• Journal of Chest Surgery
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• v.37 no.12
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• pp.1015-1018
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• 2004

Lymphangioleiomyomatosis (LAM) is a rare disease in women in childbearing age leading to progressive respiratory failure. LAM is characterized by an abnormal harmartomatous proliferation of smooth muscle cells surrounding the blood vessels, lymphatics and airways in the lung. This proliferation leads to airway obstruction, cystic alveolar change and lymphatic obstruction. Patients present with dyspnea, pneumothorax, cough, chest pain, hemoptysis, and chylous effusion. Although lung transplantation is the only therapeutic modality in end-stage LAM with respiratory failure, there has not been any report of successful treatment in Korea. We report one case of successful left single lung transplantation in a 40 year old woman suffering from end-stage LAM.

• Journal of Chest Surgery
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• v.38 no.9 s.254
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• pp.640-643
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• 2005

Intravenous leiomyomatosis is a rare neoplasm characterized by intravenous growth of histologically benign smooth muscle cell tumor. We report a case of intravenous leiomyomatosis with right atrial extension in a 19-year-old we-man. Various surgical techniques and approaches have been previously reported. In this case, the tumor was re-moved with a single-stage approach via laparotomy without cardiopulmorary bypass.

• Journal of Chest Surgery
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• v.37 no.11
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• pp.933-936
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• 2004

Intravenous leiomyomatosis is a rare disease entity of benign smooth muscle invading into the lumen of veins. We describe a case of intravenous leiomyomatosis originating from the uterus, growing in the inferior vena cava, and extending into the right ventricle association with multiple pulmonary metastasis. A 53-year-old woman with chest discomfort and several times attacks of syncope was treated at our hospital. The tumor was successfully removed with moderate hypothermic cardiopulmonary bypass after total hysterectomy with a bilateral salphingo-oophorectomy, and multiple pulmonary metastasis under simultaneous sternotomy and laparotomy was confirmed.

• Journal of Chest Surgery
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• v.38 no.6 s.251
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• pp.441-444
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• 2005

Abdominal tumors that can grow through vascular lumen and spread to the right heart are rare. Intravenous leiomyomatosis is a rare tumor that originates from the uterus and spreads through the vessels. Although histologically benign, tumor extension with mechanical obstruction of the inferior vena cava, right cardiac cavities, or even the pulmonary artery, may occasionally result in fatal outcome. The best treatment is complete surgical resection of the entire tumor using cardiopulmonary bypass and total circulation arrest, We report a case of intravenous leiomyomatosis of the uterus that showed intravascular growth up to the right atrium. The patient underwent successful resection of the tumor by one-stage cardiotomy with laparotomy.

• 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.

• Journal of Chest Surgery
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• v.39 no.4 s.261
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• pp.323-327
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• 2006

Lymphangioleiomyomatosis (LAM) is a rare disease in women of childbearing ages that eventually leads to respiratory failure. Lung transplantation is the only conclusive therapeutic modality in end-stage LAM. While single-lung transplantation is the preferred operation, the graft failure or recurrence of LAM was reported. We performed a single lung transplantation on a 36-year-old woman suffering from respiratory failure due to lymphangioleiomyomatosis. After a 1-year follow up, the patient was readmitted because of graft failure with collapsed transplanted lung. The lung volume reduction surgery (LVRS), tracheostomy and ventilator care were performed. However, neither the medical nor surgical treatment had any effect. Subsequently, we performed a contralateral single lung re-transplantation and had a good postoperative results.

• 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.

• Journal of Chest Surgery
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• v.27 no.7
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• pp.639-642
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• 1994

A 42-year-old woman with intravenous leiomomatosis that extended into the inferior vena cava and the right atrium is reported. A review of the literature with special comments on the surgical treatment of this rare condition is presented.

• Tuberculosis and Respiratory Diseases
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• v.53 no.2
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• pp.190-195
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• 2002

A benign metastasizing pulmonary leiomyomatosis(BMPL) is a rare and uncommon variety of leiomyoma. BML affects middle-aged women who had a previous hysterectomy due to a histologically benign appearing leiomyoma. BMPL is characterized by asymptomatic multiple smooth muscle nodules with a less aggressive course than a leiomyosarcoma. A 45-year-old woman who had a hysterectomy for a uterine leiomyoma 4 years prior to this study presented with multiple variable-sized lung nodule. A percutaneous needle biopsy confirmed the benign metastasizing pulmonary leiomyomatosis. Here, we present this case with a review of the relevant literatures.

• Journal of Chest Surgery
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• v.33 no.1
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• pp.112-115
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• 2000

A-13-urar-old with a history of Alport syndrome had been suffering from progressive dysphagia and postprandial vomiting for over 7 years. Exophagogram and manoemtric studies were consistent with achalasia. Barum study demonstrated marked esophageal dilatation and smooth tapered narrowing of the distal esophagus. However in spite of the medical treatment including the injection of the distal esophagus. However in spite of the medical treatment including the injection of botulinum toxin at the lesion site using an endoscope symptom did not improved and he suffered growth failure and malnutrition. Esophagectomy and esophagogastrostomy were performed to relieve the dysphagia. A firm circumferential intramural mass about 7$\times$5$\times$5 cm was found in the distal esophagus. The lumen of the esophagus was markedly dilated and esophageal wall was hypertrophied. Histologic examination of the neoplasm revealed a rather ill defined tumor tissue consisting of interlacing or whirling spindle cells without significant mitosis and esophageal leiomyo-matosis was confirmed. The patient was discharged uneventfully.