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Lymphangioleiomyomatosis (LAM) is a rare disease involving the proliferation of LAM cells in the lungs and the axial lymphatic system and mechanistic target of rapamycin (mTOR) inhibitors are the only effective medicines for treating it. Patients suffering from LAM, who are allergic to mTOR inhibitors can be treated by desensitizing them to the medicine. A 39-year-old woman presented with dyspnea caused by chylous pleural effusion, ascites, and retroperitoneal lymphangioleiomyomas.
View Article and Find Full Text PDFLymphangioleiomyomatosis: Rapidly Progressive Retroperitoneal Cystic Tumor Mimicking Malignancy.
Intern Med
November 2024
Department of Respiratory Medicine, Okayama City Hospital, Japan.
Case report: If it is not asthma-think of lymphangioleiomyomatosis in younger female patients.
Front Med (Lausanne)
February 2024
Department of Oncology, Aarhus University Hospital, Aarhus, Denmark.
Lymphangioleiomyomatosis (LAM) is a rare lung disease predominantly affecting women, and it is characterized by the proliferation of smooth muscle cells and cystic lung destruction. LAM diagnosis is challenging due to varied clinical presentations and resemblance to common conditions such as asthma. We present two female cases where LAM was initially misdiagnosed.
View Article and Find Full Text PDFA case report of lymphangioleiomyomatosis with retroperitoneal masses in pregnancy.
Front Med (Lausanne)
December 2023
Department of Gynecology, Shanghai First Maternity and Infant Hospital, School of Medicine, Tongji University, Shanghai, China.
Background: Lymphangioleiomyomatosis (LAM) is a rare, gradually advancing tumor of unknown origin. It is distinguished by the anomalous proliferation of pulmonary smooth muscle cells and predominantly manifests in women of childbearing age. In this study, we aim to present a noteworthy case of LAM accompanied by lymphangioleiomyoma in the retroperitoneal space during pregnancy, a scenario susceptible to misdiagnosis.
View Article and Find Full Text PDFClinicopathological Impacts of Expression of Neuronal Markers in Lymphangioleiomyomatosis.
Am J Surg Pathol
November 2023
The Study Group for Pneumothorax and Cystic Lung Diseases, Setagaya-Ku, Tokyo, Japan.
Lymphangioleiomyomatosis (LAM) is a tuberous sclerosis complex (TSC)-associated tumor, characterized by the expression of neural crest lineages including neuronal markers. Neural crest cells can differentiate into multiple cell types that contribute to tissues associated with TSC-related tumors, and TSC-related tumors could be specifically associated with distinct neural crest subtypes. This study aimed to clarify the clinicopathological effects of expression of neuronal markers in LAM.
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