Clinical Features of Lymphangioleiomyomatosis Complicated by Renal Angiomyolipomas

Abstract

Objective Renal angiomyolipomas (R-AMLs) are major complications of lymphangioleiomyomatosis (LAM). The objective of this study was to better understand the influence of R-AMLs in patients with LAM on the prognosis and other clinical factors related to respiration, and to investigate the management of RAMLs in patients with LAM. Patients and MethodsWe retrospectively investigated the clinical features of 7 patients with LAM [4 were TSC (Tuberous sclerosis complex)-LAM and 3 were S (sporadic)-LAM] complicated by R-AMLs admitted to our hospital from 1997 to 2008. Results All patients were females and the mean age at diagnosis of LAM was 40.7 years (31.7 years for TSC-LAM and 52.7 years for S-LAM). Although 5 patients had symptoms related to R-AMLs, only 1 patient experienced symptoms related to R-AMLs at the time of diagnosis. Five patients had bilateral and 2 patients had unilateral R-AMLs. R-AMLs ruptured in 4 cases (3 patients were TSC-LAM) including 2 patients in whom they ruptured bilaterally, and who underwent bilateral nephrectomy. In 1 case, unilateral R-AMLs grew larger and appeared on the other side during the follow-up period. Conclusion Although only rare cases of LAM show symptoms related to R-AMLs initially, R-AMLs are a notable complication. To avoid nephrectomy, R-AMLs should be diagnosed when they are small and should be followed up carefully by periodic echograms or CT scans.

Multiple Perivascular Epithelioid Cell Tumors: Clear Cell Tumor of the Lung Accompanied by Angiomyolipoma of the Liver

“…Some reports have documented that AML is complicated with LAM and that AML occurs multifocally in the kidney, liver, and lung. [4][5][6] This case is, to our knowledge, the first report of multiple PEComas, a combination of CCT of the lung and AML of the liver. These findings suggest that patients with PEComas may require whole-body follow-up examinations because PEComas may occur multifocally.…”

Lymphangioleiomyomatosis Presenting with Perirenal Hemorrhage

“…Although LAM is primarily a disease of the lungs, extrapulmonary manifestations are present in more than 70% of LAM patients, consisting of enlarged lymph nodes (40%), lymphangioleiomyomas (20%), chylous ascites (10%) and renal angiomyolipomas (AML) (39–93%) [ 5 6 ]. The most common of these is renal angiomyolipoma (AML).…”

“…Renal AML’s are benign tumors of mesenchymal origin. They occur in 93% of patients with TSC associated LAM and in about 30–50% of patients with sporadic LAM [ 6 ]. Renal AML’s vary in size from 1 mm to over 20 cm [ 7 ].…”