Primary lung metastases in pediatric malignant non-Wilms renal tumors: data from SIOP 93-01/GPOH and SIOP 2001/GPOH

Klin Padiatr. 2012 Apr;224(3):148-52. doi: 10.1055/s-0032-1304600. Epub 2012 Apr 18.


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

MeSH terms

  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols / therapeutic use
  • Carcinoma, Renal Cell / diagnosis
  • Carcinoma, Renal Cell / drug therapy
  • Carcinoma, Renal Cell / pathology
  • Carcinoma, Renal Cell / secondary*
  • Child
  • Child, Preschool
  • Clinical Trials as Topic
  • Combined Modality Therapy
  • Disease Progression
  • Europe
  • Female
  • Humans
  • Infant
  • Kidney Neoplasms / diagnosis
  • Kidney Neoplasms / drug therapy*
  • Kidney Neoplasms / pathology
  • Lung Neoplasms / diagnosis
  • Lung Neoplasms / drug therapy
  • Lung Neoplasms / pathology
  • Lung Neoplasms / secondary*
  • Male
  • Neoadjuvant Therapy
  • Neoplasm Staging
  • Neuroectodermal Tumors, Primitive / diagnosis
  • Neuroectodermal Tumors, Primitive / drug therapy
  • Neuroectodermal Tumors, Primitive / pathology
  • Neuroectodermal Tumors, Primitive / secondary
  • Pneumonectomy
  • Prognosis
  • Rhabdoid Tumor / diagnosis
  • Rhabdoid Tumor / drug therapy
  • Rhabdoid Tumor / pathology
  • Rhabdoid Tumor / secondary*
  • Sarcoma, Clear Cell / diagnosis
  • Sarcoma, Clear Cell / drug therapy
  • Sarcoma, Clear Cell / pathology
  • Sarcoma, Clear Cell / secondary*
  • Survival Rate