Characteristics and outcome of pediatric renal cell carcinoma patients registered in the International Society of Pediatric Oncology (SIOP) 93‐01, 2001 and UK‐IMPORT database: A report of the SIOP‐Renal Tumor Study Group

Abstract

In children, renal cell carcinoma (RCC) is rare. This study is the first report of pediatric patients with RCC registered by the International Society of Pediatric Oncology‐Renal Tumor Study Group (SIOP‐RTSG). Pediatric patients with histologically confirmed RCC, registered in SIOP 93‐01, 2001 and UK‐IMPORT databases, were included. Event‐free survival (EFS) and overall survival (OS) were analyzed using the Kaplan‐Meier method. Between 1993 and 2019, 122 pediatric patients with RCC were registered. Available detailed data (n = 111) revealed 56 localized, 30 regionally advanced, 25 metastatic and no bilateral cases. Histological classification according to World Health Organization 2004, including immunohistochemical and molecular testing for transcription factor E3 (TFE3) and/or EB (TFEB) translocation, was available for 65/122 patients. In this group, the most common histological subtypes were translocation type RCC (MiT‐RCC) (36/64, 56.3%), papillary type (19/64, 29.7%) and clear cell type (4/64, 6.3%). One histological subtype was not reported. In the remaining 57 patients, translocation testing could not be performed, or TFE‐cytogenetics and/or immunohistochemistry results were missing. In this group, the most common RCC histological subtypes were papillary type (21/47, 44.7%) and clear cell type (11/47, 23.4%). Ten histological subtypes were not reported. Estimated 5‐year (5y) EFS and 5y OS of the total group was 70.5% (95% CI = 61.7%‐80.6%) and 84.5% (95% CI = 77.5%‐92.2%), respectively. Estimated 5y OS for localized, regionally advanced, and metastatic disease was 96.8%, 92.3%, and 45.6%, respectively. In conclusion, the registered pediatric patients with RCC showed a reasonable outcome. Survival was substantially lower for patients with metastatic disease. This descriptive study stresses the importance of full, prospective registration including TFE‐testing.

Pediatric renal tumor epidemiology: Global perspectives, progress, and challenges

“…The SIOP‐RTSG reported RCC incidence from the SIOP 93‐01, 2001, and UK IMPORT database showing 46% localized, 25% regionally advanced, and 20% metastatic disease 49 . While patients with localized disease typically achieve cure with surgery alone, those with metastatic disease have poor outcomes.…”

Renal cell carcinoma in children and adolescents: a retrospective study of a French–Italian series of 93 cases

“…The 42 Italian cases were registered in the Italian CNR92 and AIEOP TW2003 protocols, or in the Paediatric Renal Tumour Registry of the AIEOP TW2003 protocol. Ten French cases out of 51 were included in the SIOP‐RTSG 93–01 and 2001 protocols 20 . The clinicopathological variables registered were age at diagnosis, gender, type of surgery, tumour size, multifocality, architecture, cytology, WHO/International Society of Urological Pathology (ISUP) grade, presence of inflammation, necrosis, calcifications, bone metaplasia, capsular invasion and vascular invasion.…”

“…In a few large paediatric series, 15–22 non‐ MiTF ::TRCCs are represented mainly by PRCC types 1 and 2 and clear cell RCC (CCRCC). Recently, some new entities have been described, such as the eosinophilic solid and cystic RCC (ESCRCC), 23 which has also been reported in three children 24 …”

Bilateral Renal Tumors in Children: The First 5 Years’ Experience of National Centralization in The Netherlands and a Narrative Review of the Literature

“…We retrospectively assessed response to chemotherapy based on tumor volume measured on MRI-DWI, using the following formula to calculate the tumor volume: 0.523 times the product of the three tumor dimensions [ 3 ]. Response was defined as a decrease of tumor volume of more than 10%, as previously described [ 17 ].…”