Journal of clinical research in pediatric endocrinology · 2016
Response to Anastrozole Treatment in a Case with Peutz-Jeghers Syndrome and a Large Cell Calcifying Sertoli Cell Tumor.
Клинический случайСтатьяНазвание и аннотация: Перевод в очереди
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Koç Yekedüz Merve, Şıklar Zeynep, Burgu Berk, Kuloğlu Zarife, Kocaay Pınar, Çamtosun Emine, İsakoca Mehmet, Kansu Aydan, Soygür Tarkan, Berberoğlu Merih
Аннотация
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.