title: Clinical features of lymphangioleiomyomatosis complicated by renal angiomyolipomas.
abstract: OBJECTIVE
  Renal angiomyolipomas (R-AMLs) are major complications of lymphangioleiomyomatosis (LAM). The objective of this study was to better understand the influence of R-AMLs in patients with LAM on the prognosis and other clinical factors related to respiration, and to investigate the management of R-AMLs in patients with LAM.
  
  
  METHODS
  We retrospectively investigated the clinical features of 7 patients with LAM [4 were TSC (Tuberous sclerosis complex)-LAM and 3 were S (sporadic)-LAM] complicated by R-AMLs admitted to our hospital from 1997 to 2008.
  
  
  RESULTS
  All patients were females and the mean age at diagnosis of LAM was 40.7 years (31.7 years for TSC-LAM and 52.7 years for S-LAM). Although 5 patients had symptoms related to R-AMLs, only 1 patient experienced symptoms related to R-AMLs at the time of diagnosis. Five patients had bilateral and 2 patients had unilateral R-AMLs. R-AMLs ruptured in 4 cases (3 patients were TSC-LAM) including 2 patients in whom they ruptured bilaterally, and who underwent bilateral nephrectomy. In 1 case, unilateral R-AMLs grew larger and appeared on the other side during the follow-up period.
  
  
  CONCLUSIONS
  Although only rare cases of LAM show symptoms related to R-AMLs initially, R-AMLs are a notable complication. To avoid nephrectomy, R-AMLs should be diagnosed when they are small and should be followed up carefully by periodic echograms or CT scans.
authors.affiliation: Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Japan. mizushina@jichi.ac.jp
authors.forename: Yoshiko
authors.initials: Y
authors.lastname: Mizushina
authors.forename: Masashi
authors.initials: M
authors.lastname: Bando
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Hosono
authors.forename: Naoko
authors.initials: N
authors.lastname: Mato
authors.forename: Takakiyo
authors.initials: T
authors.lastname: Nakaya
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Ishii
authors.forename: Hideaki
authors.initials: H
authors.lastname: Yamasawa
authors.forename: Yukihiko
authors.initials: Y
authors.lastname: Sugiyama
mesh_terms: D000328:Adult; D018207:Angiomyolipoma / Q000175:diagnosis* / Q000628:therapy; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000628:therapy; D008175:Lung Neoplasms / Q000175:diagnosis* / Q000628:therapy; D018192:Lymphangioleiomyomatosis / Q000175:diagnosis* / Q000628:therapy; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000628:therapy; D009392:Nephrectomy; D011379:Prognosis; D012189:Retrospective Studies; D014402:Tuberous Sclerosis / Q000175:diagnosis / Q000628:therapy