PEComas are a collection of generally rare tumors, defined by the World Health Organization as 'mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epitheloid cells'. We describe the case of retroperitoneal PEComa with a liposarcoma-like appearance on cross-sectional imaging, but distinctive immunohistochemistry revealing the correct diagnosis.
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| http://dx.doi.org/10.1186/1477-7819-12-3 | DOI Listing |
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Adrenal PEComa Treated by Surgical Resection and Postoperative Radiotherapy: A Case Report.
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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.
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World J Surg Oncol
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Department of Surgical Oncology, Institute of Medical Sciences, Banaras Hindu University, Varanasi, India.
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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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