Bretagne M C, Chabat B, L'Hermite J, Guillemin P, Beau A, Treheux A
J Radiol Electrol Med Nucl. 1975 Jan;56(1):5-11.
The authors report 5 cases of embryonic sarcoma of the uro-genital sinus. 3 children, aged respectively, 17 months, 2 1/2 years, 6 years and two adolescents of 17 and 19 years. There were 3 boys and 2 girls. In only one case was the tumour obvious on clinical examination. In all the other cases, it caused urinary retention. On cystography, there was a polycyclic vesical filling defect resembling a parachute, which is specific of the botryoid variety and always leads to the right diagnosis. In girls, sarcoma of the urogenital sinus above the bladder, may be confused with other pelvic tumours. The authors recall the importance of lateral views of cystography to eliminate, if necessary, low paravertebral neurogenic tumours or sacro-coccygeal dysembryomas. After discussing the pathology, and the clinical and radiological findings, the authors then review rapidly the present methods of treatment which give a large number of survivals and should lead one to treat all cases, even when there are recurrences or metastases.
作者报告了5例泌尿生殖窦胚胎性肉瘤病例。其中3例为儿童,年龄分别为17个月、2岁半、6岁,另外2例为17岁和19岁的青少年。患者中有3名男孩和2名女孩。仅1例在临床检查时肿瘤明显。在其他所有病例中,肿瘤均导致尿潴留。膀胱造影显示有一个多环状膀胱充盈缺损,类似降落伞,这是葡萄状型的特征性表现,总能得出正确诊断。在女孩中,膀胱上方泌尿生殖窦的肉瘤可能与其他盆腔肿瘤相混淆。作者提醒膀胱造影侧位片的重要性,必要时可排除低位椎旁神经源性肿瘤或骶尾部发育异常瘤。在讨论了病理学、临床和放射学表现后,作者随后简要回顾了目前的治疗方法,这些方法能使大量患者存活,即使存在复发或转移,也应适用于所有病例。