468 results match your criteria Lymphangioleiomyomatosis Imaging


A case of lymphangioleiomyomatosis with diffuse large B-cell lymphoma: Usefulness of FDG-PET.

Respir Med Case Rep 2020 16;29:100999. Epub 2020 Jan 16.

Department of Respiratory Medicine, Tokyo Women's Medical University, Japan.

Lymphangioleiomyomatosis (LAM) is characterized by cystic lung disease, abdominal tumor and involvement of the axial lymph nodes. We report a very rare case of LAM with malignant lymphoma. A 51-year-old female had medical history of recurrent pneumothorax and nephrectomy for a left renal angiomyolipoma and was diagnosed with LAM by video-assisted thoracoscopic surgery at the age of 30. Read More

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http://dx.doi.org/10.1016/j.rmcr.2020.100999DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7118407PMC
January 2020

Lymphangioleiomyomatosis revealed by Ga-NOTA-Evans Blue PET/CT.

Eur J Nucl Med Mol Imaging 2020 Jan 10. Epub 2020 Jan 10.

Department of Nuclear Medicine, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, 100730, China.

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http://dx.doi.org/10.1007/s00259-020-04685-2DOI Listing
January 2020

Detection of secondary causes of spontaneous pneumothorax: Comparison between computed tomography and chest X-ray.

Diagn Interv Imaging 2020 Apr 18;101(4):217-224. Epub 2019 Dec 18.

Department of Respiratory Medicine, AP-HP, hôpital Tenon, 75020 Paris, France; Thoracic Surgery Department, AP-HP, hôpital Tenon, 75020 Paris, France; Thoracic Oncology Department, Paris-Diderot University, AP-HP, hôpital Bichat, 75018 Paris, France. Electronic address:

Purpose: The aim of this study was to compare the effectiveness of chest X-ray to that of thoracic computed tomography (CT) for the detection of the causes of secondary spontaneous pneumothorax (SP).

Methods: A prospective cohort of patients with SP was studied. All chest X-ray and CT examinations of the patients were reviewed retrospectively by an expert radiologist blinded to clinical data. Read More

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http://dx.doi.org/10.1016/j.diii.2019.11.002DOI Listing

Mutation spectrums of TSC1 and TSC2 in Chinese women with lymphangioleiomyomatosis (LAM).

PLoS One 2019 19;14(12):e0226400. Epub 2019 Dec 19.

Department of Pulmonary and Critical Care Medicine, The First Affiliated Hospital of Guangzhou Medical University, Guangzhou, Guangdong, China.

The aim of our study was to elucidate the landscapes of genetic alterations of TSC1 and TSC2 as well as other possible non-TSC1/2 in Lymphangioleiomyomatosis (LAM) patients. Sixty-one Chinese LAM patients' clinical information was collected. Tumor biopsies and matched leukocytes from these patients were retrospectively analyzed by next generation sequencing (NGS), chromosomal microarray analysis (CMA), and multiplex ligation-dependent probe amplification (MLPA). Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0226400PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6922431PMC

Clinical CT underestimation of the percentage volume occupied by cysts in patients with lymphangioleiomyomatosis.

Clin Imaging 2020 Feb 27;59(2):119-125. Epub 2019 Nov 27.

National Heart, Lung and Blood Institute, National Institutes of Health, Bethesda, MD 20892, USA. Electronic address:

Purpose: To evaluate the accuracy of cyst score measurements by standard high-resolution helical volume chest CT (HRCT) in patients with lymphangioleiomyomatosis (LAM), using a short z-length ultra-high resolution re-scan (UH re-scan) as the reference. In cystic lung diseases, cyst score is derived from CT scans and defined as the percentage of the total lung parenchymal volume occupied by cysts, a biomarker which measures the severity of the disease.

Methods: In a prospective study of 73 LAM patients, each patient received the standard HRCT chest scan and a short z-length UH re-scan. Read More

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http://dx.doi.org/10.1016/j.clinimag.2019.11.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6941194PMC
February 2020

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

Eur Radiol 2020 Feb 30;30(2):735-743. Epub 2019 Aug 30.

