496 results match your criteria Lymphangioleiomyomatosis Imaging


Lymphangioleiomyomatosis associated with prolactinoma: A case report and literature review.

Respir Med Case Rep 2021 28;33:101406. Epub 2021 Mar 28.

Department of Respiratory Medicine, Iizuka Hospital, Iizuka, Japan.

The thirty-five-year-old woman had been experiencing dyspnea on exertion since her second child's birth five years before presenting to hospital A, where she was diagnosed with lymphangioleiomyomatosis (LAM) based on video-assisted thoracoscopic surgery lung biopsy and referred to our hospital. She was treated with sirolimus for one year. Although her subjective symptom of dyspnea improved, she found that her amenorrhea had persisted for six years. Read More

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A Case of Burnt-Out Langerhans Cell Histiocytosis Presenting as Postpartum Hypopituitarism.

AACE Clin Case Rep 2021 Jan-Feb;7(1):47-50. Epub 2020 Dec 28.

Department of Endocrinology, Townsville University Hospital, Douglas, Queensland, Australia.

Objective: To evaluate the case of a woman who presented with central hypogonadism and diabetes insipidus and further developed a persistent cough leading to an unexpected diagnosis of burnt-out Langerhans cell histiocytosis (LCH).

Methods: Clinical and laboratory endocrine evaluation, magnetic resonance imaging, high-resolution computed tomography, and open-lung biopsy results are discussed.

Results: A 28-year-old woman presented at 10 months postpartum with polydipsia, polyuria, and amenorrhea for 3 months. Read More

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December 2020

Quantitative Analysis of Cystic Lung Diseases by Use of Paired Inspiratory and Expiratory CT: Estimation of the Extent of Cyst-Airway Communication and Evaluation of Diagnostic Utility.

Radiol Cardiothorac Imaging 2020 Apr 30;2(2):e190097. Epub 2020 Apr 30.

Departments of Radiology (K. Suzuki, R.K.) and Respiratory Medicine (K. Seyama, H.E.), Juntendo University Faculty of Medicine and Graduate School of Medicine, 3-1-3, Hongo, Bunkyo-ku, Tokyo 113-8431, Japan; Department of Thoracic Surgery, Tokyo Metropolitan Bokutoh Hospital, Tokyo, Japan (H.E.); and Department of Pathology, Japanese Red Cross Medical Center, Tokyo, Japan (T.K.).

Purpose: To establish a method for quantitatively estimating the extent of the communication between the cyst and the airway in cystic lung diseases (CLDs) and evaluate its diagnostic utility in differentiating among CLDs.

Materials And Methods: Seventy-one patients (mean age, 49.9 years; age range, 25-79 years) with CLDs who underwent paired inspiratory and expiratory CT between July 2015 and July 2018 were enrolled in this prospective study. Read More

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CT findings of pulmonary cysts.

Clin Radiol 2021 Mar 16. Epub 2021 Mar 16.

Department of Pulmonology, Complexo Hospitalario Universitario de Santiago de Compostela (CHUS), Santiago de Compostela, Spain.

Pulmonary cysts are thin-walled radiolucent lesions that may appear in a variety of uncommon disorders known as diffuse cystic lung diseases (DCLD) that essentially includes lymphangioleiomyomatosis (LAM), Langerhans cell histiocytosis (LCH), lymphocytic interstitial pneumonia (LIP), Pneumocystis jiroveci pneumonia (PJP), and Birt-Hogg-Dubé syndrome (BHDS). Moreover, they have been reported in several cases of coronavirus disease 2019 (COVID-19). The purpose of this review is to provide a practical approach for evaluating lung cysts when encountered on CT. Read More

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A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism.

Biomed Res Int 2021 10;2021:6612776. Epub 2021 Feb 10.

Department of Pathology, First Affiliated Hospital of Weifang Medical University, Weifang People's Hospital, Weifang 261041, China.

Objective: Lymphangioleiomyomatosis (LAM) is a rare low-grade metastatic tumor; however, LAM patients were always found in young age with difficulty for diagnosis. Our study is aimed at observing the clinical characteristics of patients with lymphangiomatosis, including the clinical manifestations, imaging findings, histopathological features, and immunophenotype.

Methods: We did a systematic review on LAM/PLAM cases, especially on male cases, and collected the clinical features and molecular mechanisms of PLAM based on previous findings. Read More

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February 2021

Lymphangioleiomyomatosis.

QJM 2021 Feb 18. Epub 2021 Feb 18.

Department of Radiology, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.

We report a case of lymphangioleiomyomatosis with chest and abdominal findings. The clinical manifestation and imaging findings are described in the manuscript. Read More

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February 2021

Prognostic role of KL-6 in lymphangioleiomyomatosis patients.

Minerva Med 2021 Feb 12. Epub 2021 Feb 12.

Respiratory Diseases and Lung Transplantation, Department of Medical and Surgical Sciences & Neurosciences, Siena University Hospital, Siena, Italy.

