RESUMO
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.
Assuntos
Angiomiolipoma , Neoplasias Renais , Melanoma , Segunda Neoplasia Primária , Angiomiolipoma/diagnóstico por imagem , Angiomiolipoma/cirurgia , Humanos , Rim , Neoplasias Renais/diagnóstico por imagem , Neoplasias Renais/cirurgia , Melanoma/diagnóstico por imagemRESUMO
It is reported on a more infrequent or too little regarded clinical picture within the form circle of the disseminated intravasal coagulation disturbances, the microangiopathic haemolytic anaemia (MHA). Diagnostically heuristic is the clinical triad haemolytic anaemia, thrombocytopenia and haemorrhagic diathesis. The microangiopathic haemolytic anaemia is described on the basis of a casuistics, the present knowledge about pathogenesis and therapy are discussed.