Lymphangioleiomyomatosis (LAM) is characterised by the proliferation of abnormal smooth muscle cells (LAM cells) in the lungs, lymph nodes and other organs. We report the case of a man in his 50s who had right-sided pleural effusion. On performing a diagnostic tap, the fluid was milky white in colour. An intercostal chest tube was inserted and after complete drainage of fluid, a high-resolution CT (HRCT) was done. HRCT revealed multiple cysts throughout both of the lungs. On subsequent bronchoscopy-guided transbronchial lung biopsy and histochemical staining, diagnosis of LAM was made. We started the patient on oral sirolimus. On subsequent follow-up, subjective and objective improvements were observed.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC10124213 | PMC |
| http://dx.doi.org/10.1136/bcr-2022-251513 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Abdominal lymphangioleiomyomatosis in a man presenting with gastrointestinal hemorrhage as the first manifestation: a case report.
Front Med (Lausanne)
December 2024
Department of Gastroenterology, The First Affiliated Hospital of Dali University, Dali, Yunnan, China.
Lymphangioleiomyomatosis (LAM) is a rare, low-grade malignant condition that typically affects women of childbearing age and primarily involves the lungs. While cases involving males and affecting the gastrointestinal tract are exceedingly uncommon. This report discusses an unusual case of abdominal LAM in a male patient with gastrointestinal hemorrhage.
View Article and Find Full Text PDFLymphangioleiomyomatosis of the Pelvic Lymph Nodes Detected Incidentally During Surgical Staging of Gynecological Malignancies: Comprehensive Clinicopathological Analysis of 17 Consecutive Cases from a Single Institution.
In Vivo
December 2024
Department of Pathology and Translational Genomics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Republic of Korea
Background/aim: Lymphangioleiomyomatosis (LAM) belongs to the perivascular epithelioid cell tumor (PEComa) family. The relationship between LAM and tuberous sclerosis complex (TSC) is of particular concern in a subset of women with clinically occult LAM involving the pelvic lymph nodes. This study aimed to investigate the clinicopathological features of incidental nodal LAM detected during the surgical staging of gynecological tumors.
View Article and Find Full Text PDFSTAT1 Promotes PD-L1 Activation and Tumor Growth in Lymphangioleiomyomatosis.
bioRxiv
December 2024
Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Internal Medicine, University of Cincinnati; Cincinnati, OH 45267, USA.
Lymphangioleiomyomatosis (LAM) is a cystic lung disease that primarily affects women. LAM is caused by the invasion of metastatic smooth muscle-like cells into the lung parenchyma, leading to abnormal cell proliferation, lung remodeling and progressive respiratory failure. LAM cells have TSC gene mutations, which occur sporadically or in people with Tuberous Sclerosis Complex.
View Article and Find Full Text PDFPericardial Effusion as a Presentation of Lymphangioleiomyomatosis.
Cureus
November 2024
Internal Medicine, Unidade Local de Saúde de Braga, Braga, PRT.
Lymphangioleiomyomatosis (LAM) is a rare, systemic neoplastic disease that primarily affects women of childbearing age. The disease can arise sporadically or in association with tuberous sclerosis. It is characterized by the proliferation of abnormal smooth muscle-like cells, leading to cystic lung destruction, accumulation of chylous fluid, and development of abdominal tumors.
View Article and Find Full Text PDFUnlocking the clinical potential of paired inspiratory and expiratory CT scans in the differential diagnosis of cystic lung diseases: A systematic review.
PLoS One
December 2024
Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil.
Introduction: Currently, high-resolution computed tomography (HRCT) is the imaging of choice for the differential diagnosis of various cystic lung lesions, including true cystic lung diseases (CLD) and lesions that may mimic them. However, the traditionally used inspiratory scan still presents a significant spectrum of overlapping radiological features. Recent studies have demonstrated variation in lesion size between inspiratory and expiratory phases, probably due to cyst-airway communication.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!