Statins in Lymphangioleiomyomatosis. Simvastatin and Atorvastatin Induce Differential Effects on tuberous sclerosis complex 2-Null Cell Growth and Signaling.
Atochina-Vasserman EN, Goncharov DA, Volgina AV, Milavec M, James ML, Krymskaya VP.
Am J Respir Cell Mol Biol. 2013 Nov;49(5):704-9.

A pilot study assessing the effect of bronchodilator on dynamic hyperinflation in LAM.

Baldi BG, de Albuquerque AL, Pimenta SP, Salge JM, Kairalla RA, Carvalho CR.
Respir Med. 2013 Nov;107(11):1773-80.

Right Native Lung Pneumonectomy Due to over Inflation Three Years after Left Single Lung Transplantation for Pulmonary Lymphangioleiomyomatosis.

Liu F, Ruan Z, Wang S, Lin Q.
Ann Thorac Cardiovasc Surg. 2013 Oct 3

Sirolimus decreases circulating LAM cells in patients with lymphangioleiomyomatosis.

Cai X, Pacheco-Rodriguez G, Haughey M, Samsel L, Xu S, Wu HP, McCoy JP, Stylianou M, Darling TN, Moss J.
Chest. 2013 Sep 19. [Epub ahead of print]

Sex-specific lung diseases: effect of oestrogen on cultured cells and in animal models.

Shim B, Pacheco-Rodriguez G, Kato J, Darling TN, Vaughan M, Moss J.
Eur Respir Rev. 2013 Sep 1;22(129):302-11.

Integration of mTOR and estrogen-ERK2 signaling in lymphangioleiomyomatosis pathogenesis.

Gu X, Yu JJ, Ilter D, Blenis N, Henske EP, Blenis J.
Proc Natl Acad Sci U S A. 2013 Sep 10;110(37):14960-5.

Osteoprotegerin contributes to the metastatic potential of cells with a dysfunctional TSC2 tumor-suppressor gene.
Steagall WK, Pacheco-Rodriguez G, Glasgow CG, Ikeda Y, Lin JP, Zheng G, Moss J.
Am J Pathol. 2013 Sep;183(3):938-50.

Uterine-specific loss of Tsc2 leads to myometrial tumors in both the uterus and lungs.

Prizant H, Sen A, Light A, Cho SN, DeMayo FJ, Lydon JP, Hammes SR.
Mol Endocrinol. 2013 Sep;27(9):1403-14.

Serum VEGF-D concentration as a biomarker of lymphangioleiomyomatosis severity and treatment response: a prospective analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) trial.
Young LR, Lee HS, Inoue Y, Moss J, Singer LG, Strange C, Nakata K, Barker AF, Chapman JT, Brantly ML, Stocks JM, Brown KK, Lynch JP 3rd, Goldberg HJ, Downey GP, Swigris JJ, Taveira-Dasilva AM, Krischer JP, Trapnell BC, McCormack FX.
Lancet Respir Med. 2013 Aug;1(6):445-452

Characterization of lymphangioleiomyomatosis as a neoplastic disease.

Burger CD.
Am J Respir Crit Care Med. 2013 Aug 1;188(3):398.

Prevalence and clinical features of lymphedema in patients with lymphangioleiomyomatosis.

Hoshika Y, Hamamoto T, Sato K, Eto H, Kuriyama S, Yoshimi K, Iwakami S, Takahashi K, Seyama K.
Respir Med. 2013 Aug;107(8):1253-9.

Positioning ganglioside D3 as an immunotherapeutic target in lymphangioleiomyomatosis.

Gilbert ER, Eby JM, Hammer AM, Klarquist J, Christensen DG, Barfuss AJ, Boissy RE, Picken MM, Love RB, Dilling DF, Le Poole IC.
Am J Pathol. 2013 Jul;183(1):226-34.

MicroRNA-21 is induced by rapamycin in a model of tuberous sclerosis (TSC) and lymphangioleiomyomatosis (LAM).
Trindade AJ, Medvetz DA, Neuman NA, Myachina F, Yu J, Priolo C, Henske EP.
PLoS One. 2013;8(3):e60014.

Lymphangioleiomyomatosis screening in women with tuberous sclerosis.
Cudzilo CJ, Szczesniak RD, Brody AS, Rattan MS, Krueger DA, Bissler JJ, Franz DN, McCormack FX, Young LR.
Chest. 2013 Aug;144(2):578-85.


