- Vicente E Torres, MD
Vicente E Torres, MD
- Professor of Medicine
- Mayo Clinic College of Medicine
- William M Bennett, MD
William M Bennett, MD
- Medical Director
- Legacy Good Samaritan Transplant Services
Angiomyolipomas (AMLs) belong to a family of tumors collectively referred to as neoplasms with perivascular epithelioid differentiation, which were previously called perivascular epithelioid cell tumors and are now referred to as PEComas . These tumors arise by clonal proliferation of epithelioid cells distributed around blood vessels .
Renal AMLs may occur in association with tuberous sclerosis complex (TSC) or pulmonary lymphangioleiomyomatosis (LAM), or occur as a sporadic finding among patients who have neither TSC nor pulmonary LAM.
This topic reviews sporadic renal AMLs that occur among patients who do not have TSC and renal AMLS that occur among patients with sporadic pulmonary LAM. Renal AMLs that occur in association with TSC are discussed elsewhere. (See "Renal manifestations of tuberous sclerosis complex", section on 'Angiomyolipomas'.)
SPORADIC RENAL ANGIOMYOLIPOMAS
Epidemiology — Renal AMLs are found in approximately 0.3 to 2.1 percent of kidneys at routine autopsy  and, due to both an increase in the use of imaging and advances in imaging technology, are increasingly identified in the general population as an incidental finding [4-9].
The following findings are from studies that evaluated the frequency of renal AMLs in different groups and used different radiologic techniques:To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:
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- SPORADIC RENAL ANGIOMYOLIPOMAS
- Clinical features
- - Classic variant
- - Epithelioid variant
- Malignant renal epithelioid angiomyolipomas
- - Intraglomerular lesions
- Differential diagnosis
- Identification of patients with tuberous sclerosis complex
- Treatment and surveillance
- - Potential issues in women
- RENAL ANGIOMYOLIPOMAS IN SPORADIC PULMONARY LYMPHANGIOLEIOMYOMATOSIS
- SUMMARY AND RECOMMENDATIONS