443 results match your criteria Lymphangioleiomyomatosis Imaging


Differentiation Between Lymphangioleiomyomatosis and Birt-Hogg-Dubé Syndrome: Analysis of Pulmonary Cysts on CT Images.

AJR Am J Roentgenol 2019 Jan 23:1-7. Epub 2019 Jan 23.

2 Department of Pulmonary and Critical Care Medicine, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Republic of Korea.

Objective: The purposes of this study were to identify diagnostic imaging markers for differentiating pulmonary cysts in lymphangioleiomyomatosis and Birt-Hogg-Dubé syndrome and to identify potential risk factors for spontaneous pneumothorax in the two diseases.

Materials And Methods: This retrospective study included 44 patients with lymphangioleiomyomatosis (44 women; mean age, 35 ± 10.9 years) and 13 patients with Birt-Hogg-Dubé syndrome (nine men, four women; mean age, 45. Read More

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https://www.ajronline.org/doi/10.2214/AJR.18.20232
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http://dx.doi.org/10.2214/AJR.18.20232DOI Listing
January 2019
5 Reads
2.731 Impact Factor

Use of CT Scanning to Quantify Progression and Response to Treatment in LAM.

Chest 2019 Jan 17. Epub 2019 Jan 17.

Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD. Electronic address:

Background: In lymphangioleiomyomatosis (LAM), infiltration of the lungs with smooth muscle-like LAM cells results in cystic destruction and decline in lung function, effects stabilized by sirolimus therapy. LAM lung disease is followed, in part, by high-resolution CT scans. To obtain further information from these scans, we quantified changes in lung parenchyma by analyzing image "texture. Read More

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http://dx.doi.org/10.1016/j.chest.2019.01.004DOI Listing
January 2019
2 Reads

Lymphangioleiomyomatosis (LAM) presenting as recurrent pneumothorax in an infant with tuberous sclerosis: treated successfully with sirolimus.

BMJ Case Rep 2018 Nov 8;2018. Epub 2018 Nov 8.

Pediatrics, All India Institute of Medical Sciences, New Delhi, India.

Lymphangioleiomyomatosis (LAM) either sporadic or a part of tuberous sclerosis complex is rare in paediatric age group. Here, we report a case of LAM with tuberous sclerosis in an infant. She was referred to our institute at the age of 4 months as a case of recurrent bilateral pneumothorax requiring intercostal tube drainage. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22624
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http://dx.doi.org/10.1136/bcr-2018-226244DOI Listing
November 2018
9 Reads

Chest CT Scan at Radiation Dose of a Posteroanterior and Lateral Chest Radiograph Series: A Proof of Principle in Lymphangioleiomyomatosis.

Chest 2018 Oct 3. Epub 2018 Oct 3.

Cardiovascular and Pulmonary Branches, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD. Electronic address:

Background: Given the rising utilization of medical imaging and the risks of radiation, there is increased interest in reducing radiation exposure. The objective of this study was to evaluate, as a proof of principle, CT scans performed at radiation doses equivalent to that of a posteroanterior and lateral chest radiograph series in the cystic lung disease lymphangioleiomyomatosis (LAM).

Methods: From November 2016 to May 2018, 105 consecutive subjects with LAM received chest CT scans at standard and ultra-low radiation doses. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S00123692183248
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http://dx.doi.org/10.1016/j.chest.2018.09.007DOI Listing
October 2018
18 Reads

Immune Checkpoint Ligand PD-L1 Is Upregulated in Pulmonary Lymphangioleiomyomatosis.

Am J Respir Cell Mol Biol 2018 Dec;59(6):723-732

3 Penn Center for Pulmonary Biology, Pulmonary, Allergy and Critical Care Division, and.

Pulmonary lymphangioleiomyomatosis (LAM) is a slow-progressing metastatic disease that is driven by mutations in the tumor suppressor tuberous sclerosis complex 1/2 (TSC1/2). Rapamycin inhibits LAM cell proliferation and is the only approved treatment, but it cannot cause the regression of existing lesions and can only stabilize the disease. However, in other cancers, immunotherapies such as checkpoint blockade against PD-1 and its ligand PD-L1 have shown promise in causing tumor regression and even curing some patients. Read More

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https://www.atsjournals.org/doi/10.1165/rcmb.2018-0123OC
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http://dx.doi.org/10.1165/rcmb.2018-0123OCDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6293078PMC
December 2018
25 Reads

Smoking-Related Diffuse Cystic Lung Disease.

Chest 2018 Aug;154(2):e31-e35

Division of Pathology & Laboratory Medicine and Perinatal Institute, Division of Pulmonary Biology, Cincinnati Children's Hospital Medical Center, Cincinnati, OH; Department of Pathology & Laboratory Medicine, University of Cincinnati, Cincinnati, OH.

