Kidney tumor - cysts, children, adult benign
Childhood neoplasms
Oncocytoid renal cell carcinoma after neuroblastoma

Author: Maria Tretiakova, M.D., Ph.D. (see Authors page)

Revised: 23 February 2018, last major update February 2018

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Oncocytoid renal cell carcinoma neuroblastoma kidney

Table of Contents
Definition / general
Cite this page: Tretiakova, M. Oncocytoid renal cell carcinoma after neuroblastoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneytumoroncocytoidRCC.html. Accessed February 25th, 2018.
Definition / general
  • Renal neoplasms occurring in patients with a history of neuroblastoma (NB) do not represent a single entity but a heterogenous group of renal cell carcinomas (RCCs) (Am J Surg Pathol 2016;40:989)
  • This RCC subtype is no longer listed as an independent WHO entity due to lack of distinctive immunohistochemical and molecular markers, however it remains a provisional RCC entity in the 2016 WHO classification
  • Originally described in 1999 (Am J Surg Pathol 1999;23:772), see also Urology 2007;70:178.e13
  • Patients with NB have a well documented increased risk of RCC compared with the general population but tumors that arise in these patients demonstrate diverse morphologic features, including:
    1. Oncocytoid appearance
    2. Appearance similar to the classic morphology of Xp11 or t(6;11) translocation RCC
    3. Features of hybrid oncocytic / chromophobe tumor
    4. Papillary RCC-like histology (Arch Pathol Lab Med 2016;140:1026, Am J Surg Pathol 2016;40:989)