Department of Radiology, Ansan Hospital, Korea University College of Medicine, 123, Jeokgeum-ro, Danwon-gu, Ansan-si, Gyeonggi, 15355, South Korea.

Objectives: To determine whether sirolimus has beneficial effects on lymphangioleiomyomatosis (LAM) lung cysts in CT with long-term follow-up (FU) and to investigate whether CT is an appropriate imaging biomarker to monitor and evaluate LAM progression.

Methods: In this retrospective study, 73 female patients diagnosed with definite LAM between May 2001 and June 2018 were included. Among these, 39 (53. Read More

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http://dx.doi.org/10.1007/s00330-019-06412-4DOI Listing
February 2020
17 Reads
4.014 Impact Factor

Role of thoracic imaging in the management of lymphangioleiomyomatosis.

Respir Med 2019 10 22;157:14-20. Epub 2019 Aug 22.

Department of Medical Sciences, Respiratory Unit, University of Milan, Italy.

Lymphangioleiomyomatosis (LAM) is a rare diffuse lung cystic disease (DLCD) that occurs sporadically or in association with Tuberous Sclerosis Complex (TSC). The diagnostic pathway is tracked on the identification of the disease hallmarks on chest High-Resolution Computed Tomography (HRCT). Aim of this review is to discuss the thoracic HRCT pathognomonic features, essential to rule out other DLCD. Read More

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http://dx.doi.org/10.1016/j.rmed.2019.08.013DOI Listing
October 2019
11 Reads

Lymphangioleiomyomatosis manifesting as refractory chylothorax and chyloperitoneum.

BMJ Case Rep 2019 Jul 11;12(7). Epub 2019 Jul 11.

Division of Medical Education, Department of Medicine, Barnes-Jewish Hospital, Saint Louis, Missouri, USA.

This is a rare case of sporadic lymphangioleiomyomatosis (S-LAM) manifesting as refractory chylothorax and chyloperitoneum. A middle-aged woman with unremarkable medical history presented with respiratory failure, abdominal distension and anasarca. She was found to have high-output chylous effusion that required chest tube drainage, as well as chylous ascites. Read More

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http://dx.doi.org/10.1136/bcr-2019-229958DOI Listing
July 2019
10 Reads

Low-dose sirolimus in retroperitoneal lymphangioleiomyomas.

Lung India 2019 Jul-Aug;36(4):349-352

Division of Pulmonary and Critical Care, Department of Internal Medicine, Mayo Clinic, Jacksonville, FL, USA.

Lymphangioleiomyomatosis (LAM) is a rare disease associated with cystic lung destruction and abdominal tumors, including lymphangioleiomyomas, which frequently occur in the retroperitoneal region. Sirolimus therapy is currently recommended for LAM patients with abnormal or declining lung function with an adjusted dose to maintain a serum trough level of 5-15 ng/mL. We describe a significant reduction of retroperitoneal lymphangioleiomyomas after treatment with low-dose sirolimus therapy (serum trough level <5 ng/mL) in a patient with sporadic LAM. Read More

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http://dx.doi.org/10.4103/lungindia.lungindia_433_18DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6625232PMC
July 2019
6 Reads

Characteristics and outcomes of patients hospitalized with interstitial lung diseases in Spain, 2014 to 2015.

Medicine (Baltimore) 2019 May;98(21):e15779

Respiratory Department, Hospital General Universitario Gregorio Marañón, Facultad de Medicina, Universidad Complutense de Madrid (UCM), Instituto de Investigación Sanitaria Gregorio Marañón (IiSGM).

To assess characteristics and outcomes of patients hospitalized with interstitial lung diseases (ILD) and to analyze patient's comorbidities, procedures, and in-hospital outcomes.We identified patients hospitalized with idiopathic pulmonary fibrosis and others ILD such as hypersensitivity pneumonitis, cryptogenic organizing pneumonia, lymphangioleiomyomatosis, pulmonary Langerhans cell histiocytosis, and sarcoidosis in Spain during 2014 and 2015.We identified 14,565 discharges among patients admitted for ILD in Spain during the study period: idiopathic pulmonary fibrosis (IPF) in 42. Read More

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http://dx.doi.org/10.1097/MD.0000000000015779DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6571208PMC
May 2019
18 Reads

Cyst Ventilation Heterogeneity and Alveolar Airspace Dilation as Early Disease Markers in Lymphangioleiomyomatosis.

