Late recurrence of Wilms’ tumour with exclusive skeletal muscle phenotype 23 years after primary diagnosis

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

“…Among the previously reported late recurrence cases, all but two cases showed similar histological features between the original and the recurrent tumors, with persistence of immature blastematous elements or nephrogenic rests [1,7]. The two cases with different morphologic features between the primary and recurrent tumors had a tumor relapsing interval of 23 years.…”

“…Most recurrent Wilms' tumors have been reported to occur in the first 2 years after the primary diagnosis. By contrast, long-delayed late recurrences, defined as 5 years after the initial diagnosis, are extremely rare events, and most of them have been reported in the form of individual case reports (Table 1) [1,7-10]. …”

“…In a recent report by Senetta et al [1] preoperative chemotherapy was shown to be able to enhance tumor differentiation, thus inducing tumor maturation with ablation of immature tumor components. In that study, the authors hypothesized that there are two different types of Wilms' tumor recurrence as follows: 1) the first group comprises cases in which chemotherapy is not effective, in which tumor recurrences have a short disease-free interval with a more undifferentiated tumor and more aggressive behavior, and 2) the second group comprises cases in which chemotherapy is effective, and tumor recurrences show a long disease-free interval with a high degree of differentiation and better survival related to the previous therapy.…”

“…In that study, the authors hypothesized that there are two different types of Wilms' tumor recurrence as follows: 1) the first group comprises cases in which chemotherapy is not effective, in which tumor recurrences have a short disease-free interval with a more undifferentiated tumor and more aggressive behavior, and 2) the second group comprises cases in which chemotherapy is effective, and tumor recurrences show a long disease-free interval with a high degree of differentiation and better survival related to the previous therapy. It can be postulated that if treatment is effective, the immature components of the tumor are more sensitive to preoperative chemotherapy and radiation treatment and are prone to be ablated more easily than are the mature components [1]. …”

“…It typically occurs in children less than 5 years of age and rarely in adults. Wilms' tumor is highly responsive to chemotherapy and radical nephrectomy, with about 90% of patients surviving at least 5 years, particularly patients with localized disease and favorable histology [1-3]. Despite therapeutic improvements, however, a subset of patients suffers from tumor relapse.…”