title: Use of aromatase inhibitors in large cell calcifying sertoli cell tumors: effects on gynecomastia, growth velocity, and bone age.
abstract: BACKGROUND
  Large cell calcifying Sertoli cell tumors (LCCSCT) present in isolation or, especially in children, in association with Carney Complex (CNC) or Peutz-Jeghers Syndrome (PJS). These tumors overexpress aromatase (CYP19A1), which leads to increased conversion of delta-4-androstenedione to estrone and testosterone to estradiol. Prepubertal boys may present with growth acceleration, advanced bone age, and gynecomastia.
  
  
  OBJECTIVE
  To investigate the outcomes of aromatase inhibitor therapy (AIT) in prepubertal boys with LCCSCTs.
  
  
  METHODS
  Case series of a very rare tumor and chart review of cases treated at other institutions.
  
  
  METHODS
  Tertiary care and referral center.
  
  
  METHODS
  Six boys, five with PJS and one with CNC, were referred to the National Institutes of Health for treatment of LCCSCT. All patients had gynecomastia, testicular enlargement, and advanced bone ages, and were being treated by their referring physicians with AIT.
  
  
  METHODS
  Patients were treated for a total of 6-60 months on AIT.
  
  
  METHODS
  Height, breast tissue mass, and testicular size were all followed; physical examination, scrotal ultrasounds, and bone ages were obtained, and hormonal concentrations and tumor markers were measured.
  
  
  RESULTS
  Tumor markers were negative. All patients had decreases in breast tissue while on therapy. Height percentiles declined, and predicted adult height moved closer to midparental height as bone age advancement slowed. Testicular enlargement stabilized until entry into central puberty. Only one patient required unilateral orchiectomy.
  
  
  CONCLUSIONS
  Patients with LCCSCT benefit from AIT with reduction and/or elimination of gynecomastia and slowing of linear growth and bone age advancement. Further study of long-term outcomes and safety monitoring are needed but these preliminary data suggest that mammoplasty and/or orchiectomy may be foregone in light of the availability of medical therapy.
authors.affiliation: Section on Endocrinology and Genetics (M.K.C., E.G., M.L., C.A.S.), Program on Developmental Endocrinology and Genetics, Eunice Kennedy Shriver National Institutes of Child Health and Human Development, National Institute of Health, Bethesda, Maryland 20892; Division of Endocrinology (M.K.C.), Boston Children's Hospital, Boston, Massachusetts 02115; and Division of Pediatric Endocrinology (E.G.), Georgetown University, Washington, D.C. 20007.
authors.forename: Melissa K
authors.initials: MK
authors.lastname: Crocker
authors.forename: Evgenia
authors.initials: E
authors.lastname: Gourgari
authors.forename: Maya
authors.initials: M
authors.lastname: Lodish
authors.forename: Constantine A
authors.initials: CA
authors.lastname: Stratakis
mesh_terms: D000091162:AMP-Activated Protein Kinase Kinases; D000077384:Anastrozole; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D047072:Aromatase Inhibitors / Q000627:therapeutic use*; D001846:Bone Development / Q000187:drug effects*; D002114:Calcinosis / Q000188:drug therapy* / Q000473:pathology; D056733:Carney Complex / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D006128:Growth / Q000187:drug effects*; D006177:Gynecomastia / Q000188:drug therapy* / Q000209:etiology; D006801:Humans; D007223:Infant; D000077289:Letrozole; D008297:Male; D009570:Nitriles / Q000627:therapeutic use; D010580:Peutz-Jeghers Syndrome / Q000235:genetics; D017346:Protein Serine-Threonine Kinases / Q000235:genetics; D012707:Sertoli Cell Tumor / Q000150:complications / Q000188:drug therapy* / Q000473:pathology; D016896:Treatment Outcome; D014230:Triazoles / Q000627:therapeutic use
chemical_list: D000970:Antineoplastic Agents; D047072:Aromatase Inhibitors; D009570:Nitriles; D014230:Triazoles; D000077384:Anastrozole; D000077289:Letrozole; D017346:Protein Serine-Threonine Kinases; C109790:STK11 protein, human; D000091162:AMP-Activated Protein Kinase Kinases