Wilms' Tumor with Long-delayed Recurrence: 25 Years after Initial Treatment

Abstract

Wilms' tumor is one of the most frequent malignant neoplasms in childhood. Advances in treatment modalities such as the combination of chemoradiation therapy with surgery have enhanced overall survival. However, recurrence of Wilms' tumor is still a problem. In this case, a 28-year-old female had experienced intermittent abdominal pain, and the computed tomography scan showed a huge pelvic mass. The patient had a history of radical nephrectomy for Wilms' tumor with concurrent chemotherapy at the age of three. The pelvic mass was resected in February 2010 and was confirmed to be a recurrent Wilms' tumor. The recurrent tumor showed a classic triphasic Wilms' tumor growth pattern with frequent mitoses and tumor necrosis. Our case is an extraordinary case of a long-delayed recurrent Wilms' tumor after 25 years, which is the longest disease-free interval ever reported. The possible effects of chemotherapy as well as some other mechanisms of this late relapse are discussed.

Incidence and outcomes of patients with late recurrence of Wilms' tumor

“…Different authors described isolated experiences on LR, occurring 8, 9, 13, and 25 years after the original diagnosis. The lung was the site of recurrence in all but one case (in conjunction with opposite kidney in one case) [11][12][13]15,17]. Clausen reported a series of three patients with LR at 9, 10, and 11 years after initial diagnosis [9].…”

“…About 95% of first recurrences occur within 2 years of initial diagnosis, with the most frequent sites being lung/pleura, tumor bed, and liver. The information available on WT late recurrences (LR), conventionally regarded as recurrences occurring more than 5 years after diagnosis, comes from case reports or small series [9][10][11][12][13][14][15][16][17]. The Childhood Cancer Survivor Study reported that the leading cause of death in 5year survivors of childhood tumors was recurrence of the original tumor [18].…”

Relapsed Wilms' tumor with multiple brain metastasis

“…While recurrence of Wilms' tumor has been reported up to 25 years after primary diagnosis4), brain metastases usually appear in 2 years. Malogolowkin et al5) made a meta-analysis of 10 consecutive trials conducted in Wilms' tumor patients.…”

“…Beside these tumor suppressor genes, there are many defined molecular abnormalities on chromosomes 1p, 7p, 16q, 17p, and 19q78). Late recurrent diseases might have pathologic properties different from the primary disease4). Our patient had been treated in an outside clinic previously and we could not retrieve the past pathology slides to make a comparison between initial and late recurrent disease.…”

An unusual Wilms tumor with urethral recurrence: rare case report and literature review

“…Approximate relapse rates are believed to vary between 4.3% and 22.4% for favorable histology and 42% to 60% for unfavorable histology, respectively [27,28] . Most recurrent WT have been reported to occur in the first 2 years after the primary diagnosis, however late relapse of WT can also occur with one documented case of relapse occurring 25 years after initial treatment [29] . WT has the potential for both local and distant spread.…”