• Tuberculosis and Respiratory Diseases
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• v.39 no.3
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• pp.266-270
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• 1992

Pulmonary lymphangioleiomyomatosis is a rare disease, prevalent in women of reproductive age, and characterized by proliferation of atypical smooth muscle in the lung. A 44-year-old women with pulmonary lymphangioleiomyomatosis was presented. Chest radiographs showed diffuse increased reticulonoular infiltrations on the almost entire lung and high resolution CT illustrated multiple, well defined cystic lesions which were distributed diffusely through the lung. Histologic confirmation of lymphangioleiomyomatosis was made upon open lung biopsy. Treatment with methylprogesterone was initated.

• Tuberculosis and Respiratory Diseases
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• v.39 no.6
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• pp.542-547
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• 1992

Tuberous sclerosis is an autosomal dominant disorder characterized by mental retardation, epilepsy, and adenoma sebaceum. Associated lesions include retinal phacomata, shagreen patches, subungal fibromata, and benign visceral tumors such as pulmonary lymphangioleiomyomatosis. Lymphangioleiomyomatosis occurs exclusively in women, usually during the child-bearing years, and is characterized by proliferation of smooth muscle along the lymphatic vessels of the lung, thorax, abdomen. Proliferation of smooth muscle results in interstitial and obstructive lung disease, recurrent pneumothorax, and chylous pleural effusions. We saw two cases of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in women of reproductive age. We report the cases with a brief review of the literatures.

• Tuberculosis and Respiratory Diseases
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• v.50 no.1
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• pp.127-131
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• 2001

Pulmonary lymphangioleiomyomatosis is a disease involving the proliferation of atypical smooth muscle cells from the perilymphatics, peribronchial and perivascular region of the lung and the retroperitneum. The disease usually affects women of child-bearing age. We recently experienced a case of pulmonary lymphangioleiomyomatosis in a 31-year-old women who had suffered from a chylous pleural effusion. Histologic confirmation of lymphangioleiomyomatosis Was made upon a video-associated thoracoscopic lung biopsy. Here we report this case with a brief review of the literature.

• Tuberculosis and Respiratory Diseases
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• v.42 no.2
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• pp.244-249
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• 1995

We experienced one case of pulmonary lymphangioleiomyomatosis in 26-year-old female patient. She had taken antituberculous medication under the impression of miliary tuberculosis on simple chest X-ray at peripartum period. On outpatient follow-up she complained of progressive exertional dyspnea in spite of medication. Through careful history taking and physical examination, high resolutional CT, and open lung biopsy she was diagnosed as pulmonary lymphangioleiomyomatosis combined with incomplete type of tuberous sclerosis. So, we presented the case with the brief review the literatures.

• Tuberculosis and Respiratory Diseases
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• v.48 no.5
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• pp.788-793
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• 2000

Tuberous sclerosis is a rare hereditary anomaly of variable penetrance characterized by mental retardation, epilepsy, and adenoma sebaceum. Pulmonary involvement is uncommon, but, when involved by tuberous sclerosis, it shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is a rare disease of unknown cause that affects women of reproductive age. It is characterized by progressive proliferation of smooth muscle in the lung. We experienced one case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis in a 26-year-old female patient. This case is reported with a brief review of the literature.

• Journal of Yeungnam Medical Science
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• v.27 no.1
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• pp.63-68
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• 2010

Lymphangioleiomyomatosis (LAM) is a rare, cystic lung disease that is associated with mutation in the tuberous sclerosis genes, renal angiomyolipomas, lymphatic spread and a remarkable female gender predilection. The pathology of LAM is represented by the proliferation of immature smooth muscle cells in the walls of airways, and venules and lymphatic vessels in the lung. The clinical course of LAM is characterized by progressive dyspnea on exertion, recurrent pneumothorax and collections of chylous fluid. The diagnosis of pulmonary LAM can be made on chest X-ray, a high-resolution CT scan and lung biopsy. We experienced a case of pulmonary lymphangioleiomyomatosis in a 28-years-old female patient who had suffered from progressive dyspnea on exertion, so we report on it along with a brief review of the relevant literature.

• Journal of Chest Surgery
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• v.32 no.3
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• pp.326-329
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• 1999

Pulmonary lymphangioleiomyomatosis is a rare disease occurring almost exclusively in woman of reproductive age. It is characterized by rapid deterioration of respiratory functions and results in death within an average of 10 years. This disease is caused by a progressive proliferation of atypical smooth muscle in the pulmonary lymphatic vessels, blood vessels, and airways leading to the development of distal cystic changes which causes frequent recurrent pneumothoraces. We experienced a case of pulmonary lymphangioleiomyomatosis in a 30-year old woman who had a history of bilateral, recurrent pneumothoraces. The patient underwent lung biopsy through right thoracotomy which revealed the diagnosis of pulmonary lymphangioleiomyomatosis. We report a case with a review of the literature.

• Journal of Chest Surgery
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• v.37 no.12
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• pp.1029-1031
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• 2004

We had expierienced pulmonary lymphangioleiomyomatosis(LAM) with bilateral chylothorax and chylous ascites. A twenty-one-year-old lass with chief complaint of abdominal pain was admitted through the emergency room. She received emergent pelvicoscopic surgery for the rupture of the right corpus luteum. We aspirated 1000ml of the uncoagulated blood. The bleeding point was cautherized electrically. LAM was diagnosed with tissue from the retroperitoneum. Chylous ascites and bilateral chylothorax were occurred despite of various treatments. On thoracotomy, bullous changed lung and lymphatic leakage from visceral and parietal pleura were observed. She died of respiratory insufficiency and general weakness after 6 months from admission.

• Tuberculosis and Respiratory Diseases
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• v.63 no.3
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• pp.289-293
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• 2007

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 $5{\times}3cm$ 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.

• Journal of Chest Surgery
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• v.32 no.11
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• pp.1057-1059
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• 1999

Lymphangioleiomyomatosis(LAM) is defined as an abnormal proliferation of smooth muscles in the lung tissue throughout lymphatics, vascular and bronchial structure. A 52-year-old postmenopausal woman was admitted to our hospital for recurrent pneumothorax. She was treated for medroxyprogesterone by LAM 1 month ago. We performed operation of pulmonary partial resection and pleurodesis. The patient is receiving continuous medroxyprogesterone and Leuplin administration, and currently, 6 months after the operation, is still showing good results.