title: Massive hepatic angiomyolipoma in a young woman with tuberous sclerosis complex: significant clinical improvement during tamoxifen treatment.
abstract: OBJECTIVE
  Isolated liver angiomyolipomas (AMLs) occur in about 40% of TSC patients. Because of their slow growth, these tumors are often asymptomatic. Since AMLs express estrogen and progesteron receptors we suggest the possible benefits of tamoxifen for the treatment of liver AMLs.
  
  
  METHODS
  We report the case of a 26-year-old female affected by tuberous sclerosis (TSC2) with cerebral, renal and hepatic involvement admitted to the Liver Unit for severe malnutrition, anorexia and abdominal pain. MRI showed a grossly enlarged liver, causing severe gastric compression. The liver was entirely filled with multiple nodular lesions of different sizes. Liver biopsy showed tumoral tissue with microscopic and ultrastructural features of angiomyolipoma. All liver function tests were repeatedly normal. Prior to considering the patient for partial hepatectomy, she was administered tamoxifen (20mg b.i.d).
  
  
  RESULTS
  After 6 months of tamoxifen treatment a greatly improved quality of life and a significant weight gain were observed. After 12 months the clinical conditions further improved and the MRI showed a significant reduction of the largest lesion with a liquid central area and a diminished compression of the stomach.
  
  
  CONCLUSIONS
  This is to our knowledge the first report in which tamoxifen has been successfully used in a TSC patient with multiple liver angiomyolipomas.
authors.affiliation: Liver Unit, Tor Vergata University, Viale Oxford 81, 00133 Rome, Italy. ilaria.lenci@uniroma2.it
authors.forename: Ilaria
authors.initials: I
authors.lastname: Lenci
authors.forename: Mario
authors.initials: M
authors.lastname: Angelico
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Tisone
authors.forename: Antonio
authors.initials: A
authors.lastname: Orlacchio
authors.forename: Giampiero
authors.initials: G
authors.lastname: Palmieri
authors.forename: Mariangela
authors.initials: M
authors.lastname: Pinci
authors.forename: Roberta
authors.initials: R
authors.lastname: Bombardieri
authors.forename: Paolo
authors.initials: P
authors.lastname: Curatolo
mesh_terms: D000328:Adult; D018207:Angiomyolipoma / Q000188:drug therapy* / Q000209:etiology; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use*; D004305:Dose-Response Relationship, Drug; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000188:drug therapy* / Q000209:etiology; D013629:Tamoxifen / Q000627:therapeutic use*; D016896:Treatment Outcome; D014402:Tuberous Sclerosis / Q000150:complications*
chemical_list: D018931:Antineoplastic Agents, Hormonal; D013629:Tamoxifen