Epitheloides Angiomyolipom der Niere nach stattgehabtem malignem Melanom

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. OBJE...

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Detalles Bibliográficos
Autores principales: Rothe, H., Gaber, A., Dittrich, B., Nagel, M., Tuffaha, M., Hoschke, B.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Springer Medizin 2021
Materias:
Kasuistiken
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8831349/
https://www.ncbi.nlm.nih.gov/pubmed/34605930
http://dx.doi.org/10.1007/s00120-021-01662-x
Descripción
Sumario: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.

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