Clinicohistological characteristics of renal cell carcinoma in children: A multicentre study

Abstract

Introduction: In this retrospective multicentre study, we compared the clinicohistological characteristics of renal cell carcinoma (RCC) between pediatric and adult patients. Methods: Data for patients who underwent radical or partial nephrectomy for RCC between 1988 and 2014 at multiple institutions were collected. Patients were divided into 2 groups according to age at diagnosis: pediatric patients (age ≤18 years) and adult patients (age ≥40 years). The groups were compared for clinical and pathologic variables, and survival analysis was performed. Results: The median follow-up period was 64 (range: 30-91) months for pediatric patients versus 44 (range: 19-59) months for adult patients (p = 0.026). Pediatric patients were mostly female (p = 0.003), had symptoms at presentation (p < 0.001), and had a high-stage tumour (p = 0.014) than adult patients. Among the symptomatic patients, gross hematuria was the most common symptom. The median tumour size was not different between groups. Regarding histologic types, pediatric patients had more papillary tumours (p < 0.001), more unclassified tumours (p < 0.001), and fewer clear cell carcinomas (p < 0.001). Five-year cancer-specific survival rates were 85% and 87.4% in pediatric and adult patients, respectively (log rank p = 0.901). Recurrence-free survival was better in adult patients, although this did not reach statistical significance (log rank p = 0.272). This study has several limitations, including its retrospective nature and the relatively small number of pediatric RCC cases. Conclusion: RCC in children is rare and is characterized by features that differ from those in adult RCC. Prognosis did not differ between groups.

Characteristics and Outcome of Children with Renal Cell Carcinoma: A Narrative Review

“…As a result, sixteen studies without potential overlap were included for overall analysis (nr. 1–16) [ 3 , 5 , 6 , 8 , 13 , 14 , 17 , 34 , 35 , 38 , 39 , 40 , 41 , 42 , 43 , 44 ] ( Figure 1 , Table 1 ). In order to provide transparency of all manuscripts that met the inclusion criteria and the selection process, we kept the eight excluded studies visible, but separate ( Table 1 ).…”

“…So far, seven pediatric studies used the WHO 2004 classification system including Xp11 / MiT translocation analysis, using predominantly immunohistochemical testing, and also fluorescence in situ hybridization (FISH) and/or next-generation sequencing (NGS) ( Table 3 ) [ 5 , 6 , 13 , 14 , 38 , 40 , 42 ]. In these studies, 140/317 (44%) patients were classified as MiT -RCC, 80/363 (22%) as papillary type and 36/363 (10%) as clear cell-type RCC ( Table 3 ).…”

Clinicopathologic Classification of Renal Cell Carcinoma in Patients ≤40 Years Old From Peru

“…One of the 7 studies included in the van der Beek review by Kim et al was a retrospective review without any pathologist as an author. 10 The remaining 5 studies were predominantly pathology focused yet are still difficult to compare to the current study for 2 reasons. First, their use of the 2004 WHO classification predated the acceptance of more currently accepted RCCs that were evaluated for in our study, including SDHB and FH-deficient RCCs, RCC with fibromyomatous stroma, and ESC RCC.…”

Distinguishing pediatric and adolescent renal cell carcinoma from other renal malignancies

“…In addition, the distinction between RCC and WT may implicate surgical approach. This issue has become increasingly discussed as recent reports have suggested a role for nephron sparing surgery (NSS) in pediatric RCC . This is in contrast to the surgical management of WT, as NSS is only indicated in a few rare settings.…”