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

Abstract

Background:Multicystic renal tumors which include cystic nephroma, cystic partially differentiated nephroblastoma (CPDN) and cystic Wilms tumor has been a diagnostic and therapeutic challenge. Histopathological examination has been the only reliable differentiating method. Management of these tumors is still riddled with controversy as a definitive preoperative differentiation between the three has not been possible.Methods:A retrospective evaluation was performed of the treatment strategies employed with nine cases of multicystic renal tumors treated from 2005 to 2015.Results:The median age at presentation was 12 months with all except one being boys. All except two children underwent primary surgery. The median follow-up was 50 months with six children having long-term survival. One child succumbed to the disease process, one died due to an unrelated cause and another was lost to follow-up. Although there was no ambiguity with cases of cystic nephroma (CN) and cystic Wilms tumor, three of the four cases of CPDN had problems.Conclusion:Primary surgery for multicystic renal tumors is safe and should be seriously considered as it prevents overtreatment in cases of CN and early stage CPDN. Further studies are needed to fully understand the biological behavior of CPDN.

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

“…A total of 108 patients presented with unilateral disease, while five presented with bilateral disease (4.4%) [ 8 , 12 , 13 , 14 , 15 ], and none of the patients had metastatic disease. Three of the bilateral cases had CPDN in both kidneys [ 12 , 14 , 15 ]. Of the other two patients, one had CPDN with contralateral nephroblastomatosis [ 13 ] and one had CPDN with Wilms tumor, with focal anaplasia in the other kidney [ 8 ].…”

“…Pre-operative chemotherapy administration was reported in 20 out of 98 patients with reported details on the pre-operative therapy and response ( Table 2 ) [ 12 , 14 , 15 , 17 , 19 , 20 , 21 , 22 , 23 , 24 ], including two of the five patients with bilateral disease [ 12 , 14 ]. None of the pre-operatively treated patients showed response to chemotherapy on imaging or histological examination.…”

“…None of the pre-operatively treated patients showed response to chemotherapy on imaging or histological examination. In three of them, pre-operative imaging revealed an increase in tumor size after chemotherapy [ 14 , 15 , 23 ]. Seventy-eight patients underwent upfront surgery without chemotherapy.…”

“…The only patient who did not have surgery was a patient with extensive comorbidity, where a conservative treatment approach was chosen, following the parents’ wishes [ 25 ]. The surgical approach was radical nephrectomy in 94 cases and nephron sparing surgery (NSS) in eight patients, with the latter including all five bilateral patients [ 8 , 12 , 13 , 14 , 15 ]. The type of surgery was not described in 10 cases.…”

Expression and clinical significance of p73 in Wilms' tumor in children

“…Highly homologous to the partial sequence of p53 gene, the p73 gene is located at 1p36.2~36.3 and has been proven to be closely related to the occurrence and development of various tumors with overexpression (4). Lowly expressed in normal tissues and excessively expressed in tumor tissues, the p73 is closely related to the proliferation and migration of tumor cells and the prognosis of patients (5). Moreover, it has unique functions in the metabolic regulation (6), neurodevelopment and differentiation (7), as well as the regulation of spermatogenesis and male fertility (8).…”