title: Coexistence of pulmonary lymphangioleiomyomatosis and pulmonary angiomyolipoma.
abstract: Lymphangioleiomyomatosis (LAM) and angiomyolipoma are two different, but related rare diseases. To the best of our knowledge, pulmonary LAM and pulmonary angiomyolipoma have not previously been observed in the same patient.
  
  
  
  A 38-year-old woman presented with a dry cough and left flank pain. She had a right nephrectomy for renal angiomyolipoma 17 years ago. A magnetic resonance imaging scan demonstrated a round mass in the left kidney. A chest computed tomography scan demonstrated scattered small thin-walled cysts and multifocal round nodules in both lungs. A lung biopsy via video-assisted thoracoscopic surgery revealed that the cysts and nodules were manifestations of LAM and angiomyolipomas, respectively. After sirolimus therapy, the renal angiomyolipoma and metastasized pulmonary angiomyolipomas shrank, but pulmonary cysts were unchanged.
  
  
  
  LAM and angiomyolipoma are significantly associated, and may coexist in the lungs in rare cases. Sirolimus is effective for both renal angiomyolipoma and metastasized pulmonary angiomyolipomas.
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Beijing, 100730, China.
authors.forename: Xuefeng
authors.initials: X
authors.lastname: Sun
authors.affiliation: Department of Pathology, Peking Union Medical College Hospital, Beijing, China.
authors.forename: Ruie
authors.initials: R
authors.lastname: Feng
authors.affiliation: Department of Thoracic Surgery, Peking Union Medical College Hospital, Beijing, China.
authors.forename: Ye
authors.initials: Y
authors.lastname: Zhang
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Beijing, 100730, China.
authors.forename: Juhong
authors.initials: J
authors.lastname: Shi
authors.affiliation: Department of Respiratory Medicine, Peking Union Medical College Hospital, Beijing, 100730, China. Kaifeng.xu@gmail.com.
authors.forename: Kai-Feng
authors.initials: KF
authors.lastname: Xu
mesh_terms: D000328:Adult; D018207:Angiomyolipoma / Q000000981:diagnostic imaging* / Q000628:therapy; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000150:complications / Q000000981:diagnostic imaging*; D008168:Lung / Q000473:pathology; D008175:Lung Neoplasms / Q000000981:diagnostic imaging* / Q000628:therapy; D018192:Lymphangioleiomyomatosis / Q000000981:diagnostic imaging* / Q000628:therapy; D008279:Magnetic Resonance Imaging; D020123:Sirolimus / Q000627:therapeutic use; D020775:Thoracic Surgery, Video-Assisted; D014057:Tomography, X-Ray Computed
chemical_list: D020123:Sirolimus
keywords: Angiomyolipoma; Case report; Lung; Lymphangioleiomyomatosis