[Epithelioid angiomyolipoma of the kidney: About one case and malignant features evaluation].
Renal epithelioid angiomyolipoma (E-AML) is a rare mesenchymal tumor of the kidney included in the family of tumor with perivascular epithelioid cell differentiation (PEComas) and is frequently associated with tuberous sclerosis complex. Since its clinical and radiological features are not specific, the diagnosis remained mostly pathological. Microscopically, E-AML demonstrate proliferation of more than 80% of epithelioid cells with atypia, often associated with necrosis, hemorrhage, mitotic activity and vascular invasion. Tumor's cells are immunohistochemically positive for melanocytic and smooth muscle markers. The pathologic prognostic predictors are not well known and the malignancy remained based on the identification of distant metastasis. We herein report a case of E-AML diagnosed in a 58-year-old woman and review analysis in the literature to discuss the prognostic indicators of malignancy useful for risk stratification of E-AML and to manage the patients.
Service d'anatomie et cytologie pathologiques, hospices civils de Lyon, CHU Lyon Sud, université Lyon 1, chemin du Grand-Revoyet, 69495 Pierre-Bénite cedex, France.
Pierre-Marie
PM
Lavrut
Service d'urologie, hospices civils de Lyon, CHU Lyon Sud, université Lyon 1, chemin du Grand-Revoyet, 69495 Pierre-Bénite cedex, France.
Philippe
P
Paparel
Service d'anatomie et cytologie pathologiques, hospices civils de Lyon, CHU Lyon Sud, université Lyon 1, chemin du Grand-Revoyet, 69495 Pierre-Bénite cedex, France. Electronic address: myriam.decaussin-petrucci@chu-lyon.fr.
Myriam
M
Decaussin-Petrucci
D018207:Angiomyolipoma / Q000473:pathology*; D015622:Epithelioid Cells; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000473:pathology*; D008875:Middle Aged
Angiomyolipome épithélioïde; Epithelioid angiomyolipoma; Facteurs pronostiques; PEComa; PECome; Prognostic factors; Renal tumor; Tumeur rénale