- 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:
- Hornick JL, Pan C-C. PEComa. In: World Health Organization classification of tumours of soft tissue and bone, 4th, Fletcher CDM, Brodge JA, Hogendoorn PCW, Mertens F. (Eds), IARC, Lyon 2013. p.230.
- Martignoni G, Pea M, Reghellin D, et al. PEComas: the past, the present and the future. Virchows Arch 2008; 452:119.
- Hajdu SI, Foote FW Jr. Angiomyolipoma of the kidney: report of 27 cases and review of the literature. J Urol 1969; 102:396.
- Bissler JJ, Kingswood JC. Renal angiomyolipomata. Kidney Int 2004; 66:924.
- Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma. J Urol 2002; 168:1315.
- Lane BR, Aydin H, Danforth TL, et al. Clinical correlates of renal angiomyolipoma subtypes in 209 patients: classic, fat poor, tuberous sclerosis associated and epithelioid. J Urol 2008; 180:836.
- Halpenny D, Snow A, McNeill G, Torreggiani WC. The radiological diagnosis and treatment of renal angiomyolipoma-current status. Clin Radiol 2010; 65:99.
- Fujii Y, Ajima J, Oka K, et al. Benign renal tumors detected among healthy adults by abdominal ultrasonography. Eur Urol 1995; 27:124.
- Rule AD, Sasiwimonphan K, Lieske JC, et al. Characteristics of renal cystic and solid lesions based on contrast-enhanced computed tomography of potential kidney donors. Am J Kidney Dis 2012; 59:611.
- Osborne JP, Fryer A, Webb D. Epidemiology of tuberous sclerosis. Ann N Y Acad Sci 1991; 615:125.
- Seyam RM, Bissada NK, Kattan SA, et al. Changing trends in presentation, diagnosis and management of renal angiomyolipoma: comparison of sporadic and tuberous sclerosis complex-associated forms. Urology 2008; 72:1077.
- Martignoni G, Pea M, Rocca PC, Bonetti F. Renal pathology in the tuberous sclerosis complex. Pathology 2003; 35:505.
- Aydin H, Magi-Galluzzi C, Lane BR, et al. Renal angiomyolipoma: clinicopathologic study of 194 cases with emphasis on the epithelioid histology and tuberous sclerosis association. Am J Surg Pathol 2009; 33:289.
- Siroky BJ, Yin H, Dixon BP, et al. Evidence for pericyte origin of TSC-associated renal angiomyolipomas and implications for angiotensin receptor inhibition therapy. Am J Physiol Renal Physiol 2014; 307:F560.
- Bloom DA, Scardino PT, Ehrlich RM, Waisman J. The significance of lymph nodal involvement in renal angiomyolipoma. J Urol 1982; 128:1292.
- Hulbert JC, Graf R. Involvement of the spleen by renal angiomyolipoma: metastasis or multicentricity? J Urol 1983; 130:328.
- Amin MB. Epithelioid angiomyolipoma. In: World Health Organization Classification of Tumours Pathology and Genetics of Tumors of the Urinary System and Male Genital Organs, Eble J, Sauter G, Epstein J (Eds), IARC Press, Lyon 2004. p.68.
- Nese N, Martignoni G, Fletcher CD, et al. Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol 2011; 35:161.
- Lei JH, Liu LR, Wei Q, et al. A Four-Year Follow-up Study of Renal Epithelioid Angiomyolipoma: A Multi-Center Experience and Literature Review. Sci Rep 2015; 5:10030.
- Brimo F, Robinson B, Guo C, et al. Renal epithelioid angiomyolipoma with atypia: a series of 40 cases with emphasis on clinicopathologic prognostic indicators of malignancy. Am J Surg Pathol 2010; 34:715.
- Nagashima Y, Ohaki Y, Tanaka Y, et al. A case of renal angiomyolipomas associated with multiple and various hamartomatous microlesions. Virchows Arch A Pathol Anat Histopathol 1988; 413:177.
