Angiomyolipoma overview: Difference between revisions
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==Causes== | ==Causes== | ||
[[Angiomyolipoma]] is caused by a defect in the ''TSC1'' gene. It can also be caused by a defect in another gene:''TSC2''. | |||
==Differentiating Angiomyolipoma from other Diseases== | ==Differentiating Angiomyolipoma from other Diseases== | ||
Revision as of 14:54, 23 September 2015
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Angiomyolipoma Microchapters |
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Angiomyolipoma overview On the Web |
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Risk calculators and risk factors for Angiomyolipoma overview |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor-In-Chief: Cafer Zorkun, M.D., Ph.D. [2], Faizan Sheraz, M.D. [3]
Overview
Angiomyolipoma is a benign renal neoplasm previously considered to be a hamartoma or choristoma, but now known to be neoplastic.[1] It is composed of variable amounts of fat, vascular, and smooth muscle elements. The fat density of the tumour on CT has been regarded to be pathognomonic, although there are now case reports of renal cell carcinoma types also possessing macroscopic fat. The lesion is well demarcated and contains mature elements. It occurs in more than 50% of individuals with tuberous sclerosis, often bilaterally. Angiomyolipomata also occur in 40% of women who have a rare, cystic lung disease called lymphangioleiomyomatosis, or LAM.[2]
Classification
Angiomyolipoma may be classified according to etiology into: tuberous sclerosis associated and isolated angiomyolipoma.
Pathophysiology
On gross pathology, well circumscribed and uniform yellow mass are characteristic findings of angiomyolipoma.
Causes
Angiomyolipoma is caused by a defect in the TSC1 gene. It can also be caused by a defect in another gene:TSC2.
Differentiating Angiomyolipoma from other Diseases
Epidemiology and Demographics
Risk Factors
Natural History, Complications and Prognosis
History and Symptoms
Physical Examination
Laboratory Findings
CT
MRI
Ultrasound
Other Diagnostic Studies
Medical Therapy
There is no treatment for angiomyolipoma; the mainstay of therapy is surgical resection or embolisation.
Surgery
Surgery is not the first-line treatment option for patients with angiomyolipoma. Transcatheter arterial embolization is usually reserved for patients with complicated disease.[3]
References
- ↑ Eble JN. Angiomyolipoma of the kidney. Semin Diagn Pathol 1988;15:21-40
- ↑ McCormack FX (2006). "Lymphangioleiomyomatosis". MedGenMed. 8 (1): 15. PMC 1682009. PMID 16915145.
- ↑ Lewis JH, Weingold AB (1985). "The use of gastrointestinal drugs during pregnancy and lactation". Am J Gastroenterol. 80 (11): 912–23. PMID 2864852.