Angiomyolipoma overview: Difference between revisions
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==History and Symptoms== | ==History and Symptoms== | ||
Symptoms of [[angiomyolipoma]] include [[nausea]], [[vomiting]], and [[abdominal pain]]. | |||
==Physical Examination== | ==Physical Examination== | ||
Revision as of 17:50, 23 September 2015
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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
Angiomyolipoma must be differentiated from retroperitoneal liposarcoma, adrenal myelolipoma, and renal cell carcinoma.
Epidemiology and Demographics
The incidence is approximately 30 per 100,000 individuals worldwide. Women are more commonly affected with angiomyolipoma than men.
Risk Factors
Natural History, Complications and Prognosis
If left untreated, small angiomyolipomas and those without dilated blood vessels (aneurysms) cause few problems. Small angiomyolipomas are associated with the most favorable prognosis.Common complications of angiomyolipoma include infection, hematuria and flank pain.
History and Symptoms
Symptoms of angiomyolipoma include nausea, vomiting, and abdominal pain.
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.