Here, we describe an unusual mesenchymal tumor of the appendix with morphologic and immunohistochemical features of transcription factor E3 (TFE3)-positive, benign perivascular epithelioid cell tumor (PEComa) presenting as acute appendicitis.
Perivascular epithelioid cell tumor of the nasal cavity with TFE3 expression.
Perivascular epithelioid cell tumor of the sigmoid colon with transcription factor E3 expression [published online ahead of print May 3, 2011].
Perivascular epithelioid cell tumor (PEComa) is a mesenchymal tumor with perivascular epithelioid clear cell that coexpresses melanocytic and muscle markers.
The concept of a perivascular epithelioid cell tumor was first proposed by Bonetti et al (3) in 1992.
The TFE3 protein has been reported regularly in alveolar soft part sarcoma, which is known to harbor the TFE3 gene translocation and, more recently, in isolated examples of perivascular epithelioid cell tumor
Perivascular epithelioid cell tumor ('PEComa') of the uterus: a subset of HMB-45-positive epithelioid mesenchymal neoplasms with an uncertain relationship to pure smooth muscle tumors.
Malignant perivascular epithelioid cell tumor involving the prostate.
Perivascular epithelioid cell tumor
(PEComa) is a mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells.
The World Health Organization defines perivascular epithelioid cell tumors (PEComas) as mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells (PECs).
Perivascular epithelioid cell tumor can be distinguished from paraganglioma in that the former is negative for chromogranin A, synaptophysin, and S100 protein, and the latter shows more organoid growth.
Angiomyolipomas (AMLs) belong to the broader group of neoplasms known as perivascular epithelioid cell tumors