Definition
Angiomyolipoma is the most common mesenchymal neoplasm of the kidney, classically composed by a variable mixture of adipocytes, smooth muscle cells, and abnormal thick-wall blood vessels. The perivascular epithelioid cell is considered to be the cell of origin for this and other related tumors.
Clinical Features
Angiomyolipoma is a benign neoplasm and can be sporadic or occurs in patients with tuberous sclerosis (Eble 1998).
Incidence
Angiomyolipoma accounts for approximately 1% of surgically removed renal tumors.
Age
Sporadic angiomyolipoma occurs in the fourth to sixth decades of life, whereas tuberous sclerosis in the third and fourth decades of life.
Sex
Sporadic angiomyolipoma has a female predominance; in patients with tuberous sclerosis, there is no gender predilection.
Site
There is no site predilection in the sporadic angiomyolipoma, whereas those occurring in patients with tuberous sclerosis are usually bilateral, small, and multifocal.
Treatment
Partial...
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References and Further Reading
Eble, J. N. (1998). Angiomyolipoma of kidney. Seminars in Diagnostic Pathology, 15, 21–40.
Martignoni, G., Bonetti, F., Chilosi, M., et al. (2012). Cathepsin K expression in the spectrum of perivascular epithelioid cell (PEC) lesions of the kidney. Modern Pathology, 25, 100–111.
Martignoni, G., Pea, M., Zampini, C., et al. (2015). PEComas of the kidney and of the genitourinary tract. Seminars in Diagnostic Pathology, 32, 140–159.
Pea, M., Bonetti, F., Zamboni, G., et al. (1991). Melanocyte-marker-HMB-45 is regularly expressed in angiomyolipoma of the kidney. Pathology, 23, 185–188.
Qin, W., Bajaj, V., Malinowska, I., et al. (2011). Angiomyolipoma have common mutations in TSC2 but no other common genetic events. PLoS One, 6, e24919.
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Caliò, A., Segala, D., Martignoni, G. (2019). Angiomyolipoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_3777-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_3777-1
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