Multiple Bilateral Renal Angiomyolipomatosis in Patients with Tuborous Sclerosis

1996 ◽  
Vol 9 (5) ◽  
pp. 627-631
Author(s):  
I. Tsitouridis ◽  
A. Dimitriadis ◽  
K. Kouskouras ◽  
I. Economou ◽  
D. Kelekis
Keyword(s):  
Tuberous Sclerosis ◽  
Skin Lesions ◽  
Cystic Disease ◽  
Middle Aged ◽  
Renal Masses ◽  
Renal Cystic Disease ◽  
Multiple Renal Masses

Angiomyolipomas are benign hamartomatous lesions which occur mainly in the kidneys. They often arise in middle aged women and they most commonly present as solitary tumours of varying size, symptomatic or asymptomatic. They may also be found incidentally on sonography, CT or at autopsy. In 20% of the cases they coexist which CNS and skin lesions, thus presenting as tuberous sclerosis syndrome. In 80% of cases of tuberous sclerosis they are found as bilateral, multiple renal masses with or without renal cystic disease.

scholarly journals Renal Cystic Disease in Tuberous Sclerosis: Role of the Polycystic Kidney Disease 1 Gene

1997 ◽  
Vol 61 (4) ◽  
pp. 843-851 ◽  
Author(s):  
Julian R. Sampson ◽  
Magitha M. Maheshwar ◽  
Richard Aspinwall ◽  
Peter Thompson ◽  
Jeremy P. Cheadle ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Tuberous Sclerosis ◽  
Polycystic Kidney Disease ◽  
Cystic Disease ◽  
Polycystic Kidney ◽  
Renal Cystic Disease

ISOLATED UNILATERAL TUBEROUS SCLEROSIS-ASSOCIATED RENAL CYSTIC DISEASE IN A NEONATE

2005 ◽  
Vol 24 (4-5) ◽  
pp. 267-275 ◽  
Author(s):  
M. A. Weber ◽  
R. A. Risdon ◽  
M. Malone ◽  
P. G. Duffy ◽  
N. J. Sebire
Keyword(s):  
Tuberous Sclerosis ◽  
Cystic Disease ◽  
Renal Cystic Disease

scholarly journals Giant Bilateral Angiomyolipoma Associated with Tuberous Sclerosis of Bourneville: Exceptional Case and Therapeutic Dilemma

2021 ◽  
pp. 17-21
Author(s):  
Benatta Mahmoud
Keyword(s):  
Serum Creatinine ◽  
Tuberous Sclerosis ◽  
Abdominal Mass ◽  
Left Kidney ◽  
Skin Lesions ◽  
Treatment Modalities ◽  
Surveillance Program ◽  
Abdominal Ultrasonography ◽  
Renal Masses ◽  
Cardiac Rhabdomyoma

Angiomyolipoma (AML) is a mesenchymal tumour composed of variable proportions of adipose tissue and vascular and smooth muscle elements. It can cause potentially life-threatening complications. This report aims to describe a bilateral angiomyolipoma associated with tuberous sclerosis of Bourneville; the second aim is to discuss the treatment modalities of this disease. A 51-year-old woman with abdominal mass and characteristic triad: Epilepsy, mental retardation and skin lesions (adenoma) (Fig. 1), with no notion of hematuria or abdominal pain, abdominal ultrasonography and computed tomography showing bilateral renal masses of 28.4×17 cm on the left kidney and 22× 11.7 cm on the right, respectively (Fig. 2-3). Serum creatinine was 13.4g/l. Body-scan imaging finds cardiac rhabdomyoma (Fig. 4) and brain calcifications (Fig. 5). the patient underwent surveillance with scan imaging every month, and at the last control, she was asymptomatic, serum creatinine was still normal. The management of giant bilateral AML is a complex and multifactorial decision. Patients can knowingly choose an active surveillance program, even with giant AML, but the economic situation and mental status can limit the therapeutic choice.

Improvement in Renal Cystic Disease of Tuberous Sclerosis Complex After Treatment with Mammalian Target of Rapamycin Inhibitor

2017 ◽  
Vol 187 ◽  
pp. 318-322.e2 ◽  
Author(s):  
Brian J. Siroky ◽  
Alexander J. Towbin ◽  
Andrew T. Trout ◽  
Hannah Schäfer ◽  
Anna R. Thamann ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Mammalian Target Of Rapamycin ◽  
Target Of Rapamycin ◽  
Cystic Disease ◽  
Renal Cystic Disease

Renal Manifestations of Tuberous Sclerosis Complex

2020 ◽  
Vol 7 (3) ◽  
pp. 5-19
Author(s):  
Nikhil Nair ◽  
Ronith Chakraborty ◽  
Zubin Mahajan ◽  
Aditya Sharma ◽  
Sidarth Sethi ◽  
...  
Keyword(s):  
Tuberous Sclerosis ◽  
Tuberous Sclerosis Complex ◽  
Gene Disruption ◽  
Treatment Guidelines ◽  
Renal Cysts ◽  
Skin Lesions ◽  
Multiple Organ ◽  
Tsc2 Gene ◽  
Genetic Condition ◽  
Organ Systems

Tuberous sclerosis complex (TSC) is a genetic condition caused by a mutation in either the TSC1 or TSC2 gene. Disruption of either of these genes leads to impaired production of hamartin or tuberin proteins, leading to the manifestation of skin lesions, tumors and seizures. TSC can manifests in multiple organ systems with the cutaneous and renal systems being the most commonly affected. These manifestations can secondarily lead to the development of hypertension, chronic kidney disease, and neurocognitive declines. The renal pathologies most commonly seen in TSC are angiomyolipoma, renal cysts and less commonly, oncocytomas. In this review, we highlight the current understanding on the renal manifestations of TSC along with current diagnosis and treatment guidelines.

A 38-Year-old Woman With Multiple Renal Masses and a History of Ovarian Cancer

Urology ◽  
2012 ◽  
Vol 79 (6) ◽  
pp. 1210-1213 ◽  
Author(s):  
Caurice Wynter ◽  
Douglas Scherr ◽  
Michael P. Herman
Keyword(s):  
Ovarian Cancer ◽  
Renal Masses ◽  
History Of ◽  
Multiple Renal Masses

Renal cystic disease

1999 ◽  
Vol 11 (2) ◽  
pp. 141-151 ◽  
Author(s):  
Friedhelm Hildebrandt
Keyword(s):  
Cystic Disease ◽  
Renal Cystic Disease
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