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February 2021

Ultra-Small Lung Cysts Impair Diffusion Without Obstructing Air Flow in Lymphangioleiomyomatosis.

Chest 2021 Feb 5. Epub 2021 Feb 5.

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

Background: Lymphangioleiomyomatosis (LAM) is a rare lung disease found primarily in women of childbearing age, characterized by the formation of air-filled cysts, which may be associated with reductions in lung function. An experimental, regional ultra-high resolution CT scan identified an additional volume of cysts relative to standard chest CT imaging, which consisted primarily of ultra-small cysts.

Research Question: What is the impact of these ultra-small cysts on the pulmonary function of patients with LAM?

Study Design And Methods: A group of 103 patients with LAM received pulmonary function tests and a CT examination in the same visit. Read More

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February 2021

Effect of everolimus on multifocal micronodular pneumocyte hyperplasia in tuberous sclerosis complex.

Respir Med Case Rep 2020 25;31:101310. Epub 2020 Nov 25.

Department of Respiratory Medicine, Lausanne University Hospital (CHUV) and University of Lausanne, Switzerland.

Multifocal micronodular pneumocyte hyperplasia (MMPH) is a benign proliferation of alveolar type II cells presenting as multiple pulmonary nodules at chest imaging, which is frequently seen in patients with tuberous sclerosis complex (TSC). We report a case of a woman with TSC and MMPH who received everolimus, a mechanistic target of rapamycin (mTOR) inhibitor, for the treatment of a subependymal giant cell astrocytoma (SEGA). After 3 months of therapy, a remarkable decrease in density of all pulmonary MMPH lesions was observed, without any change in size. Read More

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November 2020

Pulmonary lymphangioleiomyomatosis masquerading as unilateral heterogeneous emphysema.

J Surg Case Rep 2020 Nov 30;2020(11):rjaa486. Epub 2020 Nov 30.

Department of Cardiothoracic Surgery, Royal Stoke University Hospital, Stoke On Trent, UK.

Pulmonary lymphangioleiomyomatosis (LAM) is a rare, well-described pathology and usually is exclusive to females of a reproductive age. We present a 45 year-old lady who presented to the surgeons 1 year after an admission with acute dyspnoea following influenza infection. Initial computed tomography imaging findings demonstrated severe, heterogenous right-sided bullous emphysematous changes, but histopathological analysis of the post-operative specimen favoured a diagnosis of LAM. Read More

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November 2020

A Case of Tuberous Sclerosis Complex with Lymphangioleiomyomatosis and Renal Cell Carcinoma.

Onco Targets Ther 2020 2;13:12421-12426. Epub 2020 Dec 2.

Department of Respiratory Medicine, Fujian Provincial Hospital, Fujian Medical University, Fuzhou, Fujian 350001, People's Republic of China.

Lymphangioleiomyomatosis (LAM) is a rare disease affecting young women, which occurs sporadically or in patients with tuberous sclerosis complex (TSC). The main manifestations of TSC in the kidney include cysts and angiomyolipoma (AML). Although renal cell carcinoma (RCC) is not a manifestation of TSC, it has a 2-4% incidence in TSC patients. Read More

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December 2020

Lymphangioleiomyomatosis Presenting as Recurrent Pneumothorax.

Cureus 2020 Oct 23;12(10):e11102. Epub 2020 Oct 23.

Emergency Medicine, Jersey Shore University Medical Center, Neptune City, USA.

Lymphangioleiomyomatosis (LAM) is a disorder that causes cystic disease in the lungs. This condition is associated with renal angiomyolipomas and commonly occurs in individuals with tuberous sclerosis. Despite its frequent association with tuberous sclerosis, LAM is a rare condition and is often underdiagnosed. Read More

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October 2020

A 47-Year-Old Woman With Pulmonary Nodules and Facial Hemispasms.

Chest 2020 10;158(4):e197-e204

Respiratory Center, Asahikawa Medical University Hospital, Japan.

Case Presentation: A 47-year-old woman visited her primary physician for a health check, and some radiographic abnormalities were detected. She was referred to our division for further management. In recent years, she had become conscious of occasional facial hemispasms. Read More

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October 2020

Usefulness of 99mTc-ASC lymphoscintigraphy and SPECT/CT in the evaluation of rare lymphatic disorders: Gorham-Stout disease, lymphangioma, and lymphangioleiomyomatosis.

Medicine (Baltimore) 2020 Sep;99(39):e22414

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

The purpose of this study was to investigate the role of Tc-antimony sulfide colloid (ASC) lymphoscintigraphy and single photon emission computed tomography/computed tomography (SPECT/CT) in the evaluation of rare lymphatic disorders, including Gorham--Stout disease (GSD), lymphangioma, and lymphangioleiomyomatosis (LAM).Nine patients suspected to have rare lymphatic disorders were included in this retrospective study. All patients underwent Tc-ASC lymphoscintigraphy and SPECT/CT to evaluate the lesions. Read More

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September 2020

Birt-Hogg-Dubé syndrome.

