The Correlation Between Tuberous Sclerosis Complex Genotype and Renal Angiomyolipoma Phenotype
Tuberous sclerosis complex (TSC) is a rare multisystem autosomal dominant genetic disease that occurs between 1 in 6,000 and 1 in 10,000 live births. Additionally, renal angiomyolipoma is the most common form of renal disease in patients affected by TSC.
Nianyi Zhang +8 more
doaj +2 more sources
Renal angiomyolipoma in patients with tuberous sclerosis complex: findings from the TuberOus SClerosis registry to increase disease Awareness [PDF]
Background Renal angiomyolipoma occurs at a high frequency in patients with tuberous sclerosis complex (TSC) and is associated with potentially life-threatening complications.
J. Kingswood +29 more
semanticscholar +3 more sources
Angiomyolipoma rebound tumor growth after discontinuation of everolimus in patients with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis. [PDF]
INTRODUCTION:The EXIST-2 (NCT00790400) study demonstrated the superiority of everolimus over placebo for the treatment of renal angiomyolipomas associated with tuberous sclerosis complex (TSC) or sporadic lymphangioleiomyomatosis (LAM).
John J Bissler +11 more
doaj +2 more sources
SUMMARY INTRODUCTION Angiomyolipoma is one of the most common benign solid renal tumors. We investigated the characteristics of renal angiomyolipomas and the clinical outcomes of patients in the last thirteen years. METHODS The medical records of the patients who underwent nephrectomy were reviewed retrospectively from July 2005 to May 2018.
Selahattin Çalışkan +4 more
openaire +5 more sources
Epitheloid Variant of Angiomyolipoma in a Patient with Tuberous Sclerosis. [PDF]
Epitheloid angiomyolipoma of kidney is a type of Perivascular endothelial cell derived tumor with an aggressive behaviour with specific pathological, immunohistochemistrical and genetic characteristics.
Vasu Reddy Challa +4 more
doaj +2 more sources
Renal angiomyolipoma-investigating radiological signs indicative of risk for bleeding [PDF]
Objectives To compare imaging differences between bleeding and non-bleeding angiomyolipoma with respect to the proportion and attenuation of the angiomyogenic component and the occurrence and size of aneurysms.
Jesper Swärd +5 more
doaj +2 more sources
Incidental giant renal schwannoma misdiagnosed as angiomyolipoma and low-grade spindle cell neoplasm: A case report. [PDF]
Djafari AA +3 more
europepmc +2 more sources
Background Although epithelioid angiomyolipoma of the kidney has been studied by several groups, the reported prevalence of malignant behavior remains uncertain and there are not yet definitive predictive biomarkers.
Yan-Na Zhang +3 more
semanticscholar +1 more source
Clinical characteristics and outcomes of patients with hepatic angiomyolipoma: A literature review
First reported in 1976, hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumor occurring mostly in middle-aged women. Diagnosis of the liver mass is often incidental on abdominal imaging due to the frequent absence of specific symptoms.
P. Calame +7 more
semanticscholar +1 more source
Hepatic angiomyolipomas are rare benign tumours. First reported by Ishak in 1976. Since then, the world literature showed only 32 cases including 8 autopsy findings. In this paper a retrospective analysis of the published data will be presented and the first report ofthe preoperative colour doppler and intraoperative sonography appearances will also be
Bakhotmah, Mohamad Abid +1 more
openaire +4 more sources

