Article

Embolization of Renal Angiomyolipomata in Patients With Tuberous Sclerosis Complex

Division of Radiology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, Cincinnati, OH, USA.
American Journal of Kidney Diseases (Impact Factor: 5.9). 02/2006; 47(1):95-102. DOI: 10.1053/j.ajkd.2005.09.028
Source: PubMed

ABSTRACT

Renal angiomyolipomata can reduce renal reserve and lead to renal insufficiency and failure. Angiomyolipomata often have abnormal vasculature, with aneurysms that can hemorrhage. Treatment of angiomyolipomata greater than 4 cm in diameter is suggested to decrease the risk for hemorrhage. Nephron-sparing procedures are critical in patients because of their limited renal reserve. Embolization has been used to treat these tumors, but there are limited studies examining efficacy. Our study examines the efficacy of selective embolization in decreasing tumor burden, preventing hemorrhage, and preserving renal function.
We conducted a retrospective study of 16 patients with 20 angiomyolipomata on 18 kidneys who underwent 18 transcatheter transarterial embolization procedures. Aneurysm number and size were documented and tumor volumes were measured before and after embolization. Preprocedure and follow-up renal function also were measured. Changes in angiomyolipoma volume and kidney function were assessed for significance by using paired t-test.
Before embolization, 7 angiomyolipomata had more than 5 aneurysms, 9 had 1 to 5 aneurysms, and 4 had no aneurysms, but showed tortuous dysmorphic arteries. Mean aneurysm size was 5 mm. In patients available for follow-up, 15 of 16 tumors had decreased in volume (mean decrease, 56.1%; P = 0.001). At an average of 40 months' follow-up, there have been no subsequent hemorrhages. Patients' decline in renal function was not significantly different from that expected because of the natural course of the disease.
Selective embolization decreases tumor size, prevents hemorrhage, and preserves kidney function in patients with tuberous sclerosis with renal angiomyolipomata.

