Primary Lung Metastases in Pediatric Malignant Non-Wilms Renal Tumors: Data from SIOP 93-01/GPOH and SIOP 2001/GPOH

Abstract

Background:

Malignant non-Wilms renal tumors (NWRT) are a small but relevant subgroup of renal neoplasms in children. In this study we analyzed corresponding data from the trials SIOP 93-01/GPOH and SIOP 2001/GPOH of the Society of Pediatric Oncology and Hematology.

Patients and Methods:

Data of 22 patients with NWRT and primary lung metastases were retrospectively reviewed. Analyses included epidemiology, tumor characteristics, chemotherapy, local treatment, and outcome.

Results:

The following diagnoses were registered: Malignant Rhabdoid tumor of the kidney (MRTK, n=15), Renal-cell carcinoma (RCC, n=3), Clear-cell sarcoma of the kidney (CCSK, n=3), and primitive neuro ectodermal tumor (PNET, n=1). Median age of patients at diagnosis was 14 months. Overall survival was 36.36% (8/22). Of the 15 children with MRTK 3 survived, 3/3 patients with RCC, 1/3 patients with CCSK, and 1/1 patient with PNET survived. Lung metastases disappeared in 6 patients after initial chemotherapy, 6/8 patients undergoing local treatment of lung metastases (surgery, irradiation, or both) achieved complete remission. Only patients with complete clearance of lung lesions, either through neoadjuvant chemotherapy or subsequent local treatment, survived. Mean Follow up was 31 months (1–137).

Conclusion:

Survival of patients with stage IV NWRT is dismal. Complete removal of lung metastases seems mandatory for survival. An aggressive diagnostic and therapeutic approach seems justified in affected children.

Zusammenfassung

Hintergrund:

In dieser Studie analysierten wir die Therapieergebnisse von Kindern mit NWRT und primären Lungenmetastasen der multizentrischen Studien SIOP 93-01/GPOH und SIOP 2001/GPOH.

Patienten und Methodik:

Die retrospektive Auswertung untersuchte speziell Epidemiologie, Charakteristika von Tumoren und Patienten, Chemotherapie, Lokalbehandlung der Metastasen sowie Outcome der Patienten.

Ergebnisse:

Bei 22 identifizierten Patienten bestanden folgende Diagnosen: Maligner Rhabdoitumor der Niere (MRTK, n=15), Nierenzellkarzinom (RCC, n=3), Klarzellsarkom der Niere (CCSK n=3) und primitiver neuroektodermaler Tumor (PNET, n=1). Das Durchschnittsalter bei Diagnose war 40,23 Monate (2–166). Die Gesamtüberlebensrate war 36,36% (8/22). Von den 15 Kindern mit MRTK überlebten 3, 3 von 3 Kindern mit RCC, 1 von 3 mit CCSK und 1 Kind mit PNET überlebten. Bei 6 Patienten waren die Lungenmetastasen nach initialer Chemotherapie nicht mehr nachweisbar, 6 von 8 Kindern erzielten komplette Remission nach Lokalbehandlung der Metastasen (Chirurgie, Bestrahlung oder beides). Nur solche Patienten überlebten, bei denen die Metastasen letztlich beseitigt waren (entweder nach Chemotherapie oder Lokaltherapie). Das mittlere Follow-up der Studie betrug 31 Monate (1–137).

Schlussfolgerung:

Die Prognose von Kindern mit NWRT und primären Lungenmetastasen ist insgesamt schlecht. Voraussetzung für das Überleben der Patienten scheint eine komplette Entfernung der Metastasen zu sein. Ein aggressives diagnostisches und therapeutisches Vorgehen erscheint bei betroffenen Kindern gerechtfertigt.

Key words

pediatric tumors - non-Wilms renal tumors - lung metastases - local treatment

Schlüsselwörter

kindliche Tumoren - Nicht-Wilms-Nierentumoren - Lungenmetastasen - Lokaltherapie

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