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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2019  |  Volume : 30  |  Issue : 2  |  Page : 545-548
Regression of renal angiomyolipomas with oral rapamycin therapy in a patient with tuberous sclerosis complex disease


1 Department of Nephrology, Adana Dr. Turgut Noyan Research and Medical Center, Faculty of Medicine, Baskent University, Adana, Turkey
2 Department of Interventional Radiology, Adana Dr. Turgut Noyan Research and Medical Center, Faculty of Medicine, Baskent University, Adana, Turkey

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Date of Submission26-Feb-2018
Date of Acceptance27-Mar-2018
Date of Web Publication23-Apr-2019
 

   Abstract 


We present a case of a 32-year-old female who had been diagnosed tuberous sclerosis complex (TSC) two years ago. In view of serious hemorrhagic complication risk of the selective embolization, we commenced her on oral rapamycin therapy for regression of angiomyolipomas (AMLs). On the 1st year of rapamycin treatment, bilateral renal AMLs were regressed and bilateral selective embolization of the AML was performed after the 1st year of treatment. Rapamycin therapy may regress renal lesions in TSC disease. Therefore, it may increase surgical intervention.

How to cite this article:
Torun D, Micozkadioglu H, Gedikoglu M, Ozelsancak R, Tekkarismaz N. Regression of renal angiomyolipomas with oral rapamycin therapy in a patient with tuberous sclerosis complex disease. Saudi J Kidney Dis Transpl 2019;30:545-8

How to cite this URL:
Torun D, Micozkadioglu H, Gedikoglu M, Ozelsancak R, Tekkarismaz N. Regression of renal angiomyolipomas with oral rapamycin therapy in a patient with tuberous sclerosis complex disease. Saudi J Kidney Dis Transpl [serial online] 2019 [cited 2019 May 20];30:545-8. Available from: http://www.sjkdt.org/text.asp?2019/30/2/545/256866



   Introduction Top


Tuberous sclerosis complex (TSC) a multisystem disease which is characterized by the development of hamartomatous lesions in multiple organs.[1] Bilateral and multiple angiomyolipomas (AMLs) were observed in 50%–80% of TSC patients.[2] Although the AML is a benign tumor, it may be complicated with rupture during their clinical course. If the lesion size is larger than 4 cm in diameter the risk of bleeding increase.[3] The main goals of treatment are on preventing acute events, preserving renal parenchyma, and to avoid development of end-stage renal disease. According to the International TSC Consensus Conference in 2012, mammalian target of rapamycin inhibitor (mTORi) is the recommended first-line therapy for patients with asymptomatic, growing AMLs sized more than 3 cm in diameter.[4]

Sudden massive bleeding may require an urgent approach; patients may require nephrectomy or selective arterial emobilization (SAE) of the bleeding AMLs. Selective arterial embolization is the popular approach for the management of symptomatic or large size AML treatment in recent years.[5],[6] Preventive arterial embolization or treatment with mTORi is recommended in asymptomatic patients with progressive growing AMLs larger than 4 cm diameters and/or with an aneurysm larger than 5 mm in diameter in Japan.[7] We present a young adult female patient having bilateral large renal AMLs associated with TSC disease whose lesions are regressed with oral rapamycin and selective arterial embolization.


   Case Report Top


A 32-year-old female patient referred to our department due to bilateral renal mass at the abdominal magnetic resonance examination from the Department of Neurology in January 2016. TSC-related brain involvement was diagnosed in 2014 at the neurology department and she had been taking antiepileptic therapy for two years.

At her first examination in our department, she was asymptomatic. Blood tests at admission revealed the following results: hemoglobin: 11.2 g/L, blood urea nitrogen: 11 mg/ dL, and serum creatinine: 0.5 mg/dL. The patient’s glomerular filtration rate calculated with the modification of diet in renal disease formula was 128 mL/min, and the urine protein-to-creatinine ratio was 0.038.

Abdominal magnetic resonance examination disclosed bilateral renal AMLs (in the lower pole of the right kidney 74.31 mm × 50.85 mm, in the upper pole of the left kidney 75.04 mm × 63.45 mm diameter) and multiple hamartomas.