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    • "surgery Mean follow-up (months) Han, 1997 [98] 14 7.1 Alcohol + lipiodol, alcohol alone, coils + microparticles + gel foam 0 110 (40—350) 0 100 Abscess (drainage, n = 1) 14.3 0 7.1 > 12 months in 12 pts Harabayashi, 2004 [33] 12 100 Alcohol + coils 0 ND 0 ND Renal atrophy (n = 1) 50 0 0 60 (12—170) Ewalt, 2005 [37] 16 100 Microparticles 18.8 ND (40—210) 0 68.8 Pneumonia (n = 1) 0 0 0 ND Khotary, 2005 [74] 19 52.6 Alcohol + lipiodol 36.8 ND 0 100 0 31.6 a 5.3 0 51.5 (6—132) William, 2006 [82] 16 100 Microparticles ± coils 37.5 ND b 0 6.2 0 0 0 0 40 (6—62) Dabbeche, 2006 [75] 37 10.8 c Microparticles ± coils ± Alcohol 43.2 78 (45—180)/53 (40—110) d 8.1 16.2 Arterial breach (nephrectomy, n = 1), arterial dissection (stent, n = 1) 10.8 2.7 e 32.4 (50/21.1) d 21 Lenton, 2008 [80] 17 70 Microparticles (350—500 ) ± coils 43.5 ND 0 30.4 0% 17.6 4.4 0 ND Chick, 2009 [97] 34 26 Alcohol + lipiodol 0 119 (29—244) 0 32.4 Renal infarction (n = 1) 5.9 0 8.8 44 (12—116) Sooriakumaran, 2009 [83] 19 26.3 ND 31.6 ND 0 15.8 Abscess (n = 1), renal atrophy (n = 1), refractory hypertension (n = 1) 36.8 0 5.3 ND Lee, 2009 [81] 11 36.4 "
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    ABSTRACT: Objective: To deduce recommendations from the literature on the management of kidney damage caused by tuberous sclerosis complex (TSC). Material and methods: Five practitioners have written up recommendations after reviewing the literature. They were evaluated by 14 experts using a 9 level scale (1: complete disagreement; 9: complete agreement), then reworded until each item received a median score of greater than or equal to 8. Results: Forty-eight to 80% of patients with TSC have kidney disease with the presence of angiomyolipomas (AML), cysts, cancers and/or progression towards renal insufficiency. An abdominal ultrasound (and serum creatinine level if there is an abnormality) is recommended as soon as the TSC is diagnosed. The evaluation should be repeated every 3 to 5 years if it is normal. Numerous and voluminous cysts are suggestive of associated polycystosis. After 20 years of age, the monitoring should be based on CT scan or MRI, which are more precise in the monitoring of AML. The biopsy of a renal mass should be discussed if there are calcifications, central necrosis or rapid growth. Lymphangioleiomyomatosis should be screened for in women via pulmonary CT scan at 18 and 30 to 40 years of age. Haemorrhagic rupture of an AML should be treated in first-line by embolisation. Asymptomatic AMLs that cumulate risk factors for bleeding (size >80 mm, predominant vascular contingent, micro-aneurisms) should be preventively treated, if possible by embolisation. The role of mTOR inhibitors remains to be defined. Conclusion: Monitoring and a standardised treatment are necessary to improve the treatment of renal damage caused by TSC.
    Preview · Article · Feb 2013 · Diagnostic and interventional imaging
    • "14 7.1 Alcohol + lipiodol, alcohol alone, coils + microparticles + gel foam 0 110 (40—350) 0 100 Abscess (drainage, n = 1) 14.3 0 7.1 > 12 months in 12 pts Harabayashi, 2004 [33] 12 100 Alcohol + coils 0 ND 0 ND Renal atrophy (n = 1) 50 0 0 60 (12—170) Ewalt, 2005 [37] 16 100 Microparticles 18.8 ND (40—210) 0 68.8 Pneumonia (n = 1) 0 0 0 ND Khotary, 2005 [74] 19 52.6 Alcohol + lipiodol 36.8 ND 0 100 0 31.6 a 5.3 0 51.5 (6—132) William, 2006 [82] 16 100 Microparticles ± coils 37.5 ND b 0 6.2 0 0 0 0 40 (6—62) Dabbeche, 2006 [75] 37 10.8 c Microparticles ± coils ± Alcohol 43.2 78 (45—180)/53 (40—110) d 8.1 16.2 Arterial breach (nephrectomy, n = 1), arterial dissection (stent, n = 1) 10.8 2.7 e 32.4 (50/21.1) d 21 Lenton, 2008 [80] 17 70 Microparticles (350—500 ) ± coils 43.5 ND 0 30.4 0% 17.6 4.4 0 ND Chick, 2009 [97] 34 26 Alcohol + lipiodol 0 119 (29—244) 0 32.4 Renal infarction (n = 1) 5.9 0 8.8 44 (12—116) Sooriakumaran, 2009 [83] 19 26.3 ND 31.6 ND 0 15.8 Abscess (n = 1), renal atrophy (n = 1), refractory hypertension (n = 1) 36.8 0 5.3 ND Lee, 2009 [81] 11 36.4 "
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    ABSTRACT: To review existing literature and deduce guidelines for the management of renal disease in patients with tuberous sclerosis complex (TSC). After review of literature, a core panel of five physicians wrote a draft that was evaluated by 14 reviewers who used a 9-level scale (1: total disagreement; 9: total agreement). The guidelines were then reformulated until each item received a median score superior or equal to 8. Forty-eight to 80 % of TSC patients have significant renal involvement including angiomyolipomas (AMLs), cysts, malignant tumors and renal insufficiency. It is recommended to perform an abdominal ultrasound (and serum creatinine if abnormal ultrasound) when TSC is diagnosed. This work-up will be repeated every 3-5years if normal. Associated autosomal dominant polycystic kidney disease must be suspected in case of numerous and large cysts. After the age of 20, follow-up should use computed tomography (CT) or MRI that are more precise than ultrasound for the measurement of AMLs. Biopsy of a renal mass should be discussed in case of calcifications, necrosis or rapid growth. Females with TSC should undergo screening for pulmonary lymphangioleiomyomatosis by CT at the age of 18, and, if negative at the age of 30-40. Acute bleeding should be treated with percutaneous embolization. Asymptomatic angiomyolipomas with several risk factors (size>80mm, predominant vascular component, micro-aneurysms) should undergo prophylactic treatment, if possible using embolization. The role of mTOR inhibitors in the management of angiomyolipomas needs to be defined. Standardization of follow-up and treatment is necessary to improve the management of TSC renal involvement.
    No preview · Article · Jun 2012 · Progrès en Urologie
    • "The risk of hemorrhage is associated with the size of the AML (greater than 4 cm) and size of the aneurysm (greater than 5 mm), the latter having higher sensitivity and specificity.[60] Control of active bleeding and prevention of re-bleeding are achieved by embolization of the aneurysm,[6162] that preserves most of the renal parenchyma[63] [Figure 7]. On angiogram in the acute setting, localizing the symptomatic aneurysm can be difficult, as active extravasation is rare and there is typically extensive abnormal vasculature. "
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    ABSTRACT: Tuberous sclerosis (TS), also known as Bourneville disease or Bourneville-Pringle disease, is an autosomal dominant genetic disorder classically characterized by the presence of hamartomatous growths in multiple organs. TS and tuberous sclerosis complex (TSC) are different terms for the same genetic condition. Both terms describe clinical changes due to mutations involving either of the two genes named TSC1 and TSC2, which regulate cell growth. The diagnosis of TSC is established using diagnostic criteria based on clinical and imaging findings. Routine screening and surveillance of patients with TSC is needed to determine the presence and extent of organ involvement, especially the brain, kidneys, and lungs, and identify the development of associated complications. As the treatment is organ specific, imaging plays a crucial role in the management of patients with TSC.
    No preview · Article · Jul 2011 · Journal of Clinical Imaging Science
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