Adult Wilms’ tumor – diagnosis and current therapy

Abstract

IntroductionWilms’ tumour is one of the commonest malignant tumours of childhood. It appears mainly in the first 5 years of life. Incidental examples of nephroblastoma in adults have been described in literature (about 3% of all described cases). There are diagnostic and therapeutic difficulties in that older age group. The preoperative diagnosis of nephroblastoma in adults is difficult because there are no specific radiographic findings that allow to distinguished it from the more common adult renal tumors. Histopathologically, there is no difference between adult and childhood Wilms’ tumor.Materials and methodsThe PubMed database and current literature search was conducted for reports on clinical and histopathological features of nephroblastoma in adults. We also reviewed the literature in terms of treatment strategy, toxicity and prognostic factors.ResultsUp till now, several biological factors have been identified that may be in future new prognostic factors. Modern treatment regiments improved OS in this group of patients (OS rates of 90%). The prognosis remain still worse for about 25% of patients with anaplastic, bilateral and recurrent disease.ConclusionsDue to the fact that nephroblastoma is a very rare type of cancer, adult patients should be treated in an individual way based on the available schemes used in children. Toxicity in adults is higher than in children.

Retracted: Effects of MicroRNA‐19b on the Proliferation, Apoptosis, and Migration of Wilms’ Tumor Cells Via the PTEN/PI3K/AKT Signaling Pathway

“…Surprisingly, WT is correlated with congenital conditions while not associated with genetic syndromes, including urogenital malformations and mental retardation [Amirian, ]. Currently, the treatment for WT includes chemotherapy before the operation and subsequent surgery, as well as postoperative adjuvant therapy [Huszno et al, ]. Overall, the survival rate for WT reaches approximately 90%, but patients usually exhibit poor clinical prognosis if diffusely anaplastic or unfavorable histology is present [Amirian, ; Zhang et al, ].…”

Review of prognostic and predictive aspects of mutated TP53 in Wilms’ tumor biology with morphological report and molecular analysis of 37-year-old man’s nephroblastoma

“…The spectrum of histopathological morphology is the same for nephroblastomas of adulthood and childhood as both of them constitute the same entity, but schemes of postoperative therapy harbor greater toxicity in adults than in children [1]. The case presented by us showed a blastemal dominant histology in spite of the fact that epithelial type is themostcommoninadultnephroblastoma [14].In addition,thehighnumberofmitoses(8mitosesper 1HPF)definitelyexceedsthenumberof20mitoses per10HPF,andthethresholdofover20mitoseswas a feature found exclusively in intermediate or high risknephroblastomasandwasadditionallycorrelated with CD44 expression in a study of 39 cases [15].…”

“…Wilms' tumor is a common malignant neoplasm ofchildhood,whileitisincidentalinadulthoodwith afrequencyofapproximately3%ofallreportedcases [1].OnsetofbothchildhoodandadultWilms'tumor isprecededbymutationoftheWT1gene [2].However,WT1mutationsaredetectedinonly10%upto 15%ofpediatricWilms'tumors.Inaddition,adult Wilms' tumor was also reported to harbor a novel germline mutation in intron 1 of WT1: IVS1-6 C-->A [2].Incontrasttopediatrictumors,thepreoperativedetectionofadultWilms'tumor(AWT)is particularlyworrisomeasradiologicalimagingisnot specificenoughtodistinguishnephroblastomafrom the more frequent renal cancers of adulthood [1].…”

Spontaneous rupture of adult Wilms’ tumor: A case report and review of the literature

“…spontaneous rupture of adult Wilms' tumor Treatment guidelines in adults have not been established, most authors suggest adult patients can be treated according to pediatric protocols. 6 Radical nephrectomy is the treatment of choice for one-sided nephroblastoma. Multiple studies have demonstrated that laparoscopic renal surgery provides recuperative and cosmetic advantages in contrast to open surgery.…”