Department of Urology, Kyoto University Graduate School of Medicine, Kyoto, Kyoto, Japan.
J Pediatr Surg. 2010 Mar;45(3):E1-3. doi: 10.1016/j.jpedsurg.2009.12.005.
We report a case of testicular thecoma in an 11-year-old Japanese boy with nevoid basal-cell carcinoma syndrome (Gorlin syndrome). He presented with left testicular swelling and underwent a radical orchiectomy on suspicion of a malignant paratesticular tumor. The tumor arose from the testis exophytically and was diagnosed as a thecoma histopathologically. Ovarian thecoma-fibroma group tumors are closely associated with Gorlin syndrome or with abnormalities in PTCH, a candidate gene for the syndrome. The occurrence of an extremely rare testicular thecoma in this case (the second in the literature) suggests that such an etiological association may also exist in the pathogenesis of testicular tumors.
我们报告了一例患有神经嵴基底细胞痣综合征（Gorlin 综合征）的 11 岁日本男孩的睾丸支持细胞瘤病例。他因左侧睾丸肿胀就诊，因怀疑患有恶性副睾肿瘤而接受了根治性睾丸切除术。肿瘤从睾丸外生性生长，组织病理学诊断为支持细胞瘤。卵巢支持细胞瘤-纤维瘤组肿瘤与 Gorlin 综合征密切相关，或与该综合征的候选基因 PTCH 的异常相关。本例中极为罕见的睾丸支持细胞瘤的发生（文献中第二例）提示这种病因学关联也可能存在于睾丸肿瘤的发病机制中。
