Research & Literature

Regulation of mTORC1 by PI3K signaling.

Dibble CC, Cantley LC

Trends in cell biology 2015; (25(9)):545-55.

Use of sirolimus in the treatment of lymphangioleiomyomatosis: favorable responses in patients with different extrapulmonary manifestations.

Freitas CS, Baldi BG, Araújo MS, et al.

Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia 2015; (41(3)):275-80.

Mayo clinic experience of lung transplantation in pulmonary lymphangioleiomyomatosis.

Ussavarungsi K, Hu X, Scott JP, et al.

Respiratory medicine 2015; (109(10)):1354-9.

Detection of low-prevalence somatic TSC2 mutations in sporadic pulmonary lymphangioleiomyomatosis tissues by deep sequencing.

Fujita A, Ando K, Kobayashi E, et al.

Human genetics 2016; (135(1)):61-8 doi:10.1007/s00439-015-1611-0.

SUCCESSFUL TREATMENT OF PULMONARY AND LYMPHATIC MANIFESTATIONS OF LYMPHANGIOLEIOMYOMATOSIS WITH SIROLIMUS.

Hecimovic A, Jakopovic M, Pavlisa G, et al.

Lymphology 2015; (48(2)):97-102.

Lung Transplantation for Lymphangioleiomyomatosis in Japan.

Ando K, Okada Y, Akiba M, et al.

PloS one 2016; (11(1)):e0146749 doi:10.1371/journal.pone.0146749.

A Novel Quantitative Computed Tomographic Analysis Suggests How Sirolimus Stabilizes Progressive Air Trapping in Lymphangioleiomyomatosis.

Argula RG, Kokosi M, Lo P, et al.

Annals of the American Thoracic Society 2016; (13(3)):342-9 doi:10.1513/AnnalsATS.201509-631OC.

Pulmonary rehabilitation in lymphangioleiomyomatosis: a controlled clinical trial.

Araujo MS, Baldi BG, Freitas CS, et al.

The European respiratory journal 2016; (47(5)):1452-60 doi:10.1183/13993003.01683-2015.

Recurrence of lymphangioleiomyomatosis: Nine years after a bilateral lung transplantation.

Zaki KS, Aryan Z, Mehta AC, et al.

World journal of transplantation 2016; (6(1)):249-54 doi:10.5500/wjt.v6.i1.249.

Tuberous Sclerosis Complex Associated with Vascular Anomalies or Overgrowth.

Jenkins D, McCuaig C, Drolet BA, et al.

Pediatric dermatology 2016; (33(5)):536-42 doi:10.1111/pde.12946.

Efficacy and Safety of Long-Term Sirolimus Therapy for Asian Patients with Lymphangioleiomyomatosis.

Takada T, Mikami A, Kitamura N, et al.

Annals of the American Thoracic Society 2016; (13(11)):1912-1922 doi:10.1513/AnnalsATS.201605-335OC.

Coexistence of pulmonary lymphangioleiomyomatosis and pulmonary angiomyolipoma.

Sun X, Feng R, Zhang Y, et al.

BMC pulmonary medicine 2016; (16(1)):120 doi:10.1186/s12890-016-0286-1.

Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management.

McCormack FX, Gupta N, Finlay GR, et al.

American journal of respiratory and critical care medicine 2016; (194(6)):748-61 doi:10.1164/rccm.201607-1384ST.

[Pulmonary lymphangioleimyomatosis : review and case report].

Excoffier S, Guinand O, Rochat T

Revue medicale suisse 2016; (12(527)):1390-1393.

Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management. An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline.

Gupta N, Finlay GA, Kotloff RM, et al.

American journal of respiratory and critical care medicine 2017; (196(10)):1337-1348 doi:10.1164/rccm.201709-1965ST.

Cystic Lung Disease in Genetic Syndromes with Deficient Tumor Suppressor Gene Function.

Daccord C, Nicod LP, Lazor R

Respiration; international review of thoracic diseases 2017; (94(6)):467-485 doi:10.1159/000485106.

Emerging biomarkers of lymphangioleiomyomatosis.

Nijmeh J, El-Chemaly S, Henske EP

Expert review of respiratory medicine 2018; (12(2)):95-102 doi:10.1080/17476348.2018.1409622.

