Pseudoxanthoma elasticum is a systemic metabolic disease presenting calcifications and progressive fragmentation of elastic fibers. Actually, no targeted therapies are available for the treatment; only prevention of complications is possible. Classically, pseudoxanthoma elasticum is a "benign" disease, without cancer association. Herein, we reported a singular association of pseudoxanthoma elasticum with breast carcinoma, describing the clinical management, in particular intra-operative treatment, focusing on intra-operative radiotherapy since no specific guidelines are available in literature.
Keywords: breast cancer; pseudoxanthoma elasticum; radiotherapy.
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