Ann Am Thorac Soc 2019 08;16(8):1008-1016

1Center for Pulmonary Imaging Research, Division of Pulmonary Medicine and Department of Radiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.

Lymphangioleiomyomatosis (LAM) is a rare disease associated with cystic destruction of the pulmonary parenchyma and chronic respiratory failure, and there are trials underway to determine if early intervention can prevent disease progression. An imaging technique that is sensitive to early regional disease would therefore be valuable for patient care and clinical trials. We postulated that hyperpolarized Xe MRI would be sensitive to ventilation abnormalities and alveolar airspace dilation in patients with mild LAM disease and normal pulmonary function and that Xe MRI would reveal important features of cyst ventilation. Read More

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http://dx.doi.org/10.1513/AnnalsATS.201812-880OCDOI Listing
August 2019
8 Reads

Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil.

BMJ Case Rep 2019 Apr 23;12(4). Epub 2019 Apr 23.

Service de Pneumologie et Centre de Référence Constitutif des Maladies Pulmonaires Rares, Hôpital Bichat, Assistance Publique - Hôpitaux de Paris, Paris, France.

General lymphatic anomaly (GLA) is a very rare disorder, characterised by multifocal lymphatic malformations into various tissues that is due to congenital abnormalities of lymphatic development. No treatment has ever proved its efficiency.We report a 22-year-old man with recurrent bronchial casts due to thoracic involvement of GLA. Read More

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http://casereports.bmj.com/lookup/doi/10.1136/bcr-2018-22852
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http://dx.doi.org/10.1136/bcr-2018-228523DOI Listing
April 2019
24 Reads

Lymphangioleiomyomatosis: diagnostic imaging in an uncommon post-menopausal presentation.

Intern Med J 2019 04;49(4):549-550

Department of Medicine, Goulburn Valley Health, Shepparton, Victoria, Australia.

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http://dx.doi.org/10.1111/imj.14253DOI Listing
April 2019
8 Reads

A Rare Case of an Abdominal Aneurysm in a Patient with Lymphangioleiomyomatosis: A Case Report.

Perm J 2019 ;23

Respiralab, Respiralab Research Group, Guayaquil, Ecuador.

Introduction: Lymphangioleiomyomatosis (LAM) is a multisystemic disorder characterized by the proliferation, metastasis, and infiltration of smooth muscle cells in the lung and other tissues. Although LAM is rarely seen in men, it is usually detected in women during the third decade of life. Renal microaneurysms have been reported in patients with LAM, but abdominal aneurysms have not. Read More

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http://dx.doi.org/10.7812/TPP/18-170DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6443368PMC
September 2019
8 Reads

Tibial perivascular epithelioid cell tumour (PEComa). A case report and literature review.

Rev Esp Cir Ortop Traumatol 2019 May - Jun;63(3):239-245. Epub 2019 Mar 21.

Hospital de Ortopedia Dr. Victorio de la Fuente Narváez, Instituto Mexicano del Seguro Social, Ciudad de México, México.

Introduction: Perivascular epithelioid cell (PEC) is a cell type constantly present in a group of tumours including angiomyolipoma (AML), clear-cell «sugar» tumour (CCST) of the lung and extrapulmonary sites, lymphangioleiomyomatosis (LAM), and clear-cell tumours of other anatomical sites. It has morphologic distinctive features: epithelioid appearance with a clear to granular cytoplasm, a round to oval, centrally located nucleus and an inconspicuous nucleolus. Immunohistochemically, PEC expresses myogenic and melanocytic markers. Read More

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http://dx.doi.org/10.1016/j.recot.2018.10.003DOI Listing
December 2019
10 Reads

Isolated Cystic Lung Disease: An Algorithmic Approach to Distinguishing Birt-Hogg-Dubé Syndrome, Lymphangioleiomyomatosis, and Lymphocytic Interstitial Pneumonia.

