Perivascular epithelioid cell (PEC) tumors are rare tumors of mesenchymal origin and can affect many anatomic regions. Although these tumors are usually benign, malignant variants exist. We document for the first time a malignant PEComa arising from the pleura of a 43-year-old Malay woman. The pathology of the tumor as well as differential diagnoses is discussed.
HornickJLPanCC. PEComa. In: FletcherCDMBridgeJAHogendoornPCWMertensF, eds. WHO Classification of Tumours of Soft Tissue and Bone. 4th ed.Lyon, France: IARC Press; 2013:230-231.
ZhaoJTengHZhaoR, et al. Malignant perivascular epithelioid cell tumor of the lung synchronous with a primary adenocarcinoma: one case report and review of the literature. BMC Cancer. 2019;19:235. doi:10.1186/s12885-019-5383-0
4.
LiangWXuSChenF.Malignant perivascular epithelioid cell neoplasm of the mediastinum and the lung: one case report. Medicine (Baltimore). 2015;94:e904. doi:10.1097/MD.0000000000000904
5.
NiuHWangFWZhangPJBingZ.Cardiac epithelioid PEComa: report of two cases and review of the literature. Case Rep Med. 2012;2012:521678. doi:10.1155/2012/521678
6.
BonettiFPeaMMartignoniGZamboniG.PEC and sugar. Am J Surg Pathol. 1992;16:307-308.
7.
AcostaAMAdleyBP.Predicting the behavior of perivascular epithelioid cell tumors of the uterine corpus. Arch Pathol Lab Med. 2017;141:463-469.
8.
ZhouYChenFJiangWMengQWangF.Hepatic epithelioid angiomyolipoma with an unusual pathologic appearance: expanding the morphologic spectrum. Int J Clin Exp Pathol. 2014;7:6364-6349.
9.
MaiKTBelangerEC.Perivascular epithelioid cell tumor (PEComa) of the soft tissue. Pathology. 2006;38:415-420.
10.
ConlonNSoslowRAMuraliR.Perivascular epithelioid tumours (PEComas) of the gynaecological tract. J Clin Pathol. 2015;68:418-426.
11.
ZhengGMartignoniGAntonescuC, et al. A broad survey of cathepsin K immunoreactivity in human neoplasms. Am J Clin Pathol. 2013;139:151-159.
12.
OrdóñezNG.Pleomorphic mesothelioma: report of 10 cases. Mod Pathol. 2012;25:1011-1022.
13.
MiettinenMMcCuePASarlomo-RikalaM, et al. GATA 3: a multispecific but potentially useful marker in surgical pathology: a systematic analysis of 2500 epithelial and nonepithelial tumors. Am J Surg Pathol. 2014;38:13-22.
14.
LewisJSRitterJHEl-MoftyS.Alternative epithelial markers in sarcomatoid carcinomas of head and neck, lung, and bladder-p63, MOC-31, and TTF-1. Mod Pathol. 2005;18:1471-1481.
15.
WangQChenJDassarathM, et al. Primary malignant melanoma of the pleura with rapid progression: a case report and literature review. Oncol Lett. 2015;9:2713-2715.
16.
FerencziKLastraRRFarkasT, et al. Mum-1 expression differentiates tumors in the PEComa family from clear cell sarcoma and melanoma. Int J Surg Pathol. 2012;20:29-36.
17.
SanfilippoRJonesRLBlayJY, et al. Role of chemotherapy, VEGFR inhibitors, and mTOR inhibitors in advanced perivascular epithelioid cell tumors (PEComas). Clin Cancer Res. 2019;25:5295-5300.
18.
SchoolmeesterJKDaoLNSukovWR, et al. TFE3 translocation–associated perivascular epithelioid cell neoplasm (PEComa) of the gynecologic tract: morphology, immunophenotype, differential diagnosis. Am J Surg Pathol. 2015;39:394-404.
