Renal Angiomyolipomas

Renal Angiomyolipomas in Lymphangioleiomyomatosis (LAM)
Kidney tumors called angiomyolipomas, unusual hamartomas containing fat, smooth muscle and blood vessels, are present in about 90% of patients with TSC and 30%-50% of sporadic LAM. Hemorrhage into an angiomyolipoma can produce symptoms from chronic intermittent flank pain to acute abdomen with hypovolemic shock.

Interestingly, angiomyolipomas are the only known neoplastic lesions in which the intratumoral blood vessels are composed of the cells containing transforming mutations. In most patients, angiomyolipomas are clinically silent, however flank pain, hydronephrosis, hematuria and loss of renal function can all occur. In S-LAM, angiomyolipomas are usually unilateral, small, solitary, and restricted to the kidney while in TSC-LAM they are more often larger, bilateral, multiple, multiorgan involving the spleen or liver, and prone to hemorrhage. The risk of renal hemorrhage from angiomyolipomas is associated with large size and with profusion of aneurysms.

Click here to read article on Renal Angiomyolipomas by Drs. Bissler and Kingswood.

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