Faslodex inhibits estradiol-induced extracellular matrix dynamics and lung metastasis in a model of lymphangioleiomyomatosis.
Li C, Zhou X, Sun Y, Zhang E, Mancini JD, Parkhitko A, Morrison TA, Silverman EK, Henske EP, Yu JJ. Am J Respir Cell Mol Biol. 2013 Jul;49(1):135-42.
Perivascular epithelioid cell tumor located retroperitoneally with pulmonary lymphangioleiomyomatosis: report of a case.
Pata G, Tironi A, Solaini L, Tiziano T, Ragni F. Surg Today. 2013 Mar 21. [Epub ahead of print]


Exonic mutations of TSC2/TSC1 are common but not seen in all sporadic pulmonary lymphangioleiomyomatosis.
Badri KR, Gao L, Hyjek E, Schuger N, Schuger L, Qin W, Chekaluk Y, Kwiatkowski DJ, Zhe X.
Am J Respir Crit Care Med. 2013 Mar 15;187(6):663-5.


Everolimus for angiomyolipoma associated with tuberous sclerosis complex or sporadic lymphangioleiomyomatosis (EXIST-2): a multicentre, randomised, double-blind, placebo-controlled trial.

Bissler JJ, Kingswood JC, Radzikowska E, Zonnenberg BA, Frost M, Belousova E, Sauter M, Nonomura N, Brakemeier S, de Vries PJ, Whittemore VH, Chen D, Sahmoud T, Shah G, Lincy J, Lebwohl D, Budde K.
Lancet. 2013 Mar 9;381(9869):817-24


Targeted approaches toward understanding and treating pulmonary lymphangioleiomyomatosis (LAM).
Hammes SR, Krymskaya VP.
Horm Cancer. 2013 Apr;4(2):70-7.


The role of vascular endothelial growth factor-D in diagnosis of lymphangioleiomyomatosis (LAM).
Xu KF, Zhang P, Tian X, Ma A, Li X, Zhou J, Zeng N, Gui YS, Guo Z, Feng R, Zhang W, Sun W, Cai B.
Respir Med. 2013 Feb;107(2):263-8.


Clinical predictors of mortality and cause of death in lymphangioleiomyomatosis: a population-based registry.
Oprescu N, McCormack FX, Byrnes S, Kinder BW.
Lung. 2013 Feb;191(1):35-42.


Lymphangioleiomyomatosis: calling it what it is: a low-grade, destructive, metastasizing neoplasm.
McCormack FX, Travis WD, Colby TV, Henske EP, Moss J.
Am J Respir Crit Care Med. 2012 Dec 15;186(12):1210-2.


Utility of transbronchial biopsy in the diagnosis of lymphangioleiomyomatosis.
Meraj R, Wikenheiser-Brokamp KA, Young LR, Byrnes S, McCormack FX.
Front Med. 2012 Dec;6(4):395-405.


Lymphangioleiomyomatosis – a wolf in sheep’s clothing.
Henske EP, McCormack FX.
J Clin Invest. 2012 Nov 1;122(11):3807-16.

Prevention of alveolar destruction and airspace enlargement in a mouse model of pulmonary lymphangioleiomyomatosis (LAM).
Goncharova EA, Goncharov DA, Fehrenbach M, Khavin I, Ducka B, Hino O, Colby TV, Merrilees MJ, Haczku A, Albelda SM, Krymskaya VP.
Sci Transl Med. 2012 Oct 3;4(154):154ra134.


Lymphangioleiomyomatosis: new concepts in pathogenesis, diagnosis, and treatment.
Meraj R, Wikenheiser-Brokamp KA, Young LR, McCormack FX.
Semin Respir Crit Care Med. 2012 Oct;33(5):486-97.


Quantitative CT analysis of small pulmonary vessels in lymphangioleiomyomatosis.
Ando K, Tobino K, Kurihara M, Kataoka H, Doi T, Hoshika Y, Takahashi K, Seyama K.
Eur J Radiol. 2012 Dec;81(12):3925-30.


Resolution of chylous pulmonary congestion and respiratory failure in lymphangioleiomyomatosis with sirolimus therapy.
Moua T, Olson EJ, Jean HC, Ryu JH.
Am J Respir Crit Care Med. 2012 Aug 15;186(4):389-90.


Cystic lung disease is not uncommon in men with tuberous sclerosis complex.
Ryu JH, Sykes AM, Lee AS, Burger CD.
Respir Med. 2012 Nov;106(11):1586-90.


Real-time monitoring of tumorigenesis, dissemination, & drug response in a preclinical model of lymphangioleiomyomatosis/tuberous sclerosis complex.
Liu F, Lunsford EP, Tong J, Ashitate Y, Gibbs SL, Yu J, Choi HS, Henske EP, Frangioni JV.
PLoS One. 2012;7(6):e38589.


Exercise performance and dynamic hyperinflation in lymphangioleiomyomatosis.
Baldi BG, Albuquerque AL, Pimenta SP, Salge JM, Kairalla RA, Carvalho CR.
Am J Respir Crit Care Med. 2012 Aug 15;186(4):341-8.


