Perivascular epithelioid cell tumors are soft tissue tumors that can occur in various locations in the body whose incidence is rising. Hepatic PEComas are quite rare and diagnosis involves positivity of Melan-A and HMB45 on immunohistochemistry. Usual treatment is surgery for benign tumors and chemotherapy including mTOR inhibitors for malignant tumors. Here we discuss the radiological and path...

Purpose. Perivascular epithelioid cell tumors (PEComas) are a rare collection of tumors characterized by a myomelanocytic phenotype, and PEComas occurring in "nonclassic" anatomic distributions are known as perivascular epithelioid cell tumor not otherwise specified (PEComa-NOS). This review aims to compile and analyze cases of PEComa-NOS in an effort to better define their natural history. Des...

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...

PEComas localized in the region of falciform ligament and broad ligament are exceedingly rare. Most of them are built of spindle neoplastic cells. We report a case of epithelioid PEComa of the falciform ligament and/or broad ligament. There is only one report of such neoplasm in English-language literature. Histologically, the tumor was composed of nests of epithelioid clear cells stained posit...

BACKGROUND We present a case of perivascular epithelioid cell tumor (PEComa), which clinically and histologically mimics a gastrointestinal stromal tumor (GIST). CASE PRESENTATION A 42-year-old woman was found to have a mass in the left flank during her annual medical checkup. Computed tomography examination revealed a submucosal tumor of the descending colon. Surgeons and radiologists suspec...

Hemangioblastoma is a rare benign tumor; common by found in the central nervous system, about 25% is associated with Von Hippel-Lindau (VHL) disease. Renal hemangioblastoma is extremely rare. The leiomyoma generally occurs in organs with abundant of smooth muscle such as uterus, gastrointestinal tract or subcutaneous soft tissue. It’s unusual to see it in the kidney, while the renal epithelioid...

A perivascular epithelioid cell (PEC) tumor is a rare mesenchymal tumor characterized by abundant cytoplasmic Periodic acid-Schiff positive glycogen (also called sugar tumor or clear cell tumor of the lung for this characteristic) and is mostly benign. We report a case of a 63-year-old man who presented with an enlarging mass on chest radiograph. After a thorough workup, diagnosis of malignant ...