Pulmonary Lymphangioleiomyomatosis: A Rare Case

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Agackiran Y., Erturk A., Yesiller F. I., Hoca N., Ustun L. N., Capan N.

TURKISH JOURNAL OF PATHOLOGY, vol.30, no.3, pp.233-236, 2014 (ESCI) identifier identifier identifier

  • Publication Type: Article / Article
  • Volume: 30 Issue: 3
  • Publication Date: 2014
  • Doi Number: 10.5146/tjpath.2013.01190
  • Journal Indexes: Emerging Sources Citation Index (ESCI), Scopus, TR DİZİN (ULAKBİM)
  • Page Numbers: pp.233-236
  • Keywords: Lymphangioleiomyomatosis, Immunohistochemistry, Lung neoplasms, Differential diagnosis
  • Gazi University Affiliated: Yes


Lymphangioleiomyomatosis is an uncommon lung disease primarily affecting women of childbearing age. It is characterized by the progressive proliferation and infiltration of smooth muscle-like cells, which lead to cystic destruction of the lung parenchyma; obstruction of airways, blood vessels, and lymphatics; and loss of pulmonary function. We present the case of a 46-year-old female patient with chest pain, cough, sputum, and dyspnea on exertion for three weeks. Minimal pneumothorax was noted, and the patient was referred to our center for further investigation and treatment. High-resolution computed tomography revealed numerous bilateral thin-walled air cysts and interstitial thickening affecting the central and peripheral part of the upper zone of the lung. We performed an open-lung biopsy to confirm lymphangioleiomyomatosis. Our aim is to discuss the pathogenesis and other lesions noted in the differential diagnosis of this rare disease.