chuzhou), ChuZhou, China.
Hepatic epithelioid angiomyolipoma is a rare mesenchymal liver tumor within the perivascular epithelioid cell tumor family, known for its uncertain malignancy and potential for aggressive behavior. Clinical presentation, laboratory findings, and imaging results lack specificity, making pathological examination critical for definitive diagnosis. This case report presents a 61-year-old male patient with a single tumor in the right liver lobe, measuring approximately 101 mm × 99 mm.
View Article and Find Full Text PDFAm 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.
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