Oscar-Lambret center, department of pediatric oncology, AYA unit, Lille, France.
Trousseau hospital, department of pathology, Paris, France.
Bull Cancer. 2021 Feb;108(2):177-186. doi: 10.1016/j.bulcan.2020.09.007. Epub 2020 Oct 29.
Nephroblastoma or Wilms tumor, a common embryonal tumor in children, can occasionally occur in adults. The survival of patients older than 18 years is reported to be significantly inferior to that of pediatric patients. Establishing a diagnosis for these rare tumors can be challenging for both clinicians and pathologists, who are not accustomed to considering Wilms tumor as a potential differential in adults. This leads to misdiagnosis and a subsequent delay in the initiation of appropriate therapy. The standard of care is not well established for Wilms tumors in adults. We provide here a comprehensive review of the international literature on the subject with the current management protocols in France. We also propose the need of strong inter-disciplinary collaboration between surgeons, pathologists, and medical and pediatric oncologists for increasing knowledge and formulating treatment strategies for these rare tumors. Homogenous guidelines for treating adults with Wilms tumors have been proposed for all patients in France.
肾母细胞瘤或 Wilms 瘤,一种常见的儿童胚胎性肿瘤,偶尔也会发生于成年人。据报道,18 岁以上患者的存活率明显低于儿科患者。对于这些罕见肿瘤的诊断,临床医生和病理学家都可能感到具有挑战性,因为他们通常不会将 Wilms 瘤作为成年人的潜在鉴别诊断。这会导致误诊,并随后延误开始适当的治疗。对于成年人的 Wilms 瘤,其标准治疗方法尚未确立。我们在此提供了国际文献综述,以及法国当前的管理方案。我们还提出需要外科医生、病理学家、内科和儿科肿瘤学家之间进行强有力的跨学科合作,以增加对这些罕见肿瘤的认识并制定治疗策略。法国已经为所有成年 Wilms 瘤患者提出了同质的治疗指南。