Tuberc Respir Dis.  2007 Sep;63(3):289-293. 10.4046/trd.2007.63.3.289.

A Case of Lymphangioleiomyomatosis Presenting as a Lung Mass

• Park SC
• Park BH
• Shin SY
• Jeon HH
• Chung KS
• Park JC
• Jeong J
• Kwon JE
• Park MS
• Kim SK
• Chang J
• Kim SK
• Kim YS

• KMID: 1518422
• DOI: http://doi.org/10.4046/trd.2007.63.3.289

Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease that affects females of reproductive age. It is characterized by the abnormal proliferation of smooth muscle cells in the lung and along the axial lymphatics. We report a case of lymphangioleiomyomatosis presenting as a lung mass. The patient visited the emergency room because of dyspnea upon exertion. The chest X-ray showed a lung mass in the right lower lung field and a pneumothorax in the left lung. Chest computed tomography revealed a 5 x 3 cm sized mass in the right lower lobe and multiple thin-walled small cysts scattered in both lungs. Transbronchial biopsy of the lung mass was performed. The biopsy specimen showed atypical smooth muscle cell proliferation and cystic dilatation of the terminal bronchioles, which confirmed the diagnosis of lymphangioleiomyomatosis. To the best of our knowledge, this is the first case of lymphangioleiomyomatosis presenting as a lung mass.

Keyword

Lymphangioleiomyomatosis; Lung mass

MeSH Terms

Biopsy
Bronchioles
Diagnosis
Dilatation
Dyspnea
Emergency Service, Hospital
Female
Humans
Lung*
Lymphangioleiomyomatosis*
Myocytes, Smooth Muscle
Pneumothorax
Rare Diseases
Thorax

Figure

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  Figure 1 Chest X-ray shows a lung mass in right lower lung field and pneumothorax in left lung.

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  Figure 2 Chest computed tomography shows a 5 × 3 cm-sized mass in right lower lobe and multiple thin-walled small cysts scattered in both lungs.

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  Figure 3 Abdominal pelvic computed tomography shows multiple fat containing masses in both kidneys.

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  Figure 4 Biopsy of the right lower lobe mass shows proliferation of atypical smooth muscle cells and cystic dilatation of terminal bronchioles (H&E, × 100).

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  Figure 5 Immunohistochemical stain for HMB-45 shows cytoplasmic staining of smooth muscle cells.

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  Figure 6 Chest computed tomography after treatment of medroxyprogesterone. The size of the mass in right lower lobe has decreased.

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