Perivascular epitheloid cell tumors (PEComas) are a family of neoplasms with distinctive perivascular epitheloid cells. 36 year old female patient admitted to our clinic with a mass on neck. Biopsy was reported as PEComa. PEComas are very rare, especially in neck region. Here, we present the second case in literature.
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http://dx.doi.org/10.1007/s12070-023-03918-8 | DOI Listing |
Am J Case Rep
December 2024
Department of Pathology, Eskişehir City Hospital, Eskişehir, Turkey.
BACKGROUND Perivascular epitheloid cell neoplasm (PEComa) is a rare mesenchymal tumor that is evaluated in the same tumor family as angiomyolipoma, sugar cell tumor of the lung, and lymphangioleiomyomatosis. Immunohistochemically, the disease can express melanocytic and myogenic markers, such as HMB45, HMSA1, MelanA/Mart1, and actin. The disease can be seen in almost every organ, especially the uterus and retroperitoneum.
View Article and Find Full Text PDFIndian J Otolaryngol Head Neck Surg
December 2023
Pathology Department, Istanbul University-Cerrahpaşa, Istanbul, Turkey.
Perivascular epitheloid cell tumors (PEComas) are a family of neoplasms with distinctive perivascular epitheloid cells. 36 year old female patient admitted to our clinic with a mass on neck. Biopsy was reported as PEComa.
View Article and Find Full Text PDFWorld J Surg Oncol
March 2022
Department of Surgical Oncology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India.
Background: Perivascular epitheloid cell tumor (PEComas) are characterized by expression of both muscles, most often smooth muscle actin (in ~80% of cases) and melanocytic markers (mainly HMB-45 and Melan A). TFE 3-associated PEComas are new variant which are poorly defined due to their limited reports in literature. These tumors lack response to targeted mTOR inhibitor therapy due to lack of mutation in TSC gene.
View Article and Find Full Text PDFClin J Gastroenterol
April 2022
Department of Radiology, Tata Medical Center, Kolkata, India.
Perivascular epithelioid cell tumors (PEComas) are a group of tumours of mesenchymal origin having a characteristic pathological presence of the epitheloid cell around blood vessels. They are uncommon tumours and hence their exact etiology and pathogenesis remain unclear. They can occur at any part of the body but the common sites of involvement are the gastrointestinal system and the genitourinary system.
View Article and Find Full Text PDFJ Med Genet
September 2022
MTA-SE Hereditary Tumors Research Group, Eötvös Loránd Research Network, Budapest, Hungary.
Gorlin-Goltz syndrome (GGS) or nevoid basal cell carcinoma syndrome is a rare tumour-overgrowth syndrome associated with multiple developmental anomalies and a wide variety of tumours. Here, we describe a case of a man aged 23 years with GGS with bilateral giant tumours adjacent to both adrenals that raised the suspicion of malignancy on imaging. Histological analysis of both surgically resected tumours revealed perivascular epitheloid cell tumours (PEComas) that were independent of the adrenals.
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