Exposure to cigarette smoke can lead to a variety of parenchymal lung diseases, including diffuse cystic lung diseases (DCLDs). Lymphangioleiomyomatosis (LAM) is the prototypical DCLD and has a characteristic appearance on high-resolution CT (HRCT). We present a series of four patients with DCLD on HRCT who were referred to our institution with a presumed diagnosis of LAM and who were found instead to have smoking-related injury of the small airways on histopathological analysis. Read More

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http://dx.doi.org/10.1016/j.chest.2018.02.027DOI Listing
August 2018
5 Reads

Thoracoabdominal imaging of tuberous sclerosis.

Pediatr Radiol 2018 08 4;48(9):1307-1323. Epub 2018 Aug 4.

Department of Radiology, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave., MLC 5031, Cincinnati, OH, 45229, USA.

Imaging of tuberous sclerosis complex has rapidly evolved over the last decade in association with increased understanding of the disease process and new treatment modalities. Tuberous sclerosis complex is best known for the neurological symptoms and the associated neuroimaging findings, and children with tuberous sclerosis complex require active surveillance of associated abnormalities in the chest, abdomen and pelvis. Common findings that require regular imaging surveillance are angiomyolipomas in the kidneys and lymphangioleiomyomatosis in the chest. Read More

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http://dx.doi.org/10.1007/s00247-018-4123-yDOI Listing
August 2018
3 Reads

[F]Fluorocholine and [F]Fluoroacetate PET as Imaging Biomarkers to Assess Phosphatidylcholine and Mitochondrial Metabolism in Preclinical Models of TSC and LAM.

Clin Cancer Res 2018 Dec 27;24(23):5925-5938. Epub 2018 Jul 27.

Pulmonary and Critical Care Medicine, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.

Purpose: Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by inactivating mutations of the or gene, characterized by neurocognitive impairment and benign tumors of the brain, skin, heart, and kidneys. Lymphangioleiomyomatosis (LAM) is a diffuse proliferation of α-smooth muscle actin-positive cells associated with cystic destruction of the lung. LAM occurs almost exclusively in women, as a TSC manifestation or a sporadic disorder ( somatic mutations). Read More

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http://clincancerres.aacrjournals.org/lookup/doi/10.1158/107
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http://dx.doi.org/10.1158/1078-0432.CCR-17-3693DOI Listing
December 2018
13 Reads

Have Noninvasive Imaging Studies Supplanted the Need for Invasive Hemodynamics: Lessons Learned from Lymphangioleiomyomatosis.

Authors:
Joel A Strom

J Am Soc Echocardiogr 2018 Aug 3;31(8):902-904. Epub 2018 Jul 3.

Florida Polytechnic University, Lakeland, Florida. Electronic address:

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http://dx.doi.org/10.1016/j.echo.2018.06.003DOI Listing
August 2018
3 Reads

Lymphangioleiomyomatosis.

N Engl J Med 2018 Jun;378(23):2224

Beth Israel Deaconess Medical Center, Boston, MA.

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http://dx.doi.org/10.1056/NEJMicm1712581DOI Listing
June 2018
4 Reads

Hemodynamic Mechanisms of Exercise-Induced Pulmonary Hypertension in Patients with Lymphangioleiomyomatosis: The Role of Exercise Stress Echocardiography.

J Am Soc Echocardiogr 2018 Aug 28;31(8):888-901. Epub 2018 Mar 28.

Unità Operativa di Pneumologia e Terapia Semi-Intensiva Respiratoria - Servizio di Fisiopatologia Respiratoria e Emodinamica Polmonare, Ospedale San Giuseppe Multimedica IRCCS, Milano, Italy.

Background: The pathogenesis of pulmonary hypertension (PH) in lymphangioleiomyomatosis (LAM) has not yet been completely clarified. The aim of this study was to conduct a noninvasive evaluation of the main hemodynamic mechanisms of exercise-induced PH in patients with LAM, assessed using exercise stress echocardiography.

Methods: Fifteen patients with LAM (mean age, 47 ± 13 years; all women) without resting PH were enrolled in a prospective single-center study and compared with 15 healthy female control subjects (mean age, 45. Read More

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http://dx.doi.org/10.1016/j.echo.2018.02.004DOI Listing
August 2018
33 Reads

Assessing the outcomes of everolimus on renal angiomyolipoma associated with tuberous sclerosis complex in China: a two years trial.

Orphanet J Rare Dis 2018 Mar 27;13(1):43. Epub 2018 Mar 27.

Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 1 Shuaifuyuan Road, Beijing, 100730, China.

