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CA-125 in Disease Progression and Treatment of Lymphangioleiomyomatosis.

Authors
  • Glasgow, Connie G1
  • Pacheco-Rodriguez, Gustavo1
  • Steagall, Wendy K1
  • Haughey, Mary E2
  • Julien-Williams, Patricia A1
  • Stylianou, Mario P3
  • Gochuico, Bernadette R4
  • Moss, Joel5
  • 1 Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.
  • 2 Office of the Clinical Director, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.
  • 3 Office of Biostatistics Research, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD.
  • 4 Medical Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, MD.
  • 5 Cardiovascular and Pulmonary Branch, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD. Electronic address: [email protected]
Type
Published Article
Journal
CHEST Journal
Publisher
Elsevier
Publication Date
Feb 01, 2018
Volume
153
Issue
2
Pages
339–348
Identifiers
DOI: 10.1016/j.chest.2017.05.018
PMID: 28576630
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

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. 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. Almost 25% of patients with LAM had at least one elevated serum CA-125 measurement. Higher serum CA-125 levels correlated with lower FEV1, premenopausal status, and pleural effusion in a multivariate model (each P < .001). Serum CA-125 levels decreased following sirolimus treatment (P = .002). CA-125 and α-smooth muscle actin were co-expressed in LAM lung nodules. 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. Copyright © 2017. Published by Elsevier Inc.

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