Children's Hospital Wisconsin and Medical College of Wisconsin Milwaukee, WI.
Children's Hospital Wisconsin and Medical College of Wisconsin Milwaukee, WI.
Urology. 2019 Jul;129:197-199. doi: 10.1016/j.urology.2018.12.024. Epub 2019 Jan 2.
Extrarenal Wilms tumor is rare. We describe the first reported case of primary paratesticular extrarenal Wilms tumor with regional metastasis in 8-month old male with left undescended testis. Patient underwent left radical orchiectomy with regional lymph node excision. The inguinal node and paratesticular mass demonstrated the classic Wilms triphasic pattern, stained positively for WT-1 and demonstrated no loss of heterozygosity of chromosomes 1p and 16q. Work-up was negative for primary renal Wilms tumor or distant metastasis. Patient underwent adjuvant chemoradiation therapy for stage III disease. Patient is currently 2 years of age with no evidence of recurrence or metastatic disease.
肾外 Wilms 肿瘤罕见。我们描述了首例 8 月龄男性左侧未降睾丸患者发生的原发性睾丸旁肾外 Wilms 肿瘤伴区域性转移。患者接受了左侧根治性睾丸切除术和区域淋巴结切除术。腹股沟淋巴结和睾丸旁肿块表现出经典的 Wilms 三相模式，WT-1 染色阳性，且 1p 和 16q 染色体无杂合性缺失。进一步检查未发现原发性肾 Wilms 肿瘤或远处转移。患者因 III 期疾病接受了辅助化疗和放疗。目前患者 2 岁，无复发或转移疾病的证据。