Background: Tuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disorder characterized by the development of numerous benign tumors. Renal angiomyolipoma (RAML) occur in up to 80% of TSC patients, which is a leading cause of TSC-related death in adult patients. The aim of the study was to evaluate the efficacy and safety profiles of everolimus in Chinese patients of TSC associated with RAML(TSC-RAML). Read More

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https://ojrd.biomedcentral.com/articles/10.1186/s13023-018-0
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http://dx.doi.org/10.1186/s13023-018-0781-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5870799PMC
March 2018
10 Reads

Mounier-Kuhn Syndrome Mimicking Lymphangioleiomyomatosis.

Chest 2018 Feb;153(2):e19-e23

Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.

We present the case of a man with Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, who was referred to the National Institutes of Health Clinical Research Center because of a potential diagnosis of lymphangioleiomyomatosis (LAM), a rare condition in men. The patient was evaluated using ongoing protocols and provided written informed consent. The case demonstrates the presence of chronic inflammation surrounding the dilated airways and histologic changes of the lung parenchyma with emphysematouslike disruption in areas adjacent to the dilated airways. Read More

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http://dx.doi.org/10.1016/j.chest.2017.10.024DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6026286PMC
February 2018
2 Reads

Superselective Transcatheter Arterial Embolization for Large Unruptured Renal Angiomyolipoma in Lymphangioleiomyomatosis.

J Vasc Interv Radiol 2018 07 3;29(7):958-965. Epub 2018 Feb 3.

Department of Radiology, School of Medicine, Juntendo University, 2-1-1 Hongo Bunkyo-ku, Tokyo 113-8421, Japan.

Purpose: To retrospectively evaluate therapeutic performance and complications of superselective transcatheter arterial embolization (TAE) for unruptured renal angiomyolipoma (AML) in patients with lymphangioleiomyomatosis (LAM) and to investigate the correlation between percentage reduction in tumor volume and intratumoral fat content.

Materials And Methods: Superselective TAE was performed in 14 consecutive patients with 15 large unruptured renal AMLs associated with LAM (mean age, 38 y; range, 21-57 y). Patients had renal AML with aneurysms ≥ 5 mm in diameter, tumor-related abdominal symptoms, or both. Read More

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http://dx.doi.org/10.1016/j.jvir.2017.11.003DOI Listing
July 2018
6 Reads

Clinical Course of Histologically Proven Multifocal Micronodular Pneumocyte Hyperplasia in Tuberous Sclerosis Complex: A Case Series and Comparison with Lymphangiomyomatosis.

Respiration 2018;95(5):310-316. Epub 2018 Jan 24.

First Department of Medicine, Hokkaido University School of Medicine, Sapporo, Japan.

Background: Multifocal micronodular pneumocyte hyperplasia (MMPH) is a rare pulmonary manifestation of tuberous sclerosis complex (TSC). Because of its rarity, no previous study has described the detailed clinical course of this disease.

Objectives: This study aimed to clarify the longitudinal clinical characteristics of subjects with MMPH. Read More

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http://dx.doi.org/10.1159/000486101DOI Listing
October 2018
4 Reads

Pulmonary lymphangioleiomyomatosis and fertility treatment.

QJM 2018 Feb;111(2):123-124

From the Division of Pulmonary Medicine, Thoracic Center, St. Luke's International Hospital, Tokyo, Japan.

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https://academic.oup.com/qjmed/article/111/2/123/4705875
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http://dx.doi.org/10.1093/qjmed/hcx238DOI Listing
February 2018
3 Reads

Difference of the progression of pulmonary cysts assessed by computed tomography among COPD, lymphangioleiomyomatosis, and Birt-Hogg-Dubé syndrome.

PLoS One 2017 8;12(12):e0188771. Epub 2017 Dec 8.

Divisions of Respiratory Medicine, Juntendo University Faculty of Medicine & Graduate School of Medicine, Bunkyo-Ku, Tokyo, Japan.

Many groups developed the methods to quantitatively analyze low attenuation area (LAA) on chest CT in patients with cystic lung diseases. Especially in COPD, it was reported that the cumulative size distribution of LAA clusters follows a power law characterized by the exponent D, which reflect the fractal dimension of terminal airspace geometry. We hypoyhesized that the quantitative charateristics of LAA clusters including fractal property might indicate the different features of the progression of cysts in cystic lung diseases. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0188771PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5722335PMC
December 2017
17 Reads

Durability of Sirolimus for Lymphangioleiomyomatosis.

Am J Med Sci 2017 12 5;354(6):603-607. Epub 2016 Dec 5.

