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 tumors (PEComas) are a family of rare, poorly defined mesenchymal tumors of uncertain malignant potential.•Treatment for PEComas has most commonly involved excisional biopsy or surgical resection.•The use of mTOR inhibitors may provide the best medical treatment as well as a fertility-sparing treatment option.

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...

An intranasal glomus tumor and a sinonasal hemangiopericytoma-like tumor are reported. Both patients were elderly women suffering from nasal bleeding, and presented with a polypoid mass arising in the nasal septum. Microscopically, the glomus tumor displayed a proliferation of uniform rounded or cuboidal epithelioid cells arranged in sheets and interrupted by a rich vasculature with a character...

Perivascular epithelioid cell tumors (PEComas) represent a rare group of tumours with uncertain malignancy potential exhibiting an immunophenotype characterized by actin and Human Melanoma Black 45 (HMB45) immunoreactivity. Our case concerns about a rare malignant uterine perivascular epithelioid cell tumour diagnosed in a patient underwent to subtotal hysterectomy with unclear diagnosis, 12 ye...

Perivascular epithelioid cell tumors (PEComas) are a family of rare mesenchymal neoplasms. The PEComas, composed of epithelioid and spindle cells, have the same cellular and immunohistochemical features but are found in different visceral and soft tissue sites. Here, we report the histological and immunohistochemical features of one case of PEComa restricted in the pelvic visceral peritoneum of...

Address: 1Department of Pathology, Yale University School of Medicine, New Haven, CT, USA, 2Department of Laboratory Medicine Yale University School of Medicine, New Haven, CT, USA, 3Department of Genetics, Yale University School of Medicine, New Haven, CT, USA, 4Department of Pathology, Hospital of St Raphael, New Haven, CT, USA, 5Department of Pediatrics, Louisiana State University Health Sci...

Perivascular epithelioid cell neoplasms are a group of rare tumours reported in various organs under a variety of designations. Such tumours are of interest primarily because of the distinctive morphology of their cell population and their immunoreactivity with melanocytic and myoid markers. There is a strong association between perivascular epithelioid cell neoplasms and tuberous sclerosis com...

A 7-year-old boy presented with hematochezia and abdominal pain. A 3.7-cm-sized mass was identified in the ascending colon by abdominal computed tomography and colonoscopy. The patient underwent surgical resection. Pathological examination revealed a low-grade perivascular epithelioid cell tumor (PEComa). PEComa in the colon is very rare. Only a few cases have been reported so far. An effective...

In recent years, a group of tumors that have been designated "perivascular epithelioid cell tumors" (PEComa) have been reported with increasing frequency from a wide variety of anatomic locations. The uterus and retroperitoneum appear to be the most frequent sites of origin for these lesions. PEComas belong to an identically named family of tumors comprised of conventional angiomyolipomas, clea...