title: Neoplastic causes of abnormal puberty.
abstract: BACKGROUND
  Neoplasm-related precocious puberty (PP) is a rare presenting feature of childhood cancer. Moreover, evaluation of suspected PP in a child is complex, and cancer is often not considered. We characterized the clinicopathologic features of patients presenting with PP at a large pediatric cancer center, reviewed the relevant literature, and developed an algorithm for the diagnostic work-up of these patients.
  
  
  METHODS
  We examined the records of all patients with a neoplasm and concomitant PP treated at St. Jude Children's Research Hospital from January 1975 through October 2011, reviewed the available literature, and analyzed the demographic, clinical, endocrine, and neoplasm-related features.
  
  
  RESULTS
  Twenty-four of 13,615 children and adolescents (0.18%) were diagnosed with PP within 60 days of presentation. Primary diagnoses included brain tumor (12), adrenocortical carcinoma (5), hepatoblastoma (4), and others (3). PP was observed 0-48 months before diagnosis of neoplasm; 17 patients had peripheral PP and 7 had central PP.
  
  
  CONCLUSIONS
  Neoplasm-related PP is rare and takes the form of a paraneoplastic syndrome caused by tumor production of hormones or by alteration of physiologic gonadotropin production. PP can precede diagnosis of malignancy by months or years, and neoplastic causes should be considered early to avoid delayed cancer diagnosis. Treatment of the primary malignancy resolved or diminished PP in surviving patients with an intact hypothalamic-pituitary-gonadal axis.
authors.affiliation: Department of Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee.
authors.forename: Susanne
authors.initials: S
authors.lastname: Wendt
authors.forename: John
authors.initials: J
authors.lastname: Shelso
authors.forename: Karen
authors.initials: K
authors.lastname: Wright
authors.forename: Wayne
authors.initials: W
authors.lastname: Furman
mesh_terms: D000293:Adolescent; D000465:Algorithms; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008297:Male; D009367:Neoplasm Staging; D009369:Neoplasms / Q000150:complications* / Q000473:pathology; D011379:Prognosis; D011629:Puberty, Precocious / Q000175:diagnosis* / Q000209:etiology*
keywords: adrenocortical carcinoma; brain tumor; gonadal tumor; hepatoblastoma; paraneoplastic endocrinopathy; paraneoplastic precocious puberty