- Bernstein J, Robbins TO. Renal involvement in tuberous sclerosis. Ann N Y Acad Sci 1991; 615:36.
- Kiliçaslan I, Güllüoglu MG, Dogan O, Uysal V. Intraglomerular microlesions in renal angiomyolipoma. Hum Pathol 2000; 31:1325.
- Schieda N, Kielar AZ, Al Dandan O, et al. Ten uncommon and unusual variants of renal angiomyolipoma (AML): radiologic-pathologic correlation. Clin Radiol 2015; 70:206.
- Silverman SG, Gan YU, Mortele KJ, et al. Renal masses in the adult patient: the role of percutaneous biopsy. Radiology 2006; 240:6.
- Planché O, Correas JM, Mader B, et al. Prophylactic embolization of renal angiomyolipomas: evaluation of therapeutic response using CT 3D volume calculation and density histograms. J Vasc Interv Radiol 2011; 22:1388.
- Castle SM, Gorbatiy V, Ekwenna O, et al. Radiofrequency ablation (RFA) therapy for renal angiomyolipoma (AML): an alternative to angio-embolization and nephron-sparing surgery. BJU Int 2012; 109:384.
- Boorjian SA, Frank I, Inman B, et al. The role of partial nephrectomy for the management of sporadic renal angiomyolipoma. Urology 2007; 70:1064.
- Oesterling JE, Fishman EK, Goldman SM, Marshall FF. The management of renal angiomyolipoma. J Urol 1986; 135:1121.
- Dickinson M, Ruckle H, Beaghler M, Hadley HR. Renal angiomyolipoma: optimal treatment based on size and symptoms. Clin Nephrol 1998; 49:281.
- Harabayashi T, Shinohara N, Katano H, et al. Management of renal angiomyolipomas associated with tuberous sclerosis complex. J Urol 2004; 171:102.
- Sooriakumaran P, Gibbs P, Coughlin G, et al. Angiomyolipomata: challenges, solutions, and future prospects based on over 100 cases treated. BJU Int 2010; 105:101.
- Heidenreich A, Hegele A, Varga Z, et al. Nephron-sparing surgery for renal angiomyolipoma. Eur Urol 2002; 41:267.
- Lewis EL, Palmer JM. Renal angiomyolipoma and massive retroperitoneal hemorrhage during pregnancy. West J Med 1985; 143:675.
- Petrikovsky BM, Vintzileos AM, Cassidy SB, Egan JF. Tuberous sclerosis in pregnancy. Am J Perinatol 1990; 7:133.
- Raft J, Lalot JM, Meistelman C, Longrois D. [Renal angiomyolipoma rupture during pregnancy]. Gynecol Obstet Fertil 2006; 34:917.
- Zapardiel I, Delafuente-Valero J, Bajo-Arenas JM. Renal angiomyolipoma during pregnancy: review of the literature. Gynecol Obstet Invest 2011; 72:217.
- Gould Rothberg BE, Grooms MC, Dharnidharka VR. Rapid growth of a kidney angiomyolipoma after initiation of oral contraceptive therapy. Obstet Gynecol 2006; 108:734.
- Ryu JH, Moss J, Beck GJ, et al. The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment. Am J Respir Crit Care Med 2006; 173:105.
- Bernstein SM, Newell JD Jr, Adamczyk D, et al. How common are renal angiomyolipomas in patients with pulmonary lymphangiomyomatosis? Am J Respir Crit Care Med 1995; 152:2138.
- Blute ML. How common are renal angiomyolipomas in patients with pulmonary lymphangiomyomatosis? Am J Respir Crit Care Med 1996; 154:1580.
- Engels K, Stauber R, Magnussen H, et al. Angiomyolipomas are indicator lesions for sporadic lymphangioleiomyomatosis in women. Eur Urol 2009; 55:755.
- 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