AJR Am J Roentgenol 2019 03 19:1-5. Epub 2019 Mar 19.

1 Department of Radiology, National Jewish Health, Denver, CO.

Objective: Birt-Hogg-Dubé (BHD) syndrome, lymphangioleiomyomatosis (LAM), and lymphocytic interstitial pneumonia (LIP) frequently present as isolated cystic lung disease and can be challenging to distinguish. If imaging findings are otherwise unremarkable, the radiologist is unaided by ancillary CT findings in narrowing the diagnosis. We hypothesized that the distribution and morphologic features of lung cysts could be used to differentiate BHD syndrome, LAM, and LIP. Read More

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http://dx.doi.org/10.2214/AJR.18.20920DOI Listing
March 2019
14 Reads

Extrapulmonary uterine lymphangioleiomyomatosis (LAM) and dysfunctional uterine bleeding: the first presentation of LAM in a tuberous sclerosis complex patient.

BMJ Case Rep 2019 Feb 25;12(2). Epub 2019 Feb 25.

Department of Cellular Pathology, New Cross Hospital, Wolverhampton, UK.

Lymphangioleiomyomatosis (LAM) is a rare disease that typically affects women of childbearing age. It most commonly affects the lungs (P-LAM) but can occasionally occur in extra-pulmonary sites (E-LAM). There is a strong association between LAM and the tuberous sclerosis complex (TSC). Read More

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http://dx.doi.org/10.1136/bcr-2018-226358DOI Listing
February 2019
30 Reads

MR findings of uterine PEComa in patients with tuberous sclerosis: report of two cases.

Abdom Radiol (NY) 2019 04;44(4):1256-1260

Department of Radiology, University of Tsukuba Hospital, 2-1-1 Amakubo, Tsukuba, Ibaraki, 305-8576, Japan.

Tuberous sclerosis complex (TSC), a rare autosomal dominant neurocutaneous disorder, is characterized by the presence of benign congenital tumors in multiple organs. Neoplasms with perivascular epithelioid cell differentiation (PEComas), including angiomyolipoma (AML) and lymphangioleiomyomatosis (LAM), can occur in association with TSC. This report describes two cases of uterine PEComas presenting characteristic MR imaging features reflecting pathological findings. Read More

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http://dx.doi.org/10.1007/s00261-019-01918-3DOI Listing
April 2019
9 Reads

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.

PLoS One 2019 11;14(2):e0210975. Epub 2019 Feb 11.

Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan.

Purpose: Whereas native lung overinflation has been thought to happen in recipients of single lung transplantation for lymphangioleiomyomatosis because of its increased compliance, there is no study that has reported the details on the change of the native lung volume after single lung transplantation by three-dimensional computed tomography volumetry. The purpose of the present study was to evaluate the lung volume after single lung transplantation for lymphangioleiomyomatosis by three-dimensional computed tomography volumetry and investigate the correlation between the native lung volume change and postoperative pulmonary function.

Methods: We retrospectively reviewed the data of 17 patients who underwent single lung transplantation for lymphangioleiomyomatosis. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0210975PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6370208PMC
October 2019
5 Reads

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

AJR Am J Roentgenol 2019 04 23;212(4):766-772. 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
April 2019
34 Reads
2.731 Impact Factor

Use of CT Imaging to Quantify Progression and Response to Treatment in Lymphangioleiomyomatosis.

Chest 2019 05 17;155(5):962-971. 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6533448PMC
May 2019
17 Reads

Serum Vascular Endothelial Growth Factor D in Cystic Lung Disease.

JAMA 2019 Jan;321(4):401-402

Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville.

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http://jama.jamanetwork.com/article.aspx?doi=10.1001/jama.20
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http://dx.doi.org/10.1001/jama.2018.20926DOI Listing
January 2019
25 Reads

Somatic activating mutations in cause generalized lymphatic anomaly.

