Department of Pathology, University of Ulsan College of Medicine, Asan Medical Center, Seoul 138-736, Korea.
Pathol Int. 2010 Jan;60(1):35-41. doi: 10.1111/j.1440-1827.2009.02468.x.
Teratoid Wilms' tumor is an unusual morphological entity characterized by a classic triphasic malignancy with predominantly heterologous tissue. The authors describe two cases of teratoid Wilms' tumor with an extrarenal site: one in a 13-year-old girl with vaginal spotting (patient 1) and another in a 1-day-old girl with a sacrococcygeal mass (patient 2). The tumors were located in the vagina and coccyx, respectively. Under the initial clinical diagnosis of sarcoma botryoides in patient 1 and teratoma in patient 2, the masses were removed. Microscopically, both tumors were composed of typical triphasic Wilms' tumor tissue with primitive cartilage and skeletal muscle, and squamous and columnar mucinous epithelia. The patient with sacrococcygeal mass (patient 2) had an elevated serum AFP level. The patients were given chemotherapy and have now remained disease free for 7 years 1 month, and 2 years 5 months after surgery, respectively. Familiarity with this rare variant of Wilms' tumor might be important in arriving at a correct diagnosis.
畸胎瘤性 Wilms 瘤是一种罕见的形态学实体，其特征为具有主要异源性组织的经典三相恶性肿瘤。作者描述了两例发生在肾外部位的畸胎瘤性 Wilms 瘤：一例发生于 13 岁阴道出血女孩（患者 1），另一例发生于 1 天大的女孩骶尾部肿块（患者 2）。肿瘤分别位于阴道和尾骨。根据患者 1 的最初临床诊断为横纹肌肉瘤，患者 2 的诊断为畸胎瘤，对两个肿块进行了切除。显微镜下，两个肿瘤均由典型的三相 Wilms 瘤组织组成，有原始软骨和骨骼肌，以及鳞状和柱状黏液上皮。患有骶尾部肿块的患者（患者 2）血清 AFP 水平升高。患者接受了化疗，分别在手术后 7 年 1 个月和 2 年 5 个月后至今无病生存。熟悉这种 Wilms 瘤的罕见变异型对于正确诊断可能很重要。