Eur Respir Rev 2020 Sep 17;29(157). Epub 2020 Sep 17.

Respiratory Medicine Dept, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland

Birt-Hogg-Dubé syndrome (BHD) is a rare inherited autosomal dominant disorder caused by germline mutations in the tumour suppressor gene , encoding the protein folliculin. Its clinical expression typically includes multiple pulmonary cysts, recurrent spontaneous pneumothoraces, cutaneous fibrofolliculomas and renal tumours of various histological types. BHD has no sex predilection and tends to manifest in the third or fourth decade of life. Read More

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September 2020

Risk factors for right ventricular dysfunction in patients with lymphangioleiomyomatosis.

Int J Cardiovasc Imaging 2021 Feb 14;37(2):439-448. Epub 2020 Sep 14.

Department of Cardiology, Ruijin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, 200025, China.

Lymphangioleiomyomatosis (LAM) is a rare disease characterized by diffuse cystic lesions of the lung. The present study was designed to evaluate the right ventricular (RV) function in LAM patients via single-beat real-time three-dimensional echocardiography (RT-3DE) and to investigate the factors affecting RV function in LAM patients. According to tricuspid regurgitation velocity (TRV), forty-five female LAM patients [(44. Read More

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February 2021

A rare pulmonary lymphangioleiomyomatosis disease in a male with tuberous sclerosis complex.

Respir Med Case Rep 2020 10;31:101150. Epub 2020 Jul 10.

Department of Anaesthesiology and Critical Care Medicine, All India Institute of Medical Sciences, Rishikesh, India.

Background: Pulmonary Lymphangioleiomyomatosis (LAM) is an uncommon disease and may be associated with tuberous sclerosis complex (TSC). LAM is reported to occur exclusively in females of the premenopausal age group. Here we report a rare entity of lymphangioleiomyomatosis in a male patient of tuberous sclerosis, who developed pneumothorax following mechanical ventilation. Read More

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Needle in the Haystack: Finding the Elusive Lymphangioleiomyomatosis Cell.

Am J Respir Crit Care Med 2020 11;202(10):1329-1330

National Heart, Lung, and Blood Institute National Institutes of Health Bethesda, Maryland.

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November 2020

Chronic kidney disease caused by tuberous sclerosis complex: lymphangioleiomyomatosis diagnosed in an adult woman.

Pol Arch Intern Med 2020 10 6;130(10):895-897. Epub 2020 Jul 6.

Department of Nephrology, Jagiellonian University Medical College, Kraków, Poland.

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October 2020

Diagnostic usefulness of transbronchial lung cryobiopsy in two patients mildly affected with pulmonary lymphangioleiomyomatosis.

Respir Investig 2020 Jul 4;58(4):295-299. Epub 2020 Apr 4.

Department of Respiratory Medicine, Japanese Red Cross Medical Center, 4-1-22 Hiroo, Shibuya-ku, Tokyo, 150-8935, Japan. Electronic address:

Transbronchial lung biopsy is a non-invasive technique used primarily for the pathological diagnosis of lymphangioleiomyomatosis (LAM). However, some cases, particularly those with early-stage lung lesions, are difficult to diagnose because of the specimen size and presence of artifacts. Herein, we present two cases of LAM with relatively mild cystic changes in the lungs and slight impairment seen in pulmonary function tests. Read More

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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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January 2020

Quantitative analysis of airway obstruction in lymphangioleiomyomatosis.

Eur Respir J 2020 07 2;56(1). Epub 2020 Jul 2.

Respiratory Diseases, Dept of Chronic Diseases, Metabolism and Aging, KU Leuven, Leuven, Belgium.

Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease with progressive pulmonary function loss caused by progressively proliferating LAM cells. The degree of airway obstruction has not been well investigated within the pathogenesis of LAM.Using a combination of computed tomography (CT), microCT and histology, the site and nature of airway obstruction in LAM explant lungs was compared with matched control lungs (n=5 each). Read More

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Diffuse Cystic Lung Disease: Consider Sjögren Syndrome in the Differential Diagnosis.

Am J Med 2020 08 19;133(8):e438-e439. Epub 2020 Feb 19.

Division of Rheumatology, Department of Internal Medicine, School of Medicine, Universidad de Antioquia, Medellín, Colombia.

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Three's company: A woman with rash, uterine mass and cystic lungs.

Monaldi Arch Chest Dis 2020 Jan 21;90(1). Epub 2020 Jan 21.

Division of Pulmonary, Critical Care and Sleep Medicine, Westchester Medical Center, Valhalla, NY.

Cystic lung disease encompasses a wide variety of clinical entities, the diagnosis of which is sometimes straightforward and other times obscure.  To narrow the list of possibilities, it behooves the physician to consider the context in which the cystic lung disease is uncovered. Clues to the diagnosis might be provided by findings that are not initially obvious and are not located in the thorax. Read More

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January 2020

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

Eur J Nucl Med Mol Imaging 2020 09 10;47(10):2469-2470. 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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September 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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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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