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CA-125 in Disease Progression and Treatment of Lymphangioleiomyomatosis
- Source :
- Chest. 153(2)
- Publication Year :
- 2017
-
Abstract
- Background Lymphangioleiomyomatosis (LAM) is a destructive lung disease of women caused by proliferation of neoplastic-like LAM cells, with mutations in the TSC1/2 tumor suppressor genes. Based on case reports, levels of cancer antigen 125 (CA-125), an ovarian cancer biomarker, can be elevated in patients with LAM. We hypothesized that elevated serum CA-125 levels seen in some patients with LAM were due to LAM, not other malignancies, and might respond to sirolimus treatment. Methods Serum CA-125 levels were measured for 241 patients at each visit. Medical records were reviewed for co-morbidities, disease progression, and response to sirolimus treatment. CA-125 expression in LAM cells was determined by using immunohistochemical analysis. Results Almost 25% of patients with LAM had at least one elevated serum CA-125 measurement. Higher serum CA-125 levels correlated with lower FEV 1 , premenopausal status, and pleural effusion in a multivariate model (each P P = .002). CA-125 and α-smooth muscle actin were co-expressed in LAM lung nodules. Conclusions Higher serum CA-125 levels were associated with pleural effusions and reduced pulmonary function and were decreased with sirolimus therapy. LAM cells express CA-125. Some elevated serum CA-125 levels may reflect serosal membrane involvement.
- Subjects :
- Pulmonary and Respiratory Medicine
Adult
Pathology
medicine.medical_specialty
Angiomyolipoma
Pleural effusion
Critical Care and Intensive Care Medicine
03 medical and health sciences
Idiopathic pulmonary fibrosis
Young Adult
0302 clinical medicine
immune system diseases
hemic and lymphatic diseases
medicine
Humans
Lymphangioleiomyomatosis
Aged
Aged, 80 and over
Sirolimus
Analysis of Variance
Lung
business.industry
Mucins
Middle Aged
bacterial infections and mycoses
medicine.disease
Immunohistochemistry
Respiratory Function Tests
Pleural Effusion
medicine.anatomical_structure
030228 respiratory system
030220 oncology & carcinogenesis
CA-125 Antigen
Disease Progression
Biomarker (medicine)
lipids (amino acids, peptides, and proteins)
Female
Diffuse Lung Disease
Cardiology and Cardiovascular Medicine
Ovarian cancer
business
Biomarkers
Immunosuppressive Agents
medicine.drug
Subjects
Details
- ISSN :
- 19313543
- Volume :
- 153
- Issue :
- 2
- Database :
- OpenAIRE
- Journal :
- Chest
- Accession number :
- edsair.doi.dedup.....0d5bc4a010bb4c72b99d4d293fcb703c