Department of Internal Medicine (AM, LC), University of Texas Medical Branch, Galveston, Texas; Department of Internal Medicine, Division of Pulmonary Critical Care & Sleep Medicine (SS, JV), University of Texas Medical Branch, Galveston, Texas; Department of Internal Medicine, Division of Pulmonary, Allergy, & Critical Care Medicine (VV), University of Alabama at Birmingham, Birmingham, Alabama.

Lymphangioleiomyomatosis (LAM), a rare, multisystem disorder primarily affecting women of reproductive age, is characterized by cystic-appearing lung lesions, progressive loss of lung function, chylous effusions and renal angiomyolipomas. Sirolimus, an mammalian target of rapamycin inhibitor, has been shown to stabilize lung function, reduce symptoms and resolve chylous effusions in the short term for patients with LAM. Herein, we report a premenopausal female with LAM who experienced complete and durable resolution of her chylothoraces with significant and sustained improvement in lung function on sirolimus. Read More

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http://dx.doi.org/10.1016/j.amjms.2016.11.028DOI Listing
December 2017
4 Reads

Emerging biomarkers of lymphangioleiomyomatosis.

Expert Rev Respir Med 2018 02 1;12(2):95-102. Epub 2017 Dec 1.

a Pulmonary and Critical Care Medicine, Department of Medicine , Brigham and Women's Hospital and Harvard Medical School , Boston , MA , USA.

Introduction: Lymphangioleiomyomatosis (LAM) is a destructive lung disease affecting primarily women. LAM is caused by inactivating mutations in the tuberous sclerosis complex (TSC) genes, resulting in hyperactivation of mechanistic/mammalian target of rapamycin complex 1 (mTORC1). Over the past five years, there have been remarkable advances in the diagnosis and therapy of LAM, including the identification of vascular endothelial growth factor D (VEGF-D) as a diagnostic biomarker and the US Food and Drug Administration approval of sirolimus as therapy for LAM. Read More

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https://www.tandfonline.com/doi/full/10.1080/17476348.2018.1
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http://dx.doi.org/10.1080/17476348.2018.1409622DOI Listing
February 2018
11 Reads

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

Respiration 2017 23;94(6):467-485. Epub 2017 Nov 23.

Cystic lung diseases constitute a distinct group of rare lung disorders, among which two result from monogenic defects affecting tumor suppressor genes: lymphangioleiomyomatosis, either sporadic or associated with tuberous sclerosis complex, and Birt-Hogg-Dubé syndrome. These disorders have similarities in their clinical expression, including occurrence in young adults, multiple pulmonary cysts, recurrent pneumothorax, skin hamartomas, and renal tumors. However, they markedly differ in their gender distribution, pathogenesis, disease course, and prognosis. Read More

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http://dx.doi.org/10.1159/000485106DOI Listing
August 2018
3 Reads

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.

Am J Respir Crit Care Med 2017 11;196(10):1337-1348

Background: Recommendations regarding key aspects related to the diagnosis and pharmacological treatment of lymphangioleiomyomatosis (LAM) were recently published. We now provide additional recommendations regarding four specific questions related to the diagnosis of LAM and management of pneumothoraces in patients with LAM.

Methods: Systematic reviews were performed and then discussed by a multidisciplinary panel. Read More

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http://dx.doi.org/10.1164/rccm.201709-1965STDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5694834PMC
November 2017
24 Reads

Photoclinic.

Arch Iran Med 2017 Sep;20(9):621-622

Department of Internal Medicine, Shariati Hospital, Tehran University of Medical Sciences, Tehran, I. R. of Iran.

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http://dx.doi.org/0172009/AIM.0011DOI Listing
September 2017
4 Reads

New insights in lymphangioleiomyomatosis and pulmonary Langerhans cell histiocytosis.

Eur Respir Rev 2017 Sep 27;26(145). Epub 2017 Sep 27.

U.O. di Pneumologia e Terapia Semi-Intensiva Respiratoria, Servizio di Fisiopatologia Respiratoria ed Emodinamica Polmonare, Ospedale San Giuseppe, MultiMedica IRCCS, Milan, Italy

Lymphangioleiomyomatosis (LAM) and pulmonary Langerhans cell histiocytosis (PLCH) are rare diseases that lead to progressive cystic destruction of the lungs. Despite their distinctive characteristics, these diseases share several features. Patients affected by LAM or PLCH have similar radiological cystic patterns, a similar age of onset, and the possibility of extrapulmonary involvement. Read More

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http://dx.doi.org/10.1183/16000617.0042-2017DOI Listing
September 2017
7 Reads

MR imaging findings of vertebral involvement in Gorham-Stout disease, generalized lymphatic anomaly, and kaposiform lymphangiomatosis.

Jpn J Radiol 2017 Oct 9;35(10):606-612. Epub 2017 Aug 9.