Because of the normal renal function and bilateral large AMLs, the decision of SAE was applied instead of nephrectomy due to the risk of massive bleeding from bilateral renal AMLs in the nephrology urology council. However, due to serious hemorrhagic complication risk of the SAE procedure, the council decided to begin oral rapamycin therapy for regression of AMLs before the embolization. On the 1st year of oral daily 2 mg rapamycin treatment, bilateral renal AMLs were regressed (in the lower pole of the right kidney 52.85 mm × 37.06 mm, in the upper pole of the left kidney 44.78 mm × 42.07 mm diameters) [Figure 1]a, [Figure 1]b, and [Figure 2]a, [Figure 2]b.


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Side effects such as proteinuria and hyperlipidemia due to rapamycin treatment were not observed. Moreover, bilateral SAE of renal AMLs was performed after the 1st year of rapamycin treatment. No complications related to embolization were observed. Rapamycin treatment continued against the risk of regrowth of AMLs.

Written informed consent was obtained from the patient before reporting the case.


   Discussion Top


The most feared complication of AMLs associated with TSC is life-threatening bleeding.

mTORi and/or SAE are recommended in asymptomatic patients with lesions larger than 4 cm in diameter due to the risk of bleeding. Nephrectomy should be avoided if renal functions are normal. The other approaches for the management of bilateral AMLs with larger than 4 cm diameters is the SAE which in a systematic review of 31 studies which included a total of 524 AML cases, treatment with trans arterial embolization resulted in the technical success rate of >90% with no procedural mortality.[8] We preferred to use mTORi first-line therapy to regress the bilateral AMLs with larger than 4 cm diameters associated with TSC before SAE due to the serious hemorrhagic risk of the procedure. On the 1st year of daily 2 mg rapamycin treatment, bilateral renal AMLs were regressed, and then bilateral SAE was done without complication. Moreover to prevent the re-growing AMLs that require reembolization we continued the rapamycin treatment.


   Conclusion Top


Rapamycin therapy may regress the renal lesions in TSC disease obviating the need for invasive interventions and help preserve renal parenchyma.

Conflict of interest: None declared.



 
   References Top

1.
Northrup H, Krueger DA; International Tuberous Sclerosis Complex Consensus Group. Tuberous sclerosis complex diagnostic criteria update: Recommendations of the 2012 international tuberous sclerosis complex consensus conference. Pediatr Neurol 2013;49:243-54.  Back to cited text no. 1
    
2.
O’Callaghan FJ, Noakes MJ, Martyn CN, Osborne JP. An epidemiological study of renal pathology in tuberous sclerosis complex. BJU Int 2004;94:853-7.  Back to cited text no. 2
    
3.
Yamakado K, Tanaka N, Nakagawa T, Kobayashi S, Yanagawa M, Takeda K. Renal angiomyolipoma: Relationships between tumor size, aneurysm formation, and rupture. Radiology 2002;225:78-82.  Back to cited text no. 3
    
4.
Krueger DA. Management of CNS-related disease manifestations in patients with tuberous sclerosis complex. Curr Treat Options Neurol 2013;15:618-33.  Back to cited text no. 4
    
5.
Guziński M, Kurcz J, Tupikowski K, Antosz E, Słowik P, Garcarek J. The role of trans-arterial embolization in the treatment of renal tumors. Adv Clin Exp Med 2015;24:837-43.  Back to cited text no. 5
    
6.
Thorlund MG, Wennevik GE, Andersen M, Andersen PE, Lund L. High success rate after arterial renal embolisation. Dan Med J 2015; 62. pii: A5061.  Back to cited text no. 6
    
7.
Harabayashi T, Shinohara N, Katano H, Nonomura K, Shimizu T, Koyanagi T. Management of renal angiomyolipomas associated with tuberous sclerosis complex. J Urol 2004;171:102-5.  Back to cited text no. 7
    
8.
Murray TE, Doyle F, Lee M. Transarterial embolization of angiomyolipoma: A systematic review. J Urol 2015;194:635-9.  Back to cited text no. 8
    

Top
Correspondence Address:
Dilek Torun
Department of Nephrology, Adana Dr. Turgut Noyan Research and Medical Center, Faculty of Medicine, Baskent University, Adana
Turkey
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DOI: 10.4103/1319-2442.256866

PMID: 31031395

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    Abstract
   Introduction
   Case Report
   Discussion
   Conclusion
    References
    Article Figures
 

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