Recent Scientific Articles

Cai, Xiong, Gustavo Pacheco-Rodriguez, Mary Haughey, Leigh Samsel, Suowen Xu, Hai-Ping Wu, J. Philip McCoy, Mario Stylianou, Thomas N. Darling, and Joel Moss. 2014a. “Sirolimus Decreases Circulating Lymphangioleiomyomatosis Cells in Patients with Lymphangioleiomyomatosis.” Chest 145 (1): 108–12. doi:10.1378/chest.13-1071.

El-Chemaly, Souheil, and Elizabeth P. Henske. 2015. “The next Breakthrough in LAM Clinical Trials May Be Their Design: Challenges in Design and Execution of Future LAM Clinical Trials.” Expert Review of Respiratory Medicine 9 (2).  doi:10.1586/17476348.2015.1024663.

El-Chemaly, Souheil, Hilary J. Goldberg, and Allan R. Glanville. 2014. “Should Mammalian Target of Rapamycin Inhibitors Be Stopped in Women with Lymphangioleiomyomatosis Awaiting Lung Transplantation?” Expert Review of Respiratory Medicine 8 (6): 657–60. doi:10.1586/17476348.2014.956728.

Fan, Daming, Li Ding, Hui Liu, Jigang Wang, Wenwen Ran, Yujun Li, and Dongliang Lin. 2014. “Effusion Cytology: An Effective Method for the Diagnosis of Pulmonary Lymphangioleiomyomatosis.” Journal of Thoracic Disease 6 (5): E54–57. doi:10.3978/j.issn.2072-1439.2014.03.12.

Gao, Ling, Michael M. Yue, Jennifer Davis, Elisabeth Hyjek, and Lucia Schuger. 2014. “In Pulmonary Lymphangioleiomyomatosis Expression of Progesterone Receptor Is Frequently Higher than that of Estrogen Receptor.” Virchows Archiv : An International Journal of Pathology 464 (4). doi:10.1007/s00428-014-1559-9.

Gupta, R., M. Kitaichi, Y. Inoue, R. Kotloff, and F. X. McCormack. 2014a&b. “Lymphatic Manifestations of Lymphangioleiomyomatosis.” Lymphology 47 (3): 106–17.

Li, Jing, Alfredo Csibi, Sun Yang, Gregory R. Hoffman, Chenggang Li, Erik Zhang, Jane J. Yu, and John Blenis. 2015. “Synthetic Lethality of Combined Glutaminase and Hsp90 Inhibition in mTORC1-Driven Tumor Cells.” Proceedings of the National Academy of Sciences of the United States of America 112 (1): E21–29. doi:10.1073/pnas.1417015112.

McCormack, Francis X., William D. Travis, Thomas V. Colby, Elizabeth P. Henske, and Joel Moss. 2012. “Lymphangioleiomyomatosis: Calling It What It Is: A Low-Grade, Destructive, Metastasizing Neoplasm.” American Journal of Respiratory and Critical Care Medicine 186 (12): 1210–12. doi:10.1164/rccm.201205-0848OE.

Henske, Elizabeth P., and Francis X. McCormack. 2012. “Lymphangioleiomyomatosis - a Wolf in Sheep’s Clothing.” The Journal of Clinical Investigation 122 (11): 3807–16. doi:10.1172/JCI58709.

Priolo, Carmen, Stephane J. H. Ricoult, Damir Khabibullin, Harilaos Filippakis, Jane Yu, Brendan D. Manning, Clary Clish, and Elizabeth P. Henske. 2015. “TSC2 Loss Increases Lysophosphatidylcholine Synthesis in Lymphangioleiomyomatosis.” American Journal of Respiratory Cell and Molecular Biology, March. doi:10.1165/rcmb.2014-0379RC.

Yao, Jianhua, Angelo M. Taveira-DaSilva, Amanda M. Jones, Patricia Julien-Williams, Mario Stylianou, and Joel Moss. 2014. “Sustained Effects of Sirolimus on Lung Function and Cystic Lung Lesions in Lymphangioleiomyomatosis.” American Journal of Respiratory and Critical Care Medicine 190 (11): 1273–82. doi:10.1164/rccm.201405-0918OC.

