Affiliation:
1. Department of Gynecology, Endocrinology and Gynecological Oncology, Pomeranian Medical University in Szczecin, 71-252 Szczecin, Poland
Abstract
Lymphangioleiomyomatosis (LAM) is characterized by lung cysts that cause lung deterioration, changes in the lymphatic system, and tumors in the kidneys. It mainly affects women of reproductive age and is a progressive disease. LAM can occur as an isolated disease or coexist with tuberous sclerosis (TSC). The source of LAM cells is unknown. Patients with confirmed LAM should be treated with an mTOR inhibitor, sirolimus, or everolimus. We present a case of LAM with TSC in a patient whose symptoms, including those in the lymph nodes and chyaloperitoneum, mainly concern the abdominal cavity.
Reference30 articles.
1. Minireview: Lymphangioleiomyomatosis (LAM): The “Other” Steroid-Sensitive Cancer;Prizant;Endocrinology,2016
2. Lymphangioleiomyomatosis: Pathogenesis, clinical features, diagnosis, and management;McCarthy;Lancet Respir. Med.,2021
3. Lymphangioleiomyomatosis: Circulating Levels of FGF23 and pulmonary diffusion;Esposito;J. Bras. Pneumol.,2023
4. The epidemiology and clinical features of Lymphangioleiomyomatosis (LAM): A descriptive study of 33 case reports;Shah;Cureus,2023
5. Pulmonary lymphangioleiomyomatosis: A rare case;Hoca;Turk. Patoloji Derg.,2014