picibanil and Lymphangioleiomyomatosis

We herein describe the case of a 20-year-old woman who was diagnosed as having tuberous sclerosis complex (TSC) at the age of 10 years. The patient had a history of right pneumothorax at the age of 19. This time, a right pneumothorax recurrence was detected, and video-assisted thoracoscopic surgery (VATS) was performed. In the intraoperative findings, an infinitesimal lung cyst was detected on the lung lobe, and partial resection was performed. Pathologically, antibody-positive smooth muscle cells of the human melanoma block (HMB)-45 had grown and been diagnosed as pulmonary lymphangioleiomyomatosis (LAM). About half a year later, left pneumothorax occurred, and VATS was performed again. Pathologically, antibody-positive smooth muscle cells of HMB-45 were not detected. Occasionally, TSC is known to cause LAM complications, but in some cases it is difficult to make an accurate pathological diagnosis. Making a pathological diagnosis based on the VATS findings and taking a sufficient amount of tissue specimen are considered useful for making the diagnosis.

Topics: Adult; Antigens, Neoplasm; Antineoplastic Agents; Female; Humans; Incidental Findings; Lung Neoplasms; Lymphangioleiomyomatosis; Melanoma-Specific Antigens; Myocytes, Smooth Muscle; Neoplasm Proteins; Picibanil; Pneumothorax; Receptors, Estrogen; Receptors, Progesterone; Thoracic Surgery, Video-Assisted; Treatment Outcome; Tuberous Sclerosis

We evaluated the efficacy of pleurodesis using OK-432 (Picibanil) for the treatment of pneumothorax associated with pulmonary lymphangioleiomyomatosis (LAM). Seven episodes of pneumothorax in five patients with LAM were treated with pleurodesis using OK-432. First, all patients underwent tube thoracostomy drainage. After drainage, 5 or 10 KE of OK-432 was administered via the tube. If incomplete lung expansion or a continuous air leak occurred, an additional 5 KE of OK-432 was administered. Of the five patients, two developed pneumothorax for the first time and three had a history of previous pneumothorax. The mean total dose of OK-432 administered was 13.6 KE, and the mean period of tube drainage was 11 days. The only recurrence of ipsilateral pneumothorax after OK-432 pleurodesis was observed seven years and eight months later in association with chronic respiratory failure in one patient. This patient was successfully treated with repeated pleurodesis using OK-432 during mechanical ventilation, and no recurrence has developed in the eight years since then. The main side effects of the procedure with OK-432 were fever and chest pain, which were well controlled by non-steroidal anti-inflammatory drugs. The study concluded that pleurodesis with OK-432 was an effective and safe treatment for intractable and recurrent pneumothorax associated with LAM.

Topics: Adult; Female; Humans; Lung Neoplasms; Lymphangioleiomyomatosis; Middle Aged; Picibanil; Pleurodesis; Pneumothorax; Recurrence; Retrospective Studies; Treatment Outcome

After the establishment of transplantation law, the right pulmonary transplantation was done from the brain dead donor for the first time in Japan at the attached hospital of Institute of Development, Aging and Cancer, Tohoku University on March 29, 2000. The woman in 30's had been deteriorated from progressive lymphangiomyomatosis since 1984. She was waiting for the right lung transplantation in the related hospital of the Institute Hospital since May, 1999. A brain dead donor appeared in Tokyo. After receiving the information from Japan Organ Transplant Network, we immediately started for Tokyo in order to procure the lung. We carried the right lung by Shinkansen. The lung of the donor was transplanted to the patient under the partial extracorporeal circulation on March 29, 2000. The total operating time was 6 hours and 7 minutes: 5 hours and 20 minutes for total ischemic period of the lung and 2 hours and 7 minutes for using extracorporeal circulation. The total amount of bleeding during the operation was 3,695 ml. Postoperative course of the transplanted patient was fair except chylothorax in the operated side, which was successfully controlled by intrathoracic infusion with OK-432. The transplanted patient was discharged from the hospital after 75 days postoperatively.

Topics: Adult; Brain Death; Chylothorax; Female; Humans; Japan; Lung Neoplasms; Lung Transplantation; Lymphangioleiomyomatosis; Picibanil; Postoperative Complications; Tissue Donors; Treatment Outcome