| Literature DB >> 28318774 |
Pierre-Marie Lavrut1, Philippe Paparel2, Myriam Decaussin-Petrucci3.
Abstract
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.Entities:
Keywords: Angiomyolipome épithélioïde; Epithelioid angiomyolipoma; Facteurs pronostiques; PEComa; PECome; Prognostic factors; Renal tumor; Tumeur rénale
Mesh:
Year: 2017 PMID: 28318774 DOI: 10.1016/j.annpat.2017.01.005
Source DB: PubMed Journal: Ann Pathol ISSN: 0242-6498 Impact factor: 0.407