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 Þýklar², Berk Burgu³, Zarife Kuloðlu⁴, Pýnar Kocaay², Emine Çamtosun², Mehmet Ýsakoca², Aydan Kansu⁴, Tarkan Soygür³, Merih Berberoðlu^2
1Ankara 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

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