Pediatric Renal Cell Carcinoma

Kiersten M. Craig; Dix P. Poppas; Ardavan Akhavan

Disclosures

Curr Opin Urol. 2019;29(5):500-504. 

In This Article

Abstract and Introduction

Abstract

Purpose of review: To review the presentation, natural history and treatment of renal cell carcinoma in children and young adults with renal cell carcinoma (RCC).

Recent findings: Complete resection of lymph nodes at the time of tumor resection can improve clinical outcomes and limit the need for adjuvant chemotherapy. Genetic alterations that lead to translocation tumors are a therapeutic target of receptor tyrosine kinase inhibitors.

Summary: The incidence of RCC increases with age. Unlike adult patients, young patients with RCC present symptomatically and at higher stage and grade. Translocation tumors predominate RCC in children with biologic activity characterized by early spread to lymph nodes with small primary tumors. Preoperative imaging is poorly sensitive for positive lymph nodes; as such, surgeons should have a low threshold for lymph node sampling during tumor resection. Despite the advanced stage at presentation, the prognosis in children is more favorable than their adult counterparts. Complete resection of lymph nodes at the time of surgical resection improves patient prognosis. Chemotherapy targeting the PI3/AKT pathway has demonstrated clinical benefit.

Introduction

Renal cell carcinoma (RCC) is a rare malignancy occurring in approximately 2–12% of the pediatric population.[1–4] Most studies in the pediatric literature are retrospective in nature. Recent prospective clinical trials organized by the Children Oncology Group (COG) have advanced our knowledge of pediatric renal tumors, highlighting the differences in epidemiology, diagnostic work-up, surgical management, pathology, and prognosis compared with adult patients. We review the clinical features of renal cell carcinoma in young patients highlighting unique aspects that practitioners should consider when treating this patient population.

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