Abstract
Angiomyolipomas (AML) are benign mesenchymal tumours. It is commonly seen in the kidney. Extra-renal sites of AML are uncommon though it can occur in any part of the body. Reports of cutaneous AML are rare in the literature. We present a case of an 11-year-old female with a polypoidal swelling on the nose that was noticed a few weeks after birth, which progressively increased in size till it was causing nasal blockage. This cutaneous lesion is different from a renal angiomyolipoma because it lacks features of tuberous sclerosis, non-invasive, absence of epithelioid cells and negative HMB-45 staining. Histopathology shows a mixture of blood vessels, mature adipocytes and smooth muscle. These unique features distinguish this lesion from other possible differentials such as angiomyoma and myolipoma. Histopathology and immunohistochemistry confirm the swelling as angiomyolipoma of the nose. We concluded that histopathology and immunohistochemistry are important diagnostic tools for cutaneous AML irrespective of gender, age of onset and site of lesion.
Highlights
Angiomyolipomas (AML) are benign mesenchymal tumours with varying proportions of matured fat cells, thick walled blood vessels and smooth muscle cells [1]
We present a case of an 11-year-old female with a polypoidal swelling on the nose that was noticed a few weeks after birth, which progressively increased in size till it was causing nasal blockage. This cutaneous lesion is different from a renal angiomyolipoma because it lacks features of tuberous sclerosis, non-invasive, absence of epithelioid cells and negative human melanoma black (HMB)-45 staining
AML are one of the most common benign tumors in the kidney [3], and over 33% of patients with renal AML are associated with tuberous sclerosis complex (TSC)
Summary
Angiomyolipomas (AML) are benign mesenchymal tumours with varying proportions of matured fat cells, thick walled blood vessels and smooth muscle cells [1]. Cutaneous AML unlike renal type predominantly affects male; is solitary, non-invasive, and negative for human melanoma black (HMB)-45 antibody and is not associated with tuberous sclerosis. They are mostly within 1 - 4 cm in dimension. Cutaneous AML has been suggested to develop from replacement of degenerated tissue of neglected lesion with fat tissue following repeated trauma [4]. The purpose of this case report is to describe a rare case of large cutaneous AML located on the nose of a female child. This is the first case report of cutaneous AML in Nigeria
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