Yeoh, Zhao W., Vidya Navaratnam, Rupesh Bhatt, Ian McCafferty, Richard B. Hubbard, and Simon R. Johnson. 2014a. “Natural History of Angiomyolipoma in Lymphangioleiomyomatosis: Implications for Screening and Surveillance.” Orphanet Journal of Rare Diseases 9. doi:10.1186/s13023-014-0151-3.

Young, Lisa, Hye-Seung Lee, Yoshikazu Inoue, Joel Moss, Lianne G. Singer, Charlie Strange, Koh Nakata, et al. 2013. “Serum VEGF-D a Concentration as a Biomarker of Lymphangioleiomyomatosis Severity and Treatment Response: A Prospective Analysis of the Multicenter International Lymphangioleiomyomatosis Efficacy of Sirolimus (MILES) Trial.” The Lancet. Respiratory Medicine 1 (6): 445–52. doi:10.1016/S2213-2600(13)70090-0.

Diagnostic Blood Test Can Identify LAM in Some Patients

Click here to read about this important study conducted by Dr. Lisa Young at the University of Cincinnati and the Cincinnati Children's Hospital Medical Center.

Clinical Availability of Serum VEGF-D Test

The VEGF-D test is now available in a clinical lab at Cincinnati Children's. It can be ordered as a send out test from your clinic or hospital, and it is now done in a CAP/CLIA certified lab such that the results can be used for clinical care. Patients and referring physicians should note that although a serum VEGF-D level of 800 pg/ml or greater in the setting of a typical HRCT scan is diagnostic for VEGF-D, a normal VEGF-D level does not exclude the diagnosis of LAM.

Click here to visit the website for the lab at Cincinnati Children's that does the testing. The VEGF-D requisition form is then in an orange box on the right-hand side.

Or, click here to take you directly to the requisition form.

Listings for other scientific articles

Harari S, Torre O, Cassandro R, Taveira-Dasilva AM, Moss J. Bronchoscopic
diagnosis of Langerhans cell histiocytosis and lymphangioleiomyomatosis. Respir
Med. 2012 Sep;106(9):1286-92. Epub 2012 Jul 7. PubMed PMID: 22770823; PubMed
Central PMCID: PMC3413078.

Lesma E, Eloisa C, Isaia E, Grande V, Ancona S, Orpianesi E, Di Giulio AM,
Gorio A. Development of a Lymphangioleiomyomatosis Model by Endonasal
Administration of Human TSC2(-/-) Smooth Muscle Cells in Mice. Am J Pathol. 2012
Jul 4. [Epub ahead of print] PubMed PMID: 22770663.

Baldi BG, Albuquerque AL, Pimenta SP, Salge JM, Kairalla RA, Carvalho CR.
Exercise Performance and Dynamic Hyperinflation in Lymphangioleiomyomatosis. Am J
Respir Crit Care Med. 2012 Jun 14. [Epub ahead of print] PubMed PMID: 22700863.

Moua T, Olson EJ, St Jean HC, Ryu JH. Resolution of Chylous Pulmonary
Congestion and Respiratory Failure in LAM with Sirolimus Therapy. Am J Respir
Crit Care Med. 2012 May 3. [Epub ahead of print] PubMed PMID: 22561961.

Sandrini A, Silverstone E, Yates DH. Menstrual cycle variation of
retroperitoneal lymphangioleiomyomas in lymphangioleiomyomatosis. Intern Med J.
2011 Dec;41(12):832-5. doi: 10.1111/j.1445-5994.2011.02593.x. PubMed PMID:

Montero MA, Roman A, Berastegui C. Radical reduction of smooth muscle cells in
explanted lung of a LAM patient treated with sirolimus: first case report. J
Heart Lung Transplant. 2012 Apr;31(4):439-40. Epub 2012 Jan 23. PubMed PMID:

Hayashi T, Kumasaka T, Mitani K, Terao Y, Watanabe M, Oide T, Nakatani Y,
Hebisawa A, Konno R, Takahashi K, Yao T, Seyama K. Prevalence of uterine and
adnexal involvement in pulmonary lymphangioleiomyomatosis: a clinicopathologic
study of 10 patients. Am J Surg Pathol. 2011 Dec;35(12):1776-85. PubMed PMID:

Yu J, Parkhitko A, Henske EP. Autophagy: an 'Achilles' heel of tumorigenesis
in TSC and LAM. Autophagy. 2011 Nov;7(11):1400-1. Epub 2011 Nov 1. PubMed PMID:
21997371; PubMed Central PMCID: PMC3242802.

