SM Bernstein, JD Newell Jr, D Adamczyk, RL Mortenson, TE King Jr and DA Lynch
Department of Radiology, National Jewish Center for Immunology and Respiratory Medicine, Denver, Colorado, USA.

Pulmonary lymphangiomyomatosis has been associated with renal angiomyolipoma in case reports, but the prevalence of this association has not been well documented. The objective of this study was to determine the frequency of renal angiomyolipoma in a series of subjects with pulmonary lymphangiomyomatosis. Eighteen consecutive patients with pulmonary lymphangiomyomatosis were seen at a single institution between 1989 and 1994. Of these, one patient was excluded because she did not have an abdominal computed tomographic (CT) scan. We found eight out of 17 (47%) patients with pulmonary lymphangiomyomatosis to have renal angiomyolipomas. These were found either at surgery or on abdominal CT scanning. Thus, renal angiomyolipomas occur commonly in association with pulmonary lymphangiomyomatosis. Consequently, the early detection of renal angiomyolipoma by abdominal CT may be important, because lesions with dimensions larger than 4 cm may present an increased risk for complications related to tumor growth or hemorrhage. Serial follow-up by ultrasonography or CT scanning is important in identifying and monitoring high-risk patients. Prophylactic treatment (partial or total nephrectomy) may be considered for patients with tumors that show significant growth or other complications, such as hemorrhage.

This article has been cited by other articles:

				A. K. Attili and E. A. Kazerooni Case 116: Lymphangioleiomyomatosis Radiology, July 1, 2007; 244(1): 303 - 308. [Full Text] [PDF]

				S. R. Johnson Lymphangioleiomyomatosis. Eur. Respir. J., May 1, 2006; 27(5): 1056 - 1065. [Abstract] [Full Text] [PDF]

				A. E. Tattersfield and M. K. Glassberg Lymphangioleiomyomatosis: A National Registry for a Rare Disease Am. J. Respir. Crit. Care Med., January 1, 2006; 173(1): 2 - 4. [Full Text] [PDF]

				N. El-Hashemite and D. J. Kwiatkowski Interferon-{gamma}-Jak-Stat Signaling in Pulmonary Lymphangioleiomyomatosis and Renal Angiomyolipoma: A Potential Therapeutic Target Am. J. Respir. Cell Mol. Biol., September 1, 2005; 33(3): 227 - 230. [Abstract] [Full Text] [PDF]

				N. El-Hashemite, V. Walker, and D. J. Kwiatkowski Estrogen Enhances whereas Tamoxifen Retards Development of Tsc Mouse Liver Hemangioma: A Tumor Related to Renal Angiomyolipoma and Pulmonary Lymphangioleiomyomatosis Cancer Res., March 15, 2005; 65(6): 2474 - 2481. [Abstract] [Full Text] [PDF]

				X. Zhe, Y. Yang, and L. Schuger Imbalanced Plasminogen System in Lymphangioleiomyomatosis: Potential Role of Serum Response Factor Am. J. Respir. Cell Mol. Biol., January 1, 2005; 32(1): 28 - 34. [Abstract] [Full Text] [PDF]

				X. Zhe and L. Schuger Combined Smooth Muscle and Melanocytic Differentiation in Lymphangioleiomyomatosis J. Histochem. Cytochem., December 1, 2004; 52(12): 1537 - 1542. [Abstract] [Full Text] [PDF]

				G. Finlay The LAM cell: what is it, where does it come from, and why does it grow? Am J Physiol Lung Cell Mol Physiol, April 1, 2004; 286(4): L690 - L693. [Full Text] [PDF]

				X. Zhe, Y. Yang, S. Jakkaraju, and L. Schuger Tissue Inhibitor of Metalloproteinase-3 Downregulation in Lymphangioleiomyomatosis: Potential Consequence of Abnormal Serum Response Factor Expression Am. J. Respir. Cell Mol. Biol., April 1, 2003; 28(4): 504 - 511. [Abstract] [Full Text] [PDF]

				E. Pallisa, P. Sanz, A. Roman, J. Majo, J. Andreu, and J. Caceres Lymphangioleiomyomatosis: Pulmonary and Abdominal Findings with Pathologic Correlation RadioGraphics, October 1, 2002; 22(90001): S185 - 198. [Abstract] [Full Text] [PDF]

				S R Johnson and A E Tattersfield Clinical experience of lymphangioleiomyomatosis in the UK Thorax, December 1, 2000; 55(12): 1052 - 1057. [Abstract] [Full Text]

				N. A. Avila, J. A. Kelly, S. C. Chu, A. J. Dwyer, and J. Moss Lymphangioleiomyomatosis: Abdominopelvic CT and US Findings Radiology, July 1, 2000; 216(1): 147 - 153. [Abstract] [Full Text]

				T. Carsillo, A. Astrinidis, and E. P. Henske Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis PNAS, May 23, 2000; 97(11): 6085 - 6090. [Abstract] [Full Text] [PDF]

				A. Tazi, P. Soler, and A. J Hance Rare diseases bullet 9: Adult pulmonary Langerhans' cell histiocytosis Thorax, May 1, 2000; 55(5): 405 - 416. [Full Text]

				H. Logginidou, X. Ao, I. Russo, and E. P. Henske Frequent Estrogen and Progesterone Receptor Immunoreactivity in Renal Angiomyolipomas From Women With Pulmonary Lymphangioleiomyomatosis* Chest, January 1, 2000; 117(1): 25 - 30. [Abstract] [Full Text] [PDF]

				A. Astrinidis, L. Khare, T. Carsillo, T. Smolarek, K.-S. Au, H. Northrup, and E. P. Henske Mutational analysis of the tuberous sclerosis gene TSC2 in patients with pulmonary lymphangioleiomyomatosis J. Med. Genet., January 1, 2000; 37(1): 55 - 57. [Abstract] [Full Text]

				S. C. Chu, K. Horiba, J. Usuki, N. A. Avila, C. C. Chen, W. D. Travis, V. J. Ferrans, and J. Moss Comprehensive Evaluation of 35 Patients With Lymphangioleiomyomatosis Chest, April 1, 1999; 115(4): 1041 - 1052. [Abstract] [Full Text] [PDF]

				S. Johnson Rare diseases bullet  1: Lymphangioleiomyomatosis: clinical features, management and basic mechanisms Thorax, March 1, 1999; 54(3): 254 - 264. [Full Text]

				N. A. Avila, J. Bechtle, A. J. Dwyer, V. J. Ferrans, and J. Moss Lymphangioleiomyomatosis: CT of Diurnal Variation of Lymphangioleiomyomas Radiology, November 1, 2001; 221(2): 415 - 421. [Abstract] [Full Text] [PDF]