Cutaneous angiomyolipoma.

Sir,

We are grateful for the careful review of our article by Okoń K et al.[12] Although the triphasic nature of AML led us call this lesion “hamartoma,” current clonality studies support their classification as neoplasms. Gill et al., defined angiomyolipoma as a benign clonal neoplasm composed of thick-walled blood vessels, smooth muscle cells, and adipose tissue, belonging to the family of perivascular epithelioid cell tumors (PEComa).[3] none However, AMLs are often referred to as hamartomas-a benign tumor-like growth composed of typical cells and tissues found in the area of the body where it occurs, but growing in an unorganized fashion.[45]

We do not claim that the extrarenal AML constitutes a separated entity from renal AML, but it differs in sex predominance, clinical associations, circumscription, solitariness, and HMB-45 immunoreactivity. It is difficult to consider urachal remnant as a differential diagnosis in this case considering its clinical, gross, and microscopic findings. The diagnosis was offered considering its classical, triphasic, traditional, histological criteria. We agree with the reviewers that analysis of TSC1 and TSC2 genes would help in establishing the diagnosis. However, there are currently no data about their status in AML located in the skin.