Clear cell, papillary and chromophobe renal cell carcinoma in patients younger than 20 years old: a clinicopathologic study with follow-up

J Pediatr Urol. 2012 Oct;8(5):531-4. doi: 10.1016/j.jpurol.2011.09.010. Epub 2011 Oct 20.

Abstract

Objective: To perform a clinicopathologic study with follow-up on this rare subset of tumors in the pediatric population.

Patients and methods: A search was made through the surgical pathology files of two academic institutions for cases of non-translocation associated renal cell carcinoma in patients younger than 20 years old from 1995 to 2011.

Results: 12 cases were identified from 350 pediatric patients with renal tumors (prevalence = 3.4%). The mean age at diagnosis was 11 years (range 1-18 years). The average tumor size was 7.3 cm (range 3.0-15.0 cm). Pathological staging analysis revealed 7/12 (58%) pT1 tumors, 3/12 (25%) pT2 tumors, and 2/12 (17%) pT3 tumors. Lymph node metastasis was present in 3/12 (25%) patients. The majority of tumors 9/12 (75%) were Fuhrman nuclear grade 2, while 3/12 (25%) cases were Fuhrman nuclear grade 3. Tumor necrosis was identified in 5/12 (42%) tumors, and angiolymphatic invasion was identified in 4/12 (33%) tumors; 1 patient developed lung metastasis.

Conclusions: Pathologic parameters typically associated with poor outcome in adults, including metastasis/high tumor stage, high Fuhrman nuclear grade, angiolymphatic invasion, and tumor necrosis, did not lead to death in any of the patients in our series.

Publication types

  • Comparative Study
  • Multicenter Study

MeSH terms

  • Adenocarcinoma, Clear Cell / epidemiology
  • Adenocarcinoma, Clear Cell / pathology*
  • Adenocarcinoma, Papillary / epidemiology
  • Adenocarcinoma, Papillary / pathology*
  • Adolescent
  • Carcinoma, Renal Cell / epidemiology
  • Carcinoma, Renal Cell / pathology*
  • Child
  • Child, Preschool
  • Diagnosis, Differential
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Kidney Neoplasms / epidemiology
  • Kidney Neoplasms / pathology*
  • Male
  • Neoplasm Staging*
  • Prevalence
  • Retrospective Studies
  • United States / epidemiology