J Clin Res Pediatr Endocrinol. 2017; 9(2): 168-171 | DOI: 10.4274/jcrpe.3625
Response to Anastrozole Treatment in a Case with Peutz-Jeghers Syndrome and a Large Cell Calcifying Sertoli Cell Tumor
Merve Koç Yekedüz1, Zeynep Şıklar2, Berk Burgu3, Zarife Kuloğlu4, Pınar Kocaay2, Emine Çamtosun2, Mehmet İsakoca2, Aydan Kansu4, Tarkan Soygür3, Merih Berberoğlu21Ankara University Faculty Of Medicine, Department Of Pediatrics, Ankara, Turkey
2Ankara University Faculty Of Medicine, Department Of Pediatric Endocrinology, Ankara, Turkey
3Ankara University Faculty Of Medicine, Department Of Pediatric Urology, Ankara, Turkey
4Ankara University Faculty Of Medicine, Department Of Pediatric Gastroenterology, Ankara, Turkey
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.
Keywords: Peutz-Jeghers syndrome, large-cell calcifying Sertoli cell tumor, prepubertal gynecomastia, anastrozole
Merve Koç Yekedüz, Zeynep Şıklar, Berk Burgu, Zarife Kuloğlu, Pınar Kocaay, Emine Çamtosun, Mehmet İsakoca, Aydan Kansu, Tarkan Soygür, Merih Berberoğlu. Response to Anastrozole Treatment in a Case with Peutz-Jeghers Syndrome and a Large Cell Calcifying Sertoli Cell Tumor. J Clin Res Pediatr Endocrinol. 2017; 9(2): 168-171
Manuscript Language: English