Functional improvements in patients with lymphangioleiomyomatosis after sirolimus: an observational study.

Zhan Y, Shen L, Xu W, et al.

Orphanet journal of rare diseases 2018; (13(1)):34 doi:10.1186/s13023-018-0775-9.

Single lung transplantation for lymphangioleiomyomatosis: a single-center experience in Japan.

Oishi H, Watanabe T, Matsuda Y, et al.

Surgery today 2018; (48(10)):944-950 doi:10.1007/s00595-018-1678-z.

Smoking-Related Diffuse Cystic Lung Disease.

Gupta N, Colby TV, Meyer CA, et al.

Chest 2018; (154(2)):e31-e35 doi:10.1016/j.chest.2018.02.027.

Efficacy of sirolimus for the prevention of recurrent pneumothorax in patients with lymphangioleiomyomatosis: a case series.

Zhou L, Ouyang R, Luo H, et al.

Orphanet journal of rare diseases 2018; (13(1)):168 doi:10.1186/s13023-018-0915-2.

Correlation between the native lung volume change and postoperative pulmonary function after single lung transplantation for lymphangioleiomyomatosis: Evaluation of lung volume by three-dimensional computed tomography volumetry.

Suzuki H, Oishi H, Noda M, et al.

PloS one 2019; (14(2)):e0210975 doi:10.1371/journal.pone.0210975.

Serum vascular endothelial growth factor-D as a diagnostic and therapeutic biomarker for lymphangioleiomyomatosis.

Hirose M, Matsumuro A, Arai T, et al.

PloS one 2019; (14(2)):e0212776 doi:10.1371/journal.pone.0212776.

Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis.

Gupta N, Lee HS, Young LR, et al.

The European respiratory journal 2019; (53(4)) doi:10.1183/13993003.02066-2018.

Difficult diagnosis and rare morphology of lymphangioleiomyomatosis with giant cysts.

Welter S, Thrull P, Westhoff M, et al.

Respiratory medicine case reports 2019; (28()):100873 doi:10.1016/j.rmcr.2019.100873.

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas.

Ussavarungsi K, Laroia AT, Burger CD

Lung India : official organ of Indian Chest Society 2019; (36(4)):349-352 doi:10.4103/lungindia.lungindia_433_18.

Long-term efficacy and safety of sirolimus therapy in patients with lymphangioleiomyomatosis.

Hu S, Wu X, Xu W, et al.

Orphanet journal of rare diseases 2019; (14(1)):206 doi:10.1186/s13023-019-1178-2.

Effects of sirolimus in lymphangioleiomyomatosis patients on lung cysts and pulmonary function: long-term follow-up observational study.

Kim C, Do KH, Cha J, et al.

European radiology 2020; (30(2)):735-743 doi:10.1007/s00330-019-06412-4.

The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis.

Wang Q, Luo M, Xiang B, et al.

Respiratory research 2020; (21(1)):55 doi:10.1186/s12931-020-1316-3.

Lymphangioleiomyomatosis.

Xu KF, Xu W, Liu S, et al.

Seminars in respiratory and critical care medicine 2020; (41(2)):256-268 doi:10.1055/s-0040-1702195.

Refractory chylous effusions in lymphangioleiomyomatosis patient post lung transplant.

Jacob S, Ali M, El-Sayed Ahmed MM, et al.

SAGE open medical case reports 2020; (8()):2050313X20921332 doi:10.1177/2050313X20921332.

Molecular logic of mTORC1 signalling as a metabolic rheostat.

Valvezan AJ, Manning BD

Nature metabolism 2019; (1(3)):321-333 doi:10.1038/s42255-019-0038-7.

Pregnancy in lymphangioleiomyomatosis: clinical and lung function outcomes in two national cohorts.

Taveira-DaSilva AM, Johnson SR, Julien-Williams P, et al.

Thorax 2020; (75(10)):904-907 doi:10.1136/thoraxjnl-2020-214987.

A woman with dyspnea and recurrent pneumothorax: when dyspnea is not asthma.

Chidharla A, Sehring M, Ascencio MA, Chittivelu S

Journal of community hospital internal medicine perspectives 2020; (10(4)):334-337 doi:10.1080/20009666.2020.1771125.

Effect of non-uniform cyst distribution in lymphangioleiomyomatosis on pulmonary function: a cross-sectional study.