Department of Radiology, Gifu University School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan.

Purpose: To assess the MR imaging findings of vertebral involvement in Gorham-Stout disease (GSD), generalized lymphatic anomaly (GLA), and kaposiform lymphangiomatosis (KLA).

Methods: Nine patients (three patients with GSD, three with GLA, and three with KLA) who underwent whole-spine MR examinations were included. MR findings of fatty marrow replacement and prolonged T1 and T2 lesions of the vertebrae were retrospectively assessed. Read More

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http://dx.doi.org/10.1007/s11604-017-0674-3DOI Listing
October 2017
17 Reads

Lymphangioleiomyomatosis, multifocal micronodular pneumocyte hyperplasia, and sarcoidosis: more pathological findings in the same chest CT, or a single pathological pathway?

BMC Pulm Med 2017 Jul 28;17(1):107. Epub 2017 Jul 28.

Respiratory Unit, Ospedale San Paolo, Department of Health Sciences, Università degli Studi di Milano, Milan, Italy.

Background: Autoimmune hepatitis/primary biliary cirrhosis overlap syndrome, lymphangioleiomyomatosis/tuberous sclerosis complex (LAM-TSC), and sarcoidosis are three rare diseases. Here we present, to the best of our knowledge, the first description of a patient with the coexistence of these three diseases.

Case Presentation: A 47-year-old woman affected by LAM-TSC and primary biliary cirrosis/autoimmune hepatitis overlap syndrome. Read More

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http://dx.doi.org/10.1186/s12890-017-0447-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5534042PMC
July 2017
27 Reads

Pneumothorax caused by cystic and nodular lung metastases from a malignant uterine perivascular epithelioid cell tumor (PEComa).

Respir Med Case Rep 2017 27;22:77-82. Epub 2017 Jun 27.

Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3 Hongo, Bunkyo-ku, Tokyo, 113-8431, Japan.

Perivascular epithelioid cell tumors (PEComas) are mesenchymal neoplasms with immunoreactivity for both melanocytic and smooth muscle markers. PEComas occur at multiple sites, and malignant PEComas can undergo metastasis, recurrence and aggressive clinical courses. Although the lung is a common metastatic site of PEComas, they usually appear as multiple nodules but rarely become cystic or cavitary. Read More

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http://dx.doi.org/10.1016/j.rmcr.2017.06.011DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5496452PMC
June 2017
30 Reads

Active Surveillance of Nonfatty Renal Masses in Patients With Lymphangioleiomyomatosis: Use of CT Features and Patterns of Growth to Differentiate Angiomyolipoma From Renal Cancer.

AJR Am J Roentgenol 2017 Sep 5;209(3):611-619. Epub 2017 Jul 5.

2 Cardiovascular and Pulmonary Branch, NHLBI, NIH, Bethesda, MD.

Objective: The objective of this study was to report our experience with active surveillance of nonfatty renal masses in a large cohort of patients with lymphangioleiomyomatosis (LAM), correlate their CT features and patterns of growth with histopathology results, and provide guidelines for management.

Subjects And Methods: Yearly CT examinations were performed of 367 women (age range, 21-75 years; mean age, 47 years). For the 31 patients with 37 nonfatty renal masses that were biopsied, excised, or followed for ≥ 5 years, CT enhancement characteristics and patterns of growth were compared with the histopathology results. Read More

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http://dx.doi.org/10.2214/AJR.16.17530DOI Listing
September 2017
13 Reads

Two Cases of Tuberous Sclerosis Complex Suggestive of Complicating Multifocal Micronodular Pneumocyte Hyperplasia: A Case Report.

J UOEH 2017;39(2):133-141

Department of Respiratory Medicine, School of Medicine, University of Occupational and Environmental Health.

Multifocal micronodular pneumocyte hyperplasia (MMPH) is pathologically characterized by multifocal nodular hyperplasia of type Ⅱ pneumocyte-like cells. MMPH is usually complicated with tuberous sclerosis complex (TSC). MMPH patients tend to be asymptomatic or only slightly symptomatic. Read More

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http://dx.doi.org/10.7888/juoeh.39.133DOI Listing
October 2017
23 Reads

Lymphangioleiomyomatosis: an explosive presentation of a rare disease.

Oxf Med Case Reports 2017 Jun 7;2017(6):omx023. Epub 2017 Jun 7.

Pulmonary and Critical Care Medicine, University of California San Francisco Fresno, 155 N Fresno Street, Fresno, CA 93701, USA.

Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease, commonly affecting women in the reproductive age group. Exacerbation of pre-existing disease is common during pregnancy likely due to the up-regulation of estrogen and progesterone receptors present within the proliferating smooth muscle cells. This case highlights a dramatic presentation of LAM for the first time in pregnancy, its rapid progression during gestation, and a partial resolution with delivery. Read More

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http://dx.doi.org/10.1093/omcr/omx023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5461433PMC
June 2017
2 Reads

Renal Epithelioid Angiomyolipoma Associated with Pulmonary Lymphangioleiomyomatosis: Imaging Findings.

J Clin Imaging Sci 2017 22;7:18. Epub 2017 May 22.

Department of Clinical Radiology, Medical School, University of Ioannina, University Campus, Ioannina, Greece.

Renal angiomyolipomas (AMLs) and pulmonary lymphangioleiomyomatosis (LAM) are the most common tumors of the perivascular epithelioid cell (PEComa) family. Both may be associated with tuberous sclerosis (TS) complex. Epithelioid AML (EAML) is a rare variety of AMLs, with a potential aggressive behavior. Read More

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http://dx.doi.org/10.4103/jcis.JCIS_14_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5450460PMC
May 2017
24 Reads

A Phase II Clinical Trial of an Aromatase Inhibitor for Postmenopausal Women with Lymphangioleiomyomatosis.

Ann Am Thorac Soc 2017 Jun;14(6):919-928

12 Department of Internal Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati, Cincinnati, Ohio.

Rationale: Lymphangioleiomyomatosis (LAM) is a progressive cystic lung disease that predominantly affects women and can worsen with pregnancy, estrogen treatment, and the menstrual cycle, suggesting an important role for estrogen in disease pathogenesis.

Objectives: To assess the efficacy and safety of the aromatase inhibitor letrozole in the treatment of LAM.

Methods: Seventeen postmenopausal women with LAM were enrolled in this phase II trial and randomized to receive letrozole 2. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201610-824OCDOI Listing
June 2017
34 Reads

Abdominal Lymphangiomatosis With Intestinal Lymphangiectasia Diagnosed by Magnetic Resonance Lymphangiography: A Case Report.

Curr Probl Diagn Radiol 2018 May - Jun;47(3):200-202. Epub 2017 Apr 12.

Department of Radiodiagnosis, All India Institute of Medical Sciences, Ansari Nagar, New Delhi, India.

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http://dx.doi.org/10.1067/j.cpradiol.2017.04.006DOI Listing
August 2018
8 Reads

Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A Report of Two Cases.

Intern Med 2017 15;56(8):943-948. Epub 2017 Apr 15.

Division of Respiratory Medicine, Juntendo University Faculty of Medicine and Graduate School of Medicine, Japan.

This report describes two patients with sporadic lymphangioleiomyomatosis complicated by protein-losing enteropathy (PLE). Imaging studies indicated retroperitoneal lymphangioleiomyomas and abnormalities of the adjacent digestive tract. Endoscopic mucosal biopsy revealed colonic lymphangiectasia in one patient; whereas the site in the other patient was intestinal. Read More

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https://www.jstage.jst.go.jp/article/internalmedicine/56/8/5
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http://dx.doi.org/10.2169/internalmedicine.56.7769DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5465412PMC
June 2017
13 Reads

Delayed Presentation of Tuberour Sclerosis Complex in Adult Women.

J La State Med Soc 2017 Mar-Apr;169(2):46. Epub 2017 Apr 15.

Department of Internal Medicine, University Hospitals and Clinics - LSU Health in Lafayette, LA.

Introduction: Tuberous sclerosis complex (TSC); is an autosomal dominant disorder characterized by the formation of hamartomatous lesions in multiple organs, with a birth incidence of around one in 10,000. Although it usually manifests itself in early life, we present a case of an adult woman who we diagnosed with TSC.

Case: A 27 year old woman presented to Emergency Department with worsening right flank pain and progressive dyspnea. Read More

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September 2017
5 Reads

Retroperitoneal lymphangioleiomyoma with lymph node involvement: A pathologic-radiologic correlation of a rare form of myomelanocytic tumor.

Ann Diagn Pathol 2017 Apr 1;27:69-73. Epub 2017 Feb 1.

Department of Pathology, Medical College of Wisconsin, Milwaukee, WI, USA. Electronic address:

Lymphangioleiomyomatosis (LAM) is a rare and slowly progressive disorder that usually arises in the lung, affects exclusively women in their childbearing years, and typically presents with progressive dyspnea on exertion and pneumothorax. Infrequently, extra-pulmonary LAM can occur in the retroperitoneum, uterine wall, mediastinum and intraperitoneal lymph nodes. Histologically, LAM is characterized by a proliferation of perivascular epithelioid cells (PEC) that express markers for both melanocytes and smooth muscle cells. Read More

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http://dx.doi.org/10.1016/j.anndiagpath.2017.01.008DOI Listing
April 2017
15 Reads

Imaging of tuberous sclerosis complex: a pictorial review.

