Endocrinology Department, Hedi Chaker Hospital, 3029 Sfax, Tunisia.
Ann Diagn Pathol. 2010 Apr;14(2):133-6. doi: 10.1016/j.anndiagpath.2009.06.007. Epub 2009 Sep 8.
A 15-year-old girl with Turner syndrome was unexpectedly found to have a left suprarenal mass. Extensive investigations showed a clinically and biochemically inapparent mass. Computed tomography disclosed a well-defined solid lesion in the left adrenal measuring 6.5 x 5 cm with minimal contrast enhancement. Laparoscopic adrenalectomy was done. Histologic examination revealed an encapsulated mass originated from the left adrenal medulla. Tumor tissue comprised abundant collagen fibers and spindloid cells admixed with mature ganglion cells. The tumor was diagnosed as left adrenal ganglioneuroma. According to literature, we report the eighth case of ganglioneuroma complicating Turner syndrome. Patients with this syndrome are predisposed to the development of neuroblastoma and related tumors. Reasons for this predisposition might relate to genetic and hormonal factors. Given that these tumors are often limited stage and of good prognosis, we recommend their screening in all patients with Turner syndrome.
一位 15 岁的特纳综合征女孩意外地发现左肾上腺有一个肿块。广泛的检查显示出临床上和生化上无明显的肿块。计算机断层扫描显示左肾上腺有一个界限清楚的实性病变，大小为 6.5 x 5 厘米，对比增强程度轻微。腹腔镜肾上腺切除术。组织学检查显示来源于左肾上腺髓质的包被性肿块。肿瘤组织由丰富的胶原纤维和梭形细胞与成熟的神经节细胞混合组成。肿瘤被诊断为左肾上腺节细胞神经瘤。根据文献，我们报告了第八例合并特纳综合征的节细胞神经瘤。患有这种综合征的患者易发生神经母细胞瘤和相关肿瘤。这种易感性的原因可能与遗传和激素因素有关。鉴于这些肿瘤通常处于局限期且预后良好，我们建议对所有特纳综合征患者进行筛查。
