2020 Edition
| Editors: Maria Rosaria Raspollini, Antonio Lopez-Beltran


  • Anna Caliò
  • Diego Segala
  • Guido MartignoniEmail author
Reference work entry
DOI: https://doi.org/10.1007/978-3-030-41894-6_3777


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


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References and Further Reading

  1. Eble, J. N. (1998). Angiomyolipoma of kidney. Seminars in Diagnostic Pathology, 15, 21–40.PubMedGoogle Scholar
  2. 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.CrossRefGoogle Scholar
  3. 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.CrossRefGoogle Scholar
  4. 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.CrossRefGoogle Scholar
  5. 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.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of Diagnostic and Public Health, Section of PathologyUniversity of VeronaVeronaItaly
  2. 2.Department of PathologyPederzoli HospitalPeschiera del GardaItaly