Stanford School of Medicine

Surgical Pathology Criteria

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Neuroblastoma Associated Renal Cell Carcinoma


  • Distinctive renal carcinoma associated with history of neuroblastoma

Diagnostic Criteria

  • Carcinomas have been reported to arise in the kidneys of patients with a history of neuroblastoma
    • Most present about 10 years post diagnosis of neuroblastoma
    • Most are post-treatment but rare cases reported without radiation or chemotherapy
  • Predominantly composed of oncocytic cells
    • Abundant eosinophilic cytoplasm
    • Mild to moderate atypia
      • Irregular nuclei with prominent nucleoli
  • Papillary and solid growth patterns
    • One reported case of infiltrating cords with desmoplasia
  • Other types of conventional renal cell carcinoma may also be seen

John P Higgins MD
Robert V Rouse MD
Department of Pathology
Stanford University School of Medicine
Stanford CA 94305-5342

Original posting:: January 24, 2011

Supplemental studies


Neuroblastoma associated (oncocytoid) carcinoma
CAM5.2 keratin pos
EMA pos
Vimentin pos
CK9 & 20 variable
Ck7, S100 neg
TFE3 & TFEB neg
Sporadic classical type carcinomas express their usual phenotype

Differential Diagnosis

  • The diagnosis typically is made easily if the history of neuroblastoma is known
  • Neuroblastoma associated (oncocytoid) carcinoma may be separated from conventional types of renal cell carcinoma using standard criteria
    • It may be confused with translocation carcinoma because of the abundant tumor cell cytoplasm
      • TFE3/B stains or molecular studies may be required

Renal Oncocytoma Neuroblastoma Associated Renal Cell Carcinoma
No history of neuroblastoma History of neuroblastoma
Rare in children Most in children, some in young adults
Minimal papillary component Usually papillary
Neuroblastoma associated carcinomas are typically composed of oncocytic cells, which may pose this differential

Grading / Staging


  • Fuhrman grading recommended but significance is unclear as too few cases have been reported (see below)


  • Use TNM for renal cell carcinoma

  • WHO/ISUP grading system for clear cell and papillary renal cell carcinomas
  • Grade 1 Nucleoli are absent or inconspicuous and basophilic at 400x magnification
    Grade 2 Nucleoli are conspicuous and eosinophilic at 400x and visible but not prominent at 100x
    Grade 3 Nucleoli are conspicuous and eosinophilic at 100x
    Grade 4 Extreme nuclear pleomorphism, multinucleate giant cells, and/or rhabdoid and/or sarcomatoid differentiation
  • Sarcomatoid differentiation may be seen in many types of renal carcinomas (de Peralta-Venturina; Cheville)
    • It no longer refers to a type of carcinoma
    • It is considered an adverse prognostic factor
      • Prognosis may be worse than simple grade 4 carcinoma
    • Defined as a spindle cell component measuring at least one low power (40x) field with either
      • Adjacent carcinoma, or
      • Evidence of epithelial differentiation in the spindle cells
    • Spindle cells usually show moderate to marked atypia
      • Frequent patterns include fibrosarcoma, malignant fibrous histiocytoma, rhabdomyosarcoma
      • Occasional cases have low grade atypia in spindle component
      • No clinical significance to type of differentation or degree of atypia
    • May arise in setting of many types of carcinoma
      • Clear cell RCC (reported 5-8% incidence of sarcomatoid foci, in our experience it is less frequent)
      • Papillary RCC (2-3% incidence)
      • Chromophobe RCC (9% incidence)
      • Collecting duct carcinoma (39% incidence)


  • Too few cases to be certain of behavior
    • Few cases are reported as aggressive, but many of these patients are followed closely, resulting in early pickup of the tumors.

Classification / Lists

Renal epithelial neoplasms


  • Eble JN, Sauter G, Epstein JI, Sesterhenn IA eds. World Health Organization Classification of Tumors. Pathology and genetics of tumors of the Urinary System and Male Genital Organs. IARC Press: Lyon 2004.
  • Srigley JR, Delahunt B. Uncommon and recently described renal carcinomas. Mod Pathol. 2009 Jun;22 Suppl 2:S2-S23.
  • Medeiros LJ, Palmedo G, Krigman HR, Kovacs G, Beckwith JB. Oncocytoid renal cell carcinoma after neuroblastoma: a report of four cases of a distinct clinicopathologic entity. Am J Surg Pathol. 1999 Jul;23(7):772-80..
  • Koyle MA, Hatch DA, Furness PD 3rd, Lovell MA, Odom LF, Kurzrock EA. Long-term urological complications in survivors younger than 15 months of advanced stage abdominal neuroblastoma. J Urol. 2001 Oct;166(4):1455-8.
  • Hedgepeth RC, Zhou M, