title: Response to Anastrozole Treatment in a Case with Peutz-Jeghers Syndrome and a Large Cell Calcifying Sertoli Cell Tumor.
abstract: Peutz-Jeghers syndrome (PJS) is inherited as an autosomal dominant trait characterized by multiple gastrointestinal hamartomatous polyps, mucocutaneous pigmentation, and an increased risk of neoplasm. Large-cell calcifying Sertoli cell tumor (LCCSCT) is a kind of sex cord-stromal tumor which may co-exist with PJS and which is characterized radiologically by calcification foci within the testes. Surgical treatment options for this tumor range from testis-preserving surgery to radical orchiectomy. Not with standing this invasive approach, recently, there are some case reports demonstrating the efficacy of aromatase inhibitors in avoiding orchiectomy and its associated complications. In this paper, we have presented a LCCSCT case diagnosed in a boy with PJS and his response to anastrozole treatment.
authors.affiliation: Ankara University Faculty of Medicine, Department of Pediatrics, Ankara, Turkey, Phone: +90 506 621 39 01 E-mail: drmervekoc13@hotmail.com.
authors.forename: Merve
authors.initials: M
authors.lastname: Koç Yekedüz
authors.forename: Zeynep
authors.initials: Z
authors.lastname: Şıklar
authors.forename: Berk
authors.initials: B
authors.lastname: Burgu
authors.forename: Zarife
authors.initials: Z
authors.lastname: Kuloğlu
authors.forename: Pınar
authors.initials: P
authors.lastname: Kocaay
authors.forename: Emine
authors.initials: E
authors.lastname: Çamtosun
authors.forename: Mehmet
authors.initials: M
authors.lastname: İsakoca
authors.forename: Aydan
authors.initials: A
authors.lastname: Kansu
authors.forename: Tarkan
authors.initials: T
authors.lastname: Soygür
authors.forename: Merih
authors.initials: M
authors.lastname: Berberoğlu
mesh_terms: D000077384:Anastrozole; D047072:Aromatase Inhibitors / Q000627:therapeutic use; D002648:Child; D006177:Gynecomastia / Q000150:complications / Q000188:drug therapy; D006801:Humans; D008297:Male; D009570:Nitriles / Q000627:therapeutic use*; D010580:Peutz-Jeghers Syndrome / Q000150:complications / Q000188:drug therapy*; D012707:Sertoli Cell Tumor / Q000150:complications / Q000188:drug therapy*; D013736:Testicular Neoplasms / Q000150:complications / Q000188:drug therapy*; D016896:Treatment Outcome; D014230:Triazoles / Q000627:therapeutic use*
chemical_list: D047072:Aromatase Inhibitors; D009570:Nitriles; D014230:Triazoles; D000077384:Anastrozole
keywords: Peutz-Jeghers syndrome; large-cell calcifying Sertoli cell tumor; prepubertal gynecomastia anastrozole.