Radiol Bras 2017 Jan-Feb;50(1):48-54

MD, PhD, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro, RJ, Brazil.

Tuberous sclerosis complex (TSC) is a genetically determined hamartomatous neurocutaneous disease with high phenotypic variability. TSC is characterized by widespread hamartomas and benign, or rarely malignant, neoplasms distributed in several organs throughout the body, especially in the brain, skin, retina, kidney, heart, and lung. Common manifestations include cortical tubers, subependymal nodules, white matter abnormalities, retinal abnormalities, cardiac rhabdomyoma, lymphangioleiomyomatosis, renal angiomyolipoma, and skin lesions. Read More

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http://dx.doi.org/10.1590/0100-3984.2016.0020DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5347503PMC
March 2017
9 Reads

Computed tomography lymphangiography findings in 27 cases of lymphangioleiomyomatosis.

Acta Radiol 2017 Nov 29;58(11):1342-1348. Epub 2017 Jan 29.

1 Department of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing, PR China.

Background Lymphangioleiomyomatosis (LAM) is a rare disease involving the bronchi, lymphatic vessels, and veins. However, there are few reports about lymphatic vessel abnormalities associated with LAM. Purpose To evaluate computed tomography (CT) lymphangiography findings in cases of LAM, especially lymphatic vessel abnormalities. Read More

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http://dx.doi.org/10.1177/0284185116688381DOI Listing
November 2017
8 Reads

[A rare form of obstructive pulmonary disease].

Ned Tijdschr Geneeskd 2016;160:D757

Erasmus MC, Rotterdam.

Background: Lymphangioleiomyomatosis (LAM) is characterised by progressive dyspnoea, spontaneous pneumothorax and cystic pulmonary destruction. The disease may show similarities with emphysema clinically, radiologically and on lung function tests.

Case Description: A 44-year-old woman was referred for lung transplantation because of a 6-year history of dyspnoea and severe obstructive pulmonary function disorder with decreased diffusion capacity. Read More

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February 2018

[IMPORTANCE OF SCREENING FOR LUNG LYMPHANGIOLEIOMYOMATOSIS AMONG RENAL ANGIOMYOLIPOMA CASES].

Nihon Hinyokika Gakkai Zasshi 2017 ;108(4):188-193

Department of Urology, Toranomon Hospital.

(Background) Multiple renal angiomyolipoma (AML) may develop concurrently with tuberous sclerosis complex (TSC) and lung lymphangioleiomyomatosis (LAM). In recent years, an increase in subjects undergoing chest computed tomography examinations, including medical examinations, has been accompanied by an increase in LAM cases that are diagnosed while still asymptomatic. Surgical treatment may also be performed for renal AML to prevent bleeding. Read More

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http://dx.doi.org/10.5980/jpnjurol.108.188DOI Listing
January 2017
2 Reads

Multi-modal imaging features and lympho-venous shunt for vaginal chylous fistula in lymphangioleiomyomatosis: Case report and review.

Lymphology 2017 ;50(3):136-140

Department of LymphaticSurgery, Beijing Shijitan Hospital, Capital Medical University, Beijing, China.

Vaginal chylous fistula is a very rare lymphatic disorder and lymphangioleiomyomatosis (LAM) is also a rare multisystemic disease. LAM with the development of a vaginal chylous fistula has rarely been reported. Here we report a case of vaginal chylous fistula accompanied with LAM. Read More

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January 2017

Lymphangioleiomyomatosis and Gorham-Stout disease: Primary or secondary disorders of the lymphatic system?

Authors:
M H Witte

Lymphology 2017 ;50(3):114-119

Department of Surgery, University of Arizona, Tucson, Arizona USA.

Lymphangioleimyomatosis and Gorham- Stout disease, rare disorders which share features of proliferating lymphatic vessels, predominantly in the lung in the former and bone in the latter, commonly manifest as progressive lung disease often associated with life-threatening complications of chylous reflux from central lymphatic obstruction/ leakage. This Lymphspiration-Editorial proposes that, rather than a secondary complication, lymphatic obstruction/ dysfunction is a much earlier or even the primary event in the pathogenesis of both disorders and that lymphostasis drives the cellular proliferative response in both lung and bone and even distant sites. Read More

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January 2017

Lung cancer in a patient with lymphangioleiomyomatosis.

Arch Bronconeumol 2017 Jun 13;53(6):342. Epub 2016 Dec 13.

Servicio de Oncología Médica, Hospital Universitario Ramón y Cajal, Madrid, España.

