Case series of adult Wilms' tumor and review of the literature.

Background: Adult Wilms' tumor (AWT) is extremely rare in clinics and very difficult to diagnose preoperatively. However, a unified treatment plan of AWT is lacking and the prognosis is unfavorable. This study will present the follow-up data of AWT cases in Tongji Hospital and discuss the diagnosis, treatment, and prognosis of AWT.

Case description: The clinical data of four AWT cases admitted to the Department of Urology, Tongji Hospital, Tongji Medical College, Huazhong University of Science and Technology from August 2010 to January 2019 were followed up and analyzed. The mean age of the three males and one female patients was 38 years old. One case was admitted due to lumbar and abdominal distension, one case due to renal occupancy found on routine physical examination, and the other two cases due to lumbar pain with painless gross hematuria. The tumors were 4-10 cm in size, three of which were confined to the kidney, and one case invaded the inferior vena cava. Four cases were treated surgically: three with radical nephrectomy and one with partial nephrectomy. Two cases were treated with postoperative adjuvant chemotherapy (one case received the actinomycin D + vincristine regimen and pirarubicin + vincristine regimen; another case was treated with etoposide + carboplatin and cyclophosphamide + vincristine + adriamycin), and the other two cases were received postoperative follow-up only. Similar cases were reviewed in the literature. According to the postoperative pathology, one case was AWT germ type, two cases were AWT epithelial type, and one case was AWT mixed type. Two cases were classified in stage I, one case in stage II, and one case in stage III. All cases were followed up over 5 years, with two deaths and two survivals and a mean progression-free survival of 39 months.

Conclusions: AWT is a relatively rare malignant tumor with a low preoperative diagnostic accuracy and a poorer prognosis than children. Early diagnosis, successful surgery, and standardized postoperative adjuvant therapy according to pathological typing and staging are the key points to improve the survival of AWT patients.