Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms, and liver PEComas are extremely rare. They are usually discovered incidentally, and diagnostic pitfalls are frequent owing to similar imaging characteristics to other liver neoplasms. We present a patient whose evaluation was consistent with a right-sided hepatocellular carcinoma for which right hepatectomy was performed. Based on the final histopathological examination and immunohistochemistry, a diagnosis of PEComa was made. Immunohistochemistry plays a crucial role in arriving at the diagnosis, and resection represents the standard of care. A long-term follow-up is recommended because the natural history of PEComas is unpredictable.
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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.
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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.
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