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Background/aim: Lymphangioleiomyomatosis (LAM) belongs to the perivascular epithelioid cell tumor (PEComa) family. The relationship between LAM and tuberous sclerosis complex (TSC) is of particular concern in a subset of women with clinically occult LAM involving the pelvic lymph nodes. This study aimed to investigate the clinicopathological features of incidental nodal LAM detected during the surgical staging of gynecological tumors.

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Aims: The hepatic perivascular epithelioid cell tumour (PEComa), including angiomyolipoma, exhibits diverse morphology and clinical behaviour; however, its prognostic features remain undefined. This study aimed to investigate its histological features and prognostic factors.

Methods And Results: In total, 132 patients were included.

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Uterine mesenchymal tumors are a diverse group of tumors that can display a broad range of morphologic, immunohistochemical, and molecular profiles and are associated with varied clinical behaviors. In recent years, they have increasingly been classified by their underlying molecular alterations, leading to a more precise separation of diagnostic entities. As their diagnostic criteria have been refined, so too have the features that can be used to predict clinical outcomes.

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Intraspinal and Intracranial Melanotic Perivascular Epithelioid Cell Tumor.

Radiology

December 2024

From the Department of Medical Imaging, University Hospitals Leuven, Leuven, Belgium (B.V.B., J.D.); and Department of Medical Imaging, Jessa Ziekenhuis, Stadsomvaart 11, 3500 Hasselt, Belgium (B.V.B.).

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Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms, commonly found in the uterus and retroperitoneum. Renal PEComas are exceedingly rare, often posing diagnostic challenges due to their resemblance to renal cell carcinoma (RCC) on imaging. We present the case of an 18-year-old male who presented with non-specific symptoms of fever, chills, and shivering.

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