Renal cell carcinomas in childhood,Nierenzellkarzinome im Kindesalter

BACKGROUND: Renal cell carcinoma is a rare childhood disease. However, it should be considered in the differential diagnosis in the detection of a renal mass.

OBJECTIVES: Incidence and mortality of renal cell carcinomas in childhood. Presentation of clinical symptoms, diagnostics, histology, therapy and course of the disease.

PATIENTS AND METHODS: Evaluation of our own prospective data of a small patient population of 8 patients, as well as discussion of the current literature on this tumor entity.

RESULTS: The average follow-up period was 27 months. Preoperative staging studies showed no metastases. The histologically primary predominant subtype was papillary renal cell carcinoma. Tumor stage and lymph node status were the most important prognostic factors in our study.

CONCLUSIONS: Renal cell carcinoma in childhood is a rare disease whose treatment is challenging and should be performed in an interdisciplinary team. Typical clinical symptoms, as in adults, are absent. The partial nephrectomy should be, if practicable, the surgical treatment of choice. The most important risk factors for survival are tumor stage and lymph node status. Data on adjuvant target therapy for metastatic disease in childhood are lacking. Further prospective, multicenter studies are necessary to generate more information on the biology and course of this disease and to obtain adjuvant treatment options in locally advanced disease.