Clinical utility of diagnostic guidelines and putative biomarkers in lymphangioleiomyomatosis.
Chang WY, Cane JL, Blakey JD, Kumaran M, Pointon KS, Johnson SR.
Respir Res. 2012 Apr 18;13:34.


Cathepsin K expression in the spectrum of perivascular epithelioid cell (PEC) lesions of the kidney.
Martignoni G, Bonetti F, Chilosi M, Brunelli M, Segala D, Amin MB, Argani P, Eble JN, Gobbo S, Pea M.
Mod Pathol. 2012 Jan;25(1):100-11.


Menstrual cycle variation of retroperitoneal lymphangioleiomyomas in lymphangioleiomyomatosis.
Sandrini A, Silverstone E, Yates DH.
Intern Med J. 2011 Dec;41(12):832-5.


Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis.
Harknett EC, Chang WY, Byrnes S, Johnson J, Lazor R, Cohen MM, Gray B, Geiling S, Telford H, Tattersfield AE, Hubbard RB, Johnson SR.
QJM. 2011 Nov;104(11):971-9.


Features of Pneumothorax and Risk of Air Travel in Lymphangioleiomyomatosis.
Yoshito Hoshika, MD*; Hideyuki Kataoka, MD; Masatoshi Kurihara, PhD; Katsutoshi Ando, MD; Kuniaki Seyama, PhD and Kazuhisa Takahashi, PhD – Juntendo University, Tokyo, Japan.
Chest October 2011; vol. 140 no. 4 Meeting Abstracts 450A.

Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus.
Taveira-DaSilva AM, Hathaway O, Stylianou M, Moss J.
Ann Intern Med. 2011 Jun 21;154(12):797-805, W-292-3.


Is sirolimus a therapeutic option for patients with progressive pulmonary lymphangioleiomyomatosis?
Neurohr C, Hoffmann AL, Huppmann P, Herrera VA, Ihle F, Leuschner S, von Wulffen W, Meis T, Baezner C, Leuchte H, Baumgartner R, Zimmermann G, Behr J.
Respir Res. 2011 May 21;12:66.


Sirolimus Therapy for Angiomyolipoma in Tuberous Sclerosis and Sporadic Lymphangioleiomyomatosis: A Phase 2 Trial
D. Mark Davies, Petrus J. de Vries, Simon R. Johnson, Deborah L. McCartney, Jane A. Cox, Andreas L. Serra, Peter C. Watson, Christopher J. Howe, Tim Doyle, Kate Pointon, Justin J. Cross, Anne E. Tattersfield, J. Chris Kingswood, and Julian R. Sampson.
Clin Cancer Res 2011 April 27


Efficacy and Safety of Sirolimus in Lymphangioleiomyomatosis.
McCormack FX, Inoue Y, Moss J, Singer LG, Strange C, Nakata K, Barker AF, Chapman JT, Brantly ML, Stocks JM, Brown KK, Lynch JP, Goldberg HJ, Young LR, Kinder BW, Downey GP, Sullivan EJ, Colby TV, McKay RT, Cohen MM, Korbee L, Taveira-DaSilva AM, Lee H-S, Krischer JP, Trapnell BC, for the National Institutes of Health Rare Lung Diseases Consortium and the MILES Trial Group.
N Engl J Med. 2011 Mar 16.


Lymphangioleiomyomatosis: a disease involving the lymphatic system.
Seyama K, Kumasaka T, Kurihara M, Mitani K, Sato T.
Lymphat Res Biol. 2010 Mar;8(1):21-31. Review.


Equivalent benefit of mTORC1 blockade and combined PI3K-mTOR blockade in a mouse model of tuberous sclerosis.
Pollizzi K, Malinowska-Kolodziej I, Stumm M, Lane H, Kwiatkowski D.
Mol Cancer. 2009 Jun 15; 8: 38.


Chemokine-enhanced chemotaxis of lymphangioleiomyomatosis cells with mutations in the tumor suppressor TSC2 gene.
Pacheco-Rodriguez G, Kumaki F, Steagall WK, Zhang Y, Ikeda Y, Lin JP, Billings EM, Moss J.
J Immunol. 2009 Feb 1, 182(3):1270-7.


Clinical trials for rare lung diseases: lessons from lymphangioleiomyomatosis.
Kinder B, McCormack FX.
Lymphat Res Biol. 2010 Mar;8(1):71-9.


Serum vascular endothelial growth factor-D levels in patients with lymphangioleiomyomatosis reflect lymphatic involvement.
Glasgow CG, Avila NA, Lin JP, Stylianou MP, Moss J.
Chest. 2009 May, 135(5): 1293-300.


Why and how do tumors stimulate lymphangiogenesis?
Cao Y.
Lymphat Res Biol. 2008;6(3-4):145-8. Review.


Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.
Bissler JJ, McCormack FX, Young LR, Elwing JM, Chuck G, Leonard JM, Schmithorst VJ, Laor T, Brody AS, Bean J, Salisbury S, Franz DN.
N Engl J Med. 2008 Jan 10; 358(2): 140-51.


TSC2 loss in lymphangioleiomyomatosis cells correlated with expression of CD44v6, a molecular determinant of metastasis.
Pacheco-Rodriguez G, Steagall WK, Crooks DM, Stevens LA, Hashimoto H, Li S, Wang JA, Darling TN, Moss J.
Cancer Res. 2007 Nov 1, 67(21): 10573-81.

Taveira-DaSilva AM, Steagall WK, Moss J.
Cancer Control 2006, 13: 276-85.


The NHLBI lymphangioleiomyomatosis registry: characteristics of 230 patients at enrollment.
Ryu JH, Moss J, Beck GJ, Lee JC, Brown KK, Chapman JT, Finlay GA, Olson EJ, Ruoss SJ, Maurer JR, Raffin TA, Peavy HH, McCarthy K, Taveira-Dasilva A, McCormack FX, Avila NA, Decastro RM, Jacobs SS, Stylianou M, Fanburg BL; NHLBI LAM Registry Group.
Am J Respir Crit Care Med. 2006 Jan 1;173(1):105-11.


Abnormal growth of smooth muscle-like cells in lymphangioleiomyomatosis: role for tumor suppressor TSC2.
Goncharova EA, Goncharov DA, Spaits M, Noonan DJ, Talovskaya E, Eszterhas A, Krymskaya VP.
Am J Respir Cell Mol Biol 2006;34:561–72.


Vascular endothelial growth factor-D is increased in serum of patients with lymphangioleiomyomatosis.
Seyama K, Kumasaka T, Souma S, Sato T, Kurihara M, Mitani K, Tominaga S, Fukuchi Y.
Lymphat Res Bio 2006, 4: 143-52.


Modulation of Cell Migration and Invasiveness by Tumor Suppressor TSC2 in Lymphangioleiomyomatosis.
Goncharova EA, Goncharov DA, Lim PN, Noonan D, and Krymskaya VP.
Am J Respir Cell Mol Biol 2006, 34: 473–480.


Lymphangiogenesismediated shedding of LAM cell clusters as a mechanism for dissemination in lymphangioleiomyomatosis.
Kumasaka T, Seyama K, Mitani K, Souma S, Kashiwagi S, Hebisawa A, Sato T, Kubo H, Gomi K, Shibuya K.
Am J Surg Pathol 2005;29: 1356–1366.


Molecular and genetic analysis of disseminated neoplastic cells in lymphangioleiomyomatosis.
Crooks DM, Pacheco-Rodriguez G, DeCastro RM, McCoy JP, Wang JrJA, Kumaki F, Darling T, and Moss J.
Proc. Natl. Acad. Sci. USA 2004, 101: 17462–17467.


Lymphangiogenesis in lymphangioleiomyomatosis.
Kumasaka T, Seyama K, Mitani K, Sato T, Souma S, Kondo T, Hayashi S, Minami M, Uekusa T, Fukuchi Y, Suda K.
Am J Surg Pathol 2004, 28: 1007-16.


Recurrent lymphangiomyomatosis after transplantation: genetic analyses reveal a metastatic mechanism.
Karbowniczek M, Astrinidis A, Balsara BR, Testa JR, Lium JH, Colby TV, McCormack FX, Henske EP.
Am J Respir Crit Care Med 2003, 167:976 –982.


Prognostic significance of pulmonary lymphangioleiomyomatosis histologic score.
Matsui K, Beasley MB, Nelson WK, Barnes PM, Bechtle J, Falk R, Ferrans VJ, Moss J, Travis WD.
Am J Surg Pathol. 2001 Apr;25(4):479-84.


High frequency of pulmonary Lymphangioleiomyomatosis in women with tuberous sclerosis complex.
Costello LC, Hartman TE, Ryu JH.
Mayo Clin Proc 2000, 75: 591–594.


Mutations in the tuberous sclerosis complex gene TSC2 are a cause of sporadic pulmonary lymphangioleiomyomatosis.
Carsillo T, Astrinidis A, Henske EP.
Proc Natl Acad Sci U S A. 2000 May 23;97(11):6085-90.


Evidence that lymphangiomyomatosis is caused by TSC2 mutations: chromosome 16p13 loss of heterozygosity in angiomyolipomas and lymph nodes from women with lymphangiomyomatosis.
Smolarek TA, Wessner LL, McCormack FX, Mylet JC, Menon AG, Henske EP.
Am J Hum Genet 1998, 62:810–815