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http://dx.doi.org/10.1016/j.arbres.2016.11.005DOI Listing

Differing Presentations of Familial Tuberous Sclerosis.

J Assoc Physicians India 2016 09;64(9):74

Assistant Professor, Department of Radiology, Aarupadai Veedu Medical College, Puducherry.

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September 2016
2 Reads

Chest Computed Tomographic Image Screening for Cystic Lung Diseases in Patients with Spontaneous Pneumothorax Is Cost Effective.

Ann Am Thorac Soc 2017 Jan;14(1):17-25

3 Division of General Internal Medicine, Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio.

Rationale: Patients without a known history of lung disease presenting with a spontaneous pneumothorax are generally diagnosed as having primary spontaneous pneumothorax. However, occult diffuse cystic lung diseases such as Birt-Hogg-Dubé syndrome (BHD), lymphangioleiomyomatosis (LAM), and pulmonary Langerhans cell histiocytosis (PLCH) can also first present with a spontaneous pneumothorax, and their early identification by high-resolution computed tomographic (HRCT) chest imaging has implications for subsequent management.

Objectives: The objective of our study was to evaluate the cost-effectiveness of HRCT chest imaging to facilitate early diagnosis of LAM, BHD, and PLCH. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201606-459OCDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5461992PMC
January 2017
10 Reads
5 Citations

[Diagnosis and treatment for 2 cases of tuberous sclerosis complex with pulmonary lymphangioleiomyomatosis].

Zhong Nan Da Xue Xue Bao Yi Xue Ban 2016 Jul;41(7):771-6

Department of Respiratory Medicine, Second Xiangya Hospital, Central South University, Changsha 410011, China.

To improve the diagnosis and treatment for tuberous sclerosis complex (TSC) with pulmonary lymphangioleiomyomatosis, a retrospective analysis was performed based on the clinical data of 2 patients with such disease. Both of them have typical thin-walled cystic lesion throughout the lung field, renal angioleiomyolipoma, and various degrees of skin lesions. Central nervous system is involved in one patient. Read More

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http://www.csumed.org/xbwk/CN/10.11817/j.issn.1672-7347.2016
Publisher Site
http://dx.doi.org/10.11817/j.issn.1672-7347.2016.07.018DOI Listing
July 2016
5 Reads

[Sporadic Lymphangioleiomyomatosis (sLAM) and Tuberous Sclerosis Complex (TSC) - Pulmonary Manifestations].

Pneumologie 2017 Feb 1;71(2):86-95. Epub 2016 Sep 1.

Med. Klinik m. S. Nephrologie, Charité Universitätsmedizin Berlin, Berlin.

Lymphangioleiomyomatosis (LAM) is a rare multi-system disorder affecting predominantly women of childbearing age. The disease entity is divided in sporadic LAM (sLAM) and LAM associated with tuberous sclerosis complex (TSC). In up to 50 % of female TSC-patients pulmonary involvement (TSC-LAM) can be found, with first clinical symptoms usually starting between 25 and 30 years of age. Read More

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http://dx.doi.org/10.1055/s-0042-111522DOI Listing
February 2017
2 Reads

Coexistence of pulmonary lymphangioleiomyomatosis and pulmonary angiomyolipoma.

BMC Pulm Med 2016 08 15;16(1):120. Epub 2016 Aug 15.

Department of Respiratory Medicine, Peking Union Medical College Hospital, Beijing, 100730, China.

Background: Lymphangioleiomyomatosis (LAM) and angiomyolipoma are two different, but related rare diseases. To the best of our knowledge, pulmonary LAM and pulmonary angiomyolipoma have not previously been observed in the same patient.

Case Presentation: A 38-year-old woman presented with a dry cough and left flank pain. Read More

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http://dx.doi.org/10.1186/s12890-016-0286-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4986257PMC
August 2016
3 Reads

Nonmalignant Adult Thoracic Lymphatic Disorders.

Clin Chest Med 2016 09;37(3):409-20

Division of Pulmonary, Critical Care and Sleep Medicine, University of Cincinnati, MSB 6165, 231 Albert Sabin Way, Cincinnati, OH 45267-0564, USA.

The thoracic lymphatic disorders are a heterogeneous group of uncommon conditions that are associated with thoracic masses, interstitial pulmonary infiltrates, and chylous complications. Accurate diagnosis of the thoracic lymphatic disorders has important implications for the newest approaches to management, including embolization and treatment with antilymphangiogenic drugs. New imaging techniques to characterize lymphatic flow, such as dynamic contrast-enhanced magnetic resonance lymphangiogram, are redefining approaches to disease classification and therapy. Read More

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http://dx.doi.org/10.1016/j.ccm.2016.04.004DOI Listing
September 2016
3 Reads