Diagnosis and Treatment of Renal Cell Carcinoma in Children: A Report from the Polish Pediatric Rare Tumor Study Group

Abstract

Background: Renal cell carcinoma (RCC) in children is rare, accounting for approximately 1.9–6% of all pediatric renal malignancies. The aim of this study was to transmit our experience in the treatment of RCC in Polish children.

Methods: Clinical data from 21 children (6.3–18 years old) with RCC treated between 1992 and 2009 at Polish pediatric oncological centers were analyzed.

Results: In 2 patients, RCC developed as a second malignancy after neuroblastoma or astrocytoma fibrillare, respectively. In 6 patients, initial diagnoses based on imaging studies were unilateral Wilms’ tumor, leading to preoperative chemotherapy. The remaining patients underwent surgery at the beginning of treatment. According to the AJCC/TNM staging system, 14 patients had stage I, 5-II, 1-III, and 1-IV. Nephrectomy was performed in 19 patients, heminephrectomy in one, and biopsy in another. Histopathological diagnoses were clear-cell RCC (18 patients), papillary RCC (2 patients), and chromophobe RCC (1 patient). 10 patients were treated with chemotherapy, with or without IL-2, INFα, and antiangiogenic agents. 2 patients died due to disease progression.

Conclusions: RCC in children is mostly operable at diagnosis, resulting in good prognosis. The role of adjuvant chemo- and immunotherapies is unclear. Neoadjuvant chemotherapy proven for children with Wilms' tumors is ineffective, but the delay in adequate therapy did not worsen the outcome if complete nephrectomy is done subsequently.

Zusammenfassung

Hintergrund: Das Nierenzellkarzinom (NCC) bei Kindern ist selten und beträgt 1,9–6% aller Nierentumoren des Kindes- und Jugendalters; hier werden die Erfahrungen der polnischen Arbeitsgruppe für seltene Tumoren bei der Diagnose und Behandlung mitgeteilt.

Methodik: Retrospektiv werden die Daten von 21 Kindern im Alter von 6,3–18 Jahren und Jugendlichen mit NCC analysiert, die zwischen 1992 und 2009 an polnischen kinderonkologischen Zentren behandelt wurden.

Ergebnisse: 2 Patienten entwickelten das NCC als Zweittumor nach Neuroblastom bzw. fibrillärem Astrozytom. Bei 6 Patienten wurde radiologisch zuerst ein Wilms-Tumor diagnostiziert und präoperativ chemotherapiert; die anderen 15 Patienten wurden primär operiert. Die Stadien nach dem AJCC/TNM-System waren: Stadium I n=14, Stadium II n=5, Stadium III n=1, Stadium IVn=1. Bei 19 Patienten erfolgte eine Nephrektomie, je ein Patient wurde heminephrektomiert bzw. biopsiert. Die histopathologischen Diagnosen waren: klarzelliges NCC n=18, papillares NNC n=2 und chromophobes NCC n=1. 10 Patienten wurden chemotherapeutisch behandelt, zum Teil in Kombination mit IL-2, IFNα und antiangiogenetischer Therapie. 2 Patienten sind an Tumorprogression verstorben.

Schlussfolgerung: Bei den meisten Kindern ist ein NCC operabel, die Prognose nach kompletter Resektion ist gut. Die Rolle einer adjuvanten Therapie ist unklar. Die präoperative Chemotherapie für Wilms-Tumoren zeigte keinen therapeutischen Effekt.

Key words

renal cell carcinoma - treatment - histopathological classification - children

Schlüsselwörter

Nierenzellkarzinom - Behandlung - histopathologische Klassifikation - Kinder

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