Hasani AM, Matthew B, Goljamali M, et al.

The European respiratory journal 2021; (57(3)) doi:10.1183/13993003.03769-2020.

Structural insights into TSC complex assembly and GAP activity on Rheb.

Yang H, Yu Z, Chen X, et al.

Nature communications 2021; (12(1)):339 doi:10.1038/s41467-020-20522-4.

Methacholine reactivity in lymphangioleiomyomatosis is inversely related to FEV1 and VEGF-D.

Cassandro R, Elia D, Caminati A, et al.

The European respiratory journal 2021; (57(6)) doi:10.1183/13993003.04270-2020.

CrossTORC and WNTegration in Disease: Focus on Lymphangioleiomyomatosis.

Evans JF, Obraztsova K, Lin SM, Krymskaya VP

International journal of molecular sciences 2021; (22(5)) doi:10.3390/ijms22052233.

Chylous Ascites as a Presentation of Lymphangioleiomyomatosis.

McLain JH, Khadarian K, Shojaie L, et al.

ACG case reports journal 2021; (8(3)):e00517 doi:10.14309/crj.0000000000000517.

Pregnancy after the diagnosis of lymphangioleiomyomatosis (LAM).

Shen L, Xu W, Gao J, et al.

Orphanet journal of rare diseases 2021; (16(1)):133 doi:10.1186/s13023-021-01776-7.

Uncommon radiologic computed tomography appearances of the chest in patients with lymphangioleiomyomatosis.

Sekimoto Y, Suzuki K, Okura M, et al.

Scientific reports 2021; (11(1)):7170 doi:10.1038/s41598-021-85999-5.

Retroperitoneal Lympathic Malformations as First Presentation of Lymphangioleiomyomatosis.

Lenfant C, Dragean CA

Journal of the Belgian Society of Radiology 2021; (105(1)):32 doi:10.5334/jbsr.2468.

Tuberous sclerosis complex for the pulmonologist.

Rebaine Y, Nasser M, Girerd B, et al.

European respiratory review : an official journal of the European Respiratory Society 2021; (30(161)) doi:10.1183/16000617.0348-2020.

Lung Diseases Unique to Women.

Criner RN, Al-Abcha A, Lambert AA, Han MK

Clinics in chest medicine 2021; (42(3)):507-516 doi:10.1016/j.ccm.2021.04.014.

Cathepsin K is Superior to HMB45 for the Diagnosis of Pulmonary Lymphangioleiomyomatosis.

Rolim I, Makupson M, Lovrenski A, Farver C

Applied immunohistochemistry & molecular morphology : AIMM 2022; (30(2)):108-112 doi:10.1097/PAI.0000000000000968.

COVID-19 in a patient with sporadic lymphangioleiomyomatosis awaiting lung transplantation.

Noda Y, Shiroyama T, Amiya S, et al.

Respiratory medicine case reports 2021; (34()):101505 doi:10.1016/j.rmcr.2021.101505.

The TSC Complex-mTORC1 Axis: From Lysosomes to Stress Granules and Back.

Rehbein U, Prentzell MT, Cadena Sandoval M, et al.

Frontiers in cell and developmental biology 2021; (9()):751892 doi:10.3389/fcell.2021.751892.

A rare lung disease diagnosed by CT in a young pregnant woman.

Persad S, Dowlath N, Prasad M, Harry V

Clinical case reports 2021; (9(11)):e05071 doi:10.1002/ccr3.5071.

[Anxiety about Health of Patients Suffering from Epilepsy].

Roth-Rawald J, Friedrich J, Straub HB, Weck F

Psychotherapie, Psychosomatik, medizinische Psychologie 2022; (72(6)):243-249 doi:10.1055/a-1686-9789.

Lung-selective mRNA delivery of synthetic lipid nanoparticles for the treatment of pulmonary lymphangioleiomyomatosis.

Qiu M, Tang Y, Chen J, et al.

Proceedings of the National Academy of Sciences of the United States of America 2022; (119(8)) doi:10.1073/pnas.2116271119.

Pulmonary lymphangioleiomyomatosis (LAM): A literature overview and case report.

Cong CV, Anh TT, Ly TT, Duc NM

Radiology case reports 2022; (17(5)):1646-1655 doi:10.1016/j.radcr.2022.02.075.