J Exp Med 2019 02 27;216(2):407-418. Epub 2018 Dec 27.

Vascular Malformations Section, Institute of Medical and Molecular Genetics, Institute of Medical and Molecular Genetics-Instituto de Investigación PAZ, Hospital Universitario La Paz, Madrid, Spain

Generalized lymphatic anomaly (GLA) is a vascular disorder characterized by diffuse or multifocal lymphatic malformations (LMs). The etiology of GLA is poorly understood. We identified four distinct somatic variants (Glu542Lys, Gln546Lys, His1047Arg, and His1047Leu) in tissue samples from five out of nine patients with GLA. Read More

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http://www.jem.org/lookup/doi/10.1084/jem.20181353
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http://dx.doi.org/10.1084/jem.20181353DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6363432PMC
February 2019
73 Reads

Can we predict the existence of extrarenal feeders to renal angiomyolipomas?

Eur Radiol 2019 May 12;29(5):2499-2506. Epub 2018 Dec 12.

Department of Radiology, School of Medicine, Juntendo University, Tokyo, Japan.

Objectives: To identify factors predicting the presence of extrarenal feeders to renal angiomyolipomas (AMLs) METHODS: This is a retrospective study of 44 patients with 58 renal AMLs embolized in our department. Arteriography obtained during embolization and CT angiography obtained before and after embolization were reviewed to characterize AMLs with and without extrarenal feeders. Tumor characteristics were compared between the two groups. Read More

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http://dx.doi.org/10.1007/s00330-018-5877-1DOI Listing
May 2019
18 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
33 Reads

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

Chest 2019 03 3;155(3):528-533. 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6414786PMC
March 2019
31 Reads

Less common manifestations in TSC.

Am J Med Genet C Semin Med Genet 2018 09 29;178(3):348-354. Epub 2018 Aug 29.

Department of Pediatric Radiology, Hospital San Joan de Déu, Barcelona.

Tuberous sclerosis complex (TSC) is due to pathogenic variants in TSC1 or TSC2 genes resulting in hyperactivation of the mTOR pathway. Many organ systems can be affected, such as brain, skin, eye, heart, bone, kidney, or lung. Typical lesions of TSC usually are those included as major criteria, including angiofibromas, hypomelanotic macules, tubers, subependymal nodules, angiomyolipomas, cardiac rhabdomyomas, and lymphangioleiomyomatosis. Read More

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http://doi.wiley.com/10.1002/ajmg.c.31648
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http://dx.doi.org/10.1002/ajmg.c.31648DOI Listing
September 2018
62 Reads

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

Am J Respir Cell Mol Biol 2018 12;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
86 Reads

Smoking-Related Diffuse Cystic Lung Disease.

Chest 2018 08;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
9 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
27 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 12 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
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6816044PMC
December 2018
27 Reads

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

Authors:
Joel A Strom

J Am Soc Echocardiogr 2018 08 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
6 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
8 Reads

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

J Am Soc Echocardiogr 2018 08 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
65 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 03 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
18 Reads

Mounier-Kuhn Syndrome Mimicking Lymphangioleiomyomatosis.

Chest 2018 02;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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https://linkinghub.elsevier.com/retrieve/pii/S00123692173297
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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
8 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
13 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
13 Reads

Recurrence of retroperitoneal localized perivascular epithelioid cell tumor two years after initial diagnosis: case report.

Sao Paulo Med J 2019 Mar-Apr;137(2):206-208. Epub 2018 Jan 15.

MD. Radiologist, Department of Radiology, Kayseri Egitimve Arastirma Hastanesi, Kayseri, Turkey.

Context: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Adjuvant radiotherapy and/or chemotherapy are administered according to the patient's clinical characteristics.

Case Report: A 42-year-old female patient was operated to treat a retroperitoneal mass. Read More

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http://www.scielo.br/scielo.php?script=sci_arttext&pid=S
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http://dx.doi.org/10.1590/1516-3180.2017.0120050717DOI Listing
December 2019
12 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
6 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
55 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
10 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
17 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
21 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
56 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
8 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
18 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
24 Reads