Epithelioid angiomyolipoma of the kidney after successfully treated malignant melanoma

BACKGROUND Therapy of epitheloid angiomyolipomas (eAML) may be challenging, since unlike classical angiomyolipomas this rare subclass of benign mesenchymal angiomyolipomas may present with lymph node metastases, local recurrent disease, and/or systemic metastatic disease in up to 30% of cases. OBJECTIVES We report here for the first time in Germany a case of eAML after successful treatment of malignant melanoma. MATERIALS AND METHODS Clinical and histological findings as well as results of the genetic analysis of the angiomyolipoma are presented. RESULTS A somatic, truncating mutation of the TSC2 gene was found in the angiomyolipoma. CONCLUSION The relationship to histologically similar tumor entities are presented and therapeutic options based on the genetic classification are discussed.