Angiomyolipoma (AML) is a type of tumor in the perivascular epithelioid cell neoplasm (PEComa) family. Epithelioid angiomyolipoma (EAML), having malignant potential, is considered a rare variant of angiomyolipoma. The most common site of EAMLs is kidney, and extra-renal EAMLs are very uncommon, with liver being the most common site.1 Other locations including retroperitoneum, uterus, liver, lun...

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed of immunohistochemically and histologically distinctive perivascular epithelioid cells. Here, we report on the computed tomography (CT) and magnetic resonance imaging (MRI) findings of ovarian PEComa with pulmonary metastasis. The tumor was visible as a multilocular hemorrhagic mass that encased the ovarian vessel...

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...

BACKGROUND Perivascular epithelioid cell tumors (PEComas) are a rare group of neoplasms composed of epithelioid cells that express both melanocytic and myoid markers. When considering PEComas of the female genital tract, the uterus is the most common location. Involvement of the ovary in the context of a primary uterine PEComa, in the absence of systemic disease associated with tuberous scleros...

Uterine perivascular epithelioid cell tumor is a rare mesenchymal consisting of histologically and immunohistochemically distinctive cells. These tumors’ being rare, having different morphological features similar immunohistochemical expression findings to that some tumors lead diagnostic difficulties misdiagnoses. In the present case report, we aimed discuss traps fell into while diagnosing cu...

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Perivascular epithelioid cell tumor (PEComa) is a rare and recently described neoplasm comp...

INTRODUCTION Perivascular epithelioid cell tumors are a family of rare mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. They can originate in any visceral organ or soft tissue and include a range of lesions such as angiomyolipoma, clear cell 'sugar' tumor of the lung, lymphangioleiomyomatosis and clear cell myomelanocytic tumors...