Dilling DF, Gilbert ER, Picken MM, Eby JM, Love RB, Le Poole IC. A current
viewpoint of lymphangioleiomyomatosis supporting immunotherapeutic treatment
options. Am J Respir Cell Mol Biol. 2012 Jan;46(1):1-5. Review. PubMed PMID:

Martignoni G, Bonetti F, Chilosi M, Brunelli M, Segala D, Amin MB, Argani P,
Eble JN, Gobbo S, Pea M. Cathepsin K expression in the spectrum of perivascular
epithelioid cell (PEC) lesions of the kidney. Mod Pathol. 2012 Jan;25(1):100-11.
doi: 10.1038/modpathol.2011.136. Epub 2011 Aug 26. PubMed PMID: 21874011.
11: Ryu JH, Hartman TE, Torres VE, Decker PA. Frequency of undiagnosed cystic
lung disease in patients with sporadic renal angiomyolipomas. Chest. 2012
Jan;141(1):163-8. Epub 2011 Jul 7. PubMed PMID: 21737494.

Seibert D, Hong CH, Takeuchi F, Olsen C, Hathaway O, Moss J, Darling TN.
Recognition of tuberous sclerosis in adult women: delayed presentation with
life-threatening consequences. Ann Intern Med. 2011 Jun 21;154(12):806-13, W-294.
PubMed PMID: 21690595; PubMed Central PMCID: PMC3367307.

Taveira-DaSilva AM, Hathaway O, Stylianou M, Moss J. Changes in lung function
and chylous effusions in patients with lymphangioleiomyomatosis treated with
sirolimus. Ann Intern Med. 2011 Jun 21;154(12):797-805, W-292-3. PubMed PMID:
21690594; PubMed Central PMCID: PMC3176735.

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. Is
sirolimus a therapeutic option for patients with progressive pulmonary
lymphangioleiomyomatosis? Respir Res. 2011 May 21;12:66. PubMed PMID: 21600034;
PubMed Central PMCID: PMC3116484.

Davies DM, de Vries PJ, Johnson SR, McCartney DL, Cox JA, Serra AL, Watson
PC, Howe CJ, Doyle T, Pointon K, Cross JJ, Tattersfield AE, Kingswood JC, Sampson
JR. Sirolimus therapy for angiomyolipoma in tuberous sclerosis and sporadic
lymphangioleiomyomatosis: a phase 2 trial. Clin Cancer Res. 2011 Jun
15;17(12):4071-81. Epub 2011 Apr 27. PubMed PMID: 21525172.

Goncharova EA, Goncharov DA, Li H, Pimtong W, Lu S, Khavin I, Krymskaya VP.
mTORC2 is required for proliferation and survival of TSC2-null cells. Mol Cell
Biol. 2011 Jun;31(12):2484-98. Epub 2011 Apr 11. PubMed PMID: 21482669; PubMed
Central PMCID: PMC3133430.

Adriaensen ME, Schaefer-Prokop CM, Duyndam DA, Zonnenberg BA, Prokop M.
Radiological evidence of lymphangioleiomyomatosis in female and male patients
with tuberous sclerosis complex. Clin Radiol. 2011 Jul;66(7):625-8. Epub 2011 Apr
2. PubMed PMID: 21459371.

McCormack FX, 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, Young LR,
Kinder BW, Downey GP, Sullivan EJ, Colby TV, McKay RT, Cohen MM, Korbee L,
Taveira-DaSilva AM, Lee HS, Krischer JP, Trapnell BC; National Institutes of
Health Rare Lung Diseases Consortium; MILES Trial Group. Efficacy and safety of
sirolimus in lymphangioleiomyomatosis. N Engl J Med. 2011 Apr
28;364(17):1595-606. Epub 2011 Mar 16. PubMed PMID: 21410393; PubMed Central
PMCID: PMC3118601.

Continuing Education Opportunities

The European Respiratory Society (ERS) is offering several courses as continuing medical education. Please visit the ERS website at click here for a list of courses and a description of each.