Stage III cystic partially differentiated nephroblastoma recurring after nephrectomy and chemotherapy

Abstract

[1,2]. Pathologically, CPDN is distinct from Wilms tumor in that the tumor is entirely cystic with no solid component. The thin septa typically house blastema, with or without embryonal stromal or epithelial elements. Clinically, CPDN presents similarly to Wilms tumor, but it has been described as a relatively benign tumor with low malignant potential [2][3][4].Evidence suggests that stage I CPDN may be treated with nephrectomy alone, while chemotherapy is reserved for higher stage disease [2][3][4][5][6][7][8]. Stages II-V disease is uncommon, therefore, there is less experience with these patients, and little is known about the best way to treat them. In this report, we describe the case of a 1-year-old diagnosed with stage III CPDN having recurrent disease after total nephrectomy and chemotherapy.Cystic partially differentiated nephroblastoma (CPDN) has low malignant potential. We report a 1-year-old with stage III CPDN of the right kidney that recurred following radical nephrectomy and chemotherapy. There was evidence of tumor spillage pre-operatively and intra-operatively. During chemotherapy the disease recurred in the omentum and the peritoneum. Pathology of the recurrent resected cysts revealed a more differentiated biphasic tumor without blastemal elements. It appears that spillage of CPDN in our patient led to dissemination of disease. Chemotherapy failed to prevent recurrence but only mature elements were present following this treatment. The intensity of therapy required to treat CPDN remains undefined. Pediatr Blood Cancer 2008;50:129-131.

Multiloculated cystic renal tumors of childhood: Has the final word been spoken

“…There are 3 documented cases of recurrence with CPDN in the literature [ Table 3 ]. [ 1 4 11 ] This reiterates the caution by Joshi and Beckwith that the presence of poorly differentiated tissue and blastemal elements suggests possible malignant potential. In the cases where the histology was available, the recurrence was Wilms tumor.…”

Clinical and Molecular Characteristics and Outcome of Cystic Partially Differentiated Nephroblastoma and Cystic Nephroma: A Narrative Review of the Literature

“…Stage I was recorded for 62 patients, stage II for one patient with tumor extension beyond the kidney [12], and stage III for nine patients. Stage III disease was based on invasion of the abdominal wall (n = 1) [17], pre-or intra-operative tumor rupture (n = 7) [8,12,18,19], or not specified [14]. In six patients, the tumor prolapsed into the ureter.…”

“…Fifty-two patients did not receive any post-operative therapy based on the histological diagnosis of CPDN, while information on post-operative therapy was not available in 14 patients. Reported reasons for post-operative treatment (if specified) included tumor rupture or spill (n = 7) [8,12,18,19], the presence of nephroblastomatosis (n = 1) [13], and the aim to reduce serum α-fetoprotein levels, which occurred in one patient [21]. In the other 38/47 post-operatively treated patients, reasons for this decision were not specified.…”

“…Only four case reports described detailed treatment-related toxicity occurring in a total of seven patients [12,17,18,22]. Two of these seven patients suffered from vincristine-related peripheral neuropathy [12], two developed intermittent viral illnesses and fevers [18,22], and three developed hematologic toxicity, of which one had recurrent episodes of sepsis [12,17,22]. These toxicities resulted in dose reduction or a delay of chemotherapy in these patients and one patient required supportive care therapy consisting of blood and platelet infusions [17].…”

“…These toxicities resulted in dose reduction or a delay of chemotherapy in these patients and one patient required supportive care therapy consisting of blood and platelet infusions [17]. A total of four relapses were described (Table 3) [8,15,18]. In three of these cases, a differential diagnosis of cystic Wilms tumor could not be excluded based on the histological description in the original report, as most likely histology was not reviewed by a panel of pediatric pathologists involved in the SIOP-RTSG or Children's Oncology Group (COG), and a detailed description was lacking.…”