Long-term clinical course and progression of lymphangioleiomyomatosis in a single lung transplant referral centre in Korea.

Chang S, Choi JS, Leem AY, et al.

Scientific reports 2022; (12(1)):8260 doi:10.1038/s41598-022-12314-1.

Sirolimus reduces the risk of pneumothorax recurrence in patients with lymphangioleiomyomatosis: a historical prospective self-controlled study.

Cheng C, Xu W, Wang Y, et al.

Orphanet journal of rare diseases 2022; (17(1)):257 doi:10.1186/s13023-022-02418-2.

Two most common diagnoses among myriads of cystic lung diseases.

Swol J, Würflein D, Böhm J, Ficker JH

Hippokratia 2021; (25(2)):83-86.

Prophylactic renal artery embolization before pregnancy in patients with lymphangioleiomyomatosis and renal angiomyolipoma.

Toei H, Zhang X, Seyama K, et al.

The Journal of international medical research 2022; (50(9)):3000605221123897 doi:10.1177/03000605221123897.

Lung transplantation for lymphangioleiomyomatosis.

Warrior K, Dilling DF

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 2023; (42(1)):40-52 doi:10.1016/j.healun.2022.09.021.

Clinical features and outcomes of male patients with lymphangioleiomyomatosis: A review.

Zhang H, Hu Z, Wang S, et al.

Medicine 2022; (101(52)):e32492 doi:10.1097/MD.0000000000032492.

Predicting Individualized Lung Disease Progression in Treatment-Naive Patients With Lymphangioleiomyomatosis.

Palipana AK, Gecili E, Song S, et al.

Chest 2023; (163(6)):1458-1470 doi:10.1016/j.chest.2022.12.027.

Determinants of Progression and Mortality in Lymphangioleiomyomatosis.

Xu W, Yang C, Cheng C, et al.

Chest 2023; (164(1)):137-148 doi:10.1016/j.chest.2023.02.026.

mTORC1 upregulates B7-H3/CD276 to inhibit antitumor T cells and drive tumor immune evasion.

Liu HJ, Du H, Khabibullin D, et al.

Nature communications 2023; (14(1)):1214 doi:10.1038/s41467-023-36881-7.

Scanxiety among Adults with Cancer: A Scoping Review to Guide Research and Interventions.

Derry-Vick HM, Heathcote LC, Glesby N, et al.

Cancers 2023; (15(5)) doi:10.3390/cancers15051381.

Lymphangioleiomyomatosis: where endocrinology, immunology and tumor biology meet.

Gibbons E, Minor BMN, Hammes SR

Endocrine-related cancer 2023; (30(9)).

Survey of maternal anxiety and perceptions towards foetal MRI and pre-scan education.

Plunkett S, Dobeli K, Prior M, Tusek X

Journal of medical radiation sciences 2024; (71(1)):10-20 doi:10.1002/jmrs.725.

Lymphangioleiomyomatosis with Tuberous Sclerosis Complex-A Case Study.

Marciniak A, Nawrocka-Rutkowska J, Brodowska A, et al.

Journal of personalized medicine 2023; (13(11)) doi:10.3390/jpm13111598.

A case report of lymphangioleiomyomatosis with retroperitoneal masses in pregnancy.

Zhu Y, Wang C, Ding J, et al.

Frontiers in medicine 2023; (10()):1313503 doi:10.3389/fmed.2023.1313503.

Complications of lymphangioleiomyomatosis in pregnancy: a case report and review of the literature.

Wang-Koehler E, Kern-Goldberger AR, Srinivas SK

AJOG global reports 2024; (4(1)):100309 doi:10.1016/j.xagr.2024.100309.

Case report: If it is not asthma-think of lymphangioleiomyomatosis in younger female patients.

Kirkeby MH, Bendstrup E, Rose HK

Frontiers in medicine 2024; (11()):1328471 doi:10.3389/fmed.2024.1328471.

Clinical, tomographic and functional comparison of sporadic and tuberous sclerosis complex-associated forms of lymphangioleiomyomatosis: a retrospective cohort study.

Oliveira MR, Wanderley M, Freitas CSG, et al.

ERJ open research 2024; (10(2)) doi:10.1183/23120541.00759-2023.

Epidemiology of patients with lymphangioleiomyomatosis: A descriptive study using the national database of health insurance claims and specific health checkups of Japan.

Kimura Y, Jo T, Hashimoto Y, et al.

Respiratory investigation 2024; (62(3)):494-502 doi:10.1016/j.resinv.2024.03.010.

Giant Renal Angiomyolipomas and Pulmonary Lymphangioleiomyomatosis: Follow-up Report after More than a Decade.

Nasir K, Ahmad A

Saudi journal of kidney diseases and transplantation : an official publication of the Saudi Center for Organ Transplantation, Saudi Arabia 2023; (34(6)):671-675 doi:10.4103/sjkdt.sjkdt_324_21.

Stepwise Incremental Dose Schedule of Sirolimus Is Successfully Tolerated by a Patient With Lymphangioleiomyomatosis Who Was Initially Allergic to mTOR Inhibitors.

Seyama K, Komiyama E, Tsuchihashi H, et al.

Cureus 2024; (16(4)):e58805 doi:10.7759/cureus.58805.

Clinical and Functional Outcomes Associated with Quality of Life in Patients with Lymphangioleiomyomatosis: A Cross-Sectional Study.

Queiroz DS, da Silva CCBM, Oliveira MR, et al.

Lung 2024; (202(6)):757-765 doi:10.1007/s00408-024-00751-w.

Immature Smooth Muscle Infiltration in Sporadic Lymphangioleiomyomatosis: A Case Study.

Stearns C, Muirhead D, Rosell B

South Dakota medicine : the journal of the South Dakota State Medical Association 2024; (77(11)):511-515.

Lymphangioleiomyomatosis and Pregnancy-Do We Have All the Answers for a Woman Who Desires to Conceive?-Literature Review.

Constantin AA, Gaburici AD, Malaescu AN, et al.

Cancers 2025; (17(2)) doi:10.3390/cancers17020323.

Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis.

Baldi BG, Feitosa PHR, Rubin AS, et al.

Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia 2025; (51(1)):e20240378 doi:10.36416/1806-3756/e20240378.

Application of sirolimus in an infant presenting with a life-threatening lymphatic malformation of the head and neck: a case report.

Yu S, Guo X

Frontiers in pediatrics 2025; (13()):1587330 doi:10.3389/fped.2025.1587330.

White Adipocyte Stem Cell Expansion Through Infant Formula Feeding: New Insights into Epigenetic Programming Explaining the Early Protein Hypothesis of Obesity.

Melnik BC, Weiskirchen R, John SM, et al.

International journal of molecular sciences 2025; (26(10)) doi:10.3390/ijms26104493.

Sporadic Lymphangioleiomyomatosis in a 35-Year-Old Female Patient: A Rare Cause of Spontaneous Pneumothorax.

Rodenko PN, Elefteratos J, Rodenko EL, et al.

Cureus 2025; (17(7)):e87346 doi:10.7759/cureus.87346.

Prevalence, severity, and modifiable predictors of scanxiety in patients undergoing routine oncologic imaging: a prospective longitudinal study.

Shah MS, Memon JA, Malik U, et al.

Clinical imaging 2025; (128()):110634 doi:10.1016/j.clinimag.2025.110634.

Benefits of different characteristics of pulmonary rehabilitation programs in patients with interstitial lung disease: a systematic review and meta-analysis.

Chen Q, Qiu Y, Xu M, Dong L

Annals of medicine 2025; (57(1)):2566868 doi:10.1080/07853890.2025.2566868.

Diagnostic Value of Serum VEGF-D in Lymphangioleiomyomatosis: Results of the First Prospective Study in the Russian Federation.

Makarova M, Baimakanova G, Belevskiy A, et al.

Diagnostics (Basel, Switzerland) 2026; (16(4)) doi:10.3390/diagnostics16040533.

TSC2, cathepsin K, and β-catenin as immunohistochemical markers for lymphangioleiomyomatosis.

Alfayez A, Belanger E, Cheung S, Churg A

Human pathology 2026; (174()):106142 doi:10.1016/j.humpath.2026.106142.

Ten-year follow-up of monozygotic twin sisters with TSC-LAM: A rare case report.

Fan L, Elia D, Cassandro R, Harari SA

Respiratory medicine case reports 2026; (62()):102436 doi:10.1016/j.rmcr.2026.102436.