Primary perivascular epithelioid cell tumor (PEComa) in bone: A review of the literature and a case arising in the humerus with multiple metastases

Abstract

Highlights First case PEComa primary arising in humerus was described. Histology is the basic to determine malignancy of PEComa. TFE3 gene investigation is the key of therapy selection. mTOR inhibitor is believed to be effective for patients without TFE3 rearrangement. More study is needed to understand the role of molecular test and imaging in PEComa.

Perirenal Perivascular Epithelioid Cell Tumor (PEcoma) with Pulmonary Micro Invasive Adenocarcinoma: A Case Report and Literature Review

“…Since the first case of PEComa reported in 1996, cases involving various anatomic locations have been reported, such as gastrointestinal tract, 4 kidney, 12 pancreas, 13 liver, 14 ovary, 15 uterus 5 and so on. 16 , 17 According to the recent systematic reviews, 10 , 15 the most commonly affected organs were uterus (including uterine and cervix uteri) and colon, with approximately 65 and 20 cases reported online, respectively. Perirenal PEcoma are extremely rare, to date, only four cases have been reported and our case is the fifth one ( Table 1 ).…”

Perivascular epithelioid cell tumour-mimicking retroperitoneal leiomyosarcoma

“…Perivascular epithelioid cell tumour (PEComa) is a rare mesenchymal tumour and is defined by the presence of perivascular epithelioid cells coexpressing both myogenic markers (actin, desmin, myosin, calponin) and melanocytic markers (Human Melanoma Black-45 (HMB-45), Melan A (Mart 1), Mitf) 1. PEComas occur in a variety of visceral, cutaneous and soft tissue sites throughout the body 2–6. We report a rare case of a retroperitoneal PEComa mimicking a leiomyosarcoma.…”

Pregnancy-Associated Perivascular Epithelioid Cell Tumor of the Tarsal Navicular

“…erivascular epithelioid cell tumors (PEComas) are a group of mesenchymal neoplasms including angiomyolipoma (AML), lymphangiomyomatosis (LAM), clear cell "sugar" tumor of the lung, and other rarer tumors that share the distinctive perivascular epithelioid cell (PEC) type. The family of lesions is composed of large perivascular epitheloid and spindle cells arranged in a nested pattern around thin vessels [1][2][3] . The tumors exhibit immunoreactivity for melanocytic and smooth muscle markers 1,2 .…”

“…The family of lesions is composed of large perivascular epitheloid and spindle cells arranged in a nested pattern around thin vessels [1][2][3] . The tumors exhibit immunoreactivity for melanocytic and smooth muscle markers 1,2 . Most PEComas are benign lesions with relatively good prognosis, but a minority of them have malignant potential 4 .…”

“…PEComas are most commonly found in the kidney, liver, uterus, or gynecologic tract 5,6 . Limited cases of primary PEComa of the bone have been reported in the literature 1,[6][7][8][9] . Although the reported cases of primary bone PEComa have an equal sex distribution, PEComas typically have a predilection for females of reproductive age 1,5 .…”