Tuhan Hale, Abaci Ayhan, Sarsık Banu, Öztürk Tülay, Olguner Mustafa, Catli Gonul, Anik Ahmet, Olgun Nur, Bober Ece
a Faculty of Medicine, Department of Pediatric Endocrinology , Dokuz Eylul University , Izmir , Turkey.
b Faculty of Medicine, Department of Pathology , Ege University , Izmir , Turkey.
Acta Clin Belg. 2017 Aug;72(4):254-258. doi: 10.1080/17843286.2016.1215869. Epub 2016 Aug 4.
Intratubular large cell hyalinizing Sertoli cell neoplasia (ITLCHSCN) resulting from Sertoli cells of the testis are mainly reported in young adults and these are rarely seen in childhood. The most common presenting symptoms of the patients diagnosed with ITLCHSCN are gynecomastia, enlargement in the testicles, increase in growth velocity, and advanced bone age. Symptoms are basically resulting from increased aromatase enzyme activity in Sertoli cells. In this case report, an eight-and-a-half-year-old case presenting with complaint of bilateral gynecomastia since two years, showing no endocrine abnormality in laboratory during two years of follow-up, determined to have progression in bilateral gynecomastia, increase in testicular volumes, advanced bone age, increase in growth velocity in the clinical follow-up, and diagnosed with ITLCHSCN after testis biopsy was presented.
睾丸支持细胞来源的管内大细胞透明变性支持细胞瘤(ITLCHSCN)主要见于年轻成人,儿童罕见。诊断为ITLCHSCN的患者最常见的症状是男性乳房发育、睾丸增大、生长速度加快和骨龄提前。这些症状主要是由于支持细胞中芳香化酶活性增加所致。在本病例报告中,介绍了一名8岁半的患儿,自两年前起出现双侧男性乳房发育,在两年的随访中实验室检查未发现内分泌异常,临床随访发现双侧男性乳房发育进展、睾丸体积增大、骨龄提前、生长速度加快,经睾丸活检后诊断为ITLCHSCN。