BACKGROUND Perivascular epithelioid cell tumor (PEComa), other than angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangioleiomyomatosis (LAM), is a very rare mesenchymal tumor with an unpredictable natural history. The uterus is the most prevalent reported site of involvement of PEComa-not otherwise specified (PEComa-NOS). To the best of our knowledge, about 100 PEComa-NOS have be...

Perivascular epithelioid cell (PEC) is a unique cell which expresses both myogenic and melanocytic markers, and forms PEComa. A 36-year-old woman presented with a 35 mm-diameter liver tumor. MRI showed poor fat component in the tumor. Contrast-enhanced ultrasonography using the newly developed enhancing reagent, Sonazoid, clearly demonstrated early-phase enhancement of the tumor and rapid drain...

The first case of cutaneous clear cell perivascular epithelioid cell tumor (PEComa) with negative HMB-45 marker is presented. The tumor was a nodule 3x2 cm in size, located on the right foot in a 60-year-old man. The lesion consisted of large irregularly shaped cells with clear cytoplasm, negative for S-100 protein, HMB-45, Melan-A, pancytokeratin, epithelial membrane antigen and CAM5.2. Multif...

Perivascular epithelioid cell tumor (PEComa) is a rare neoplasm considered to arise from myomelanocytic cell lineage. The uterus is reportedly the most common site to be involved. Orbital PEComa is extremely rare with only two cases reported till date. A 5-year-old male presented with a right medial orbital mass for the last 6 months. The patient was diagnosed with alveolar soft part sarcoma el...

Benign perivascular epithelioid cell tumor (PEComa) of the lung is a rare benign neoplasm, a sclerosing variant of which is even rarer. We present a case of 51-year-old man who was diagnosed with benign sclerosing PEComa by percutaneous fine needle aspiration cytology and biopsy. The aspirate revealed a few cell clusters composed of bland-looking polygonal or spindle cells with fine granular or...

Perivascular epithelioid cell neoplasm/tumor (PEComa) was a relatively uncommon lesion of mesenchymal origin characterized by perivascular epithelioid cells with distinctive histological and immunohistochemical features. The occurrence in the sacral ligament was exceptional rare. Cytokeratin and epithelial membrane antigen (EMA) were considered as commonly useful epithelial markers to distingui...

Primary perivascular epithelioid cell tumor (PEComa) of the liver is a very rare tumor that originates from mesenchyma. Gastrointestinal tract with perivascular distribution is the most common anatomic sites of these tumors. Only few cases of hepatic PEComa have been described so far. Malignant PEComas exhibit aggressive behavior with poor prognosis, making early diagnosis crucial. Hereby, we r...

Perivascular epithelioid cell tumors (PEComa) are tumors of perivascular epithelioid cells with immunohistochemical features of smooth muscle and melanocytic tumors. The PEComa of the gastrointestinal tract is rare. The treatment is surgical, although there are data that suggest a good response to rapamycin.

Perivascular epithelioid cell tumors (PEComas) are mesenchymal tumors containing variable component of smooth muscles, fat and vessels. They occured pretty rare in the liver and no characteristic of imaging data have been demonstrated up to now. We herein present a case of a 58-year-old man with a hepatic PEComa which was correct diagnosed through immunohistochemical confirmation with HMB-45. T...

Purpose. Tumors of the perivascular epithelial cell tumor (PEComa), first described in 1992, represent a rare soft tissue neoplasm of varying malignant potential. Cases of PEComa have been previously described in a few somatic and visceral sites, most notably in the gastrointestinal tract, genitourinary tract, and one extremity case in the thigh. To date, most malignant cases of PEComa have bee...