title: Pulmonary lymphangioleiomyomatosis with concomitant tuberous sclerosis complex diagnosed by video-assisted thoracoscopic surgery.
abstract: 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.
authors.affiliation: Operating Room Division, Tottori University Hospital, 36-1 Nishi-cho, Yonago, Tottori 683-8504, Japan. kuichi@grape.med.tottori-u.ac.jp
authors.forename: Yuji
authors.initials: Y
authors.lastname: Taniguchi
authors.forename: Tomohiro
authors.initials: T
authors.lastname: Haruki
authors.forename: Shinji
authors.initials: S
authors.lastname: Fujioka
authors.forename: Yoshin
authors.initials: Y
authors.lastname: Adachi
authors.forename: Ken
authors.initials: K
authors.lastname: Miwa
authors.forename: Hiroshige
authors.initials: H
authors.lastname: Nakamura
mesh_terms: D000328:Adult; D000951:Antigens, Neoplasm; D000970:Antineoplastic Agents / Q000627:therapeutic use; D005260:Female; D006801:Humans; D033162:Incidental Findings; D008175:Lung Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D018192:Lymphangioleiomyomatosis / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy / Q000473:pathology / Q000601:surgery; D058950:Melanoma-Specific Antigens; D032389:Myocytes, Smooth Muscle / Q000737:chemistry / Q000276:immunology / Q000473:pathology*; D009363:Neoplasm Proteins / Q000032:analysis; D010844:Picibanil / Q000627:therapeutic use; D011030:Pneumothorax / Q000209:etiology / Q000601:surgery*; D011960:Receptors, Estrogen / Q000032:analysis; D011980:Receptors, Progesterone / Q000032:analysis; D020775:Thoracic Surgery, Video-Assisted; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000150:complications* / Q000601:surgery
chemical_list: D000951:Antigens, Neoplasm; D000970:Antineoplastic Agents; D058950:Melanoma-Specific Antigens; D009363:Neoplasm Proteins; D011960:Receptors, Estrogen; D011980:Receptors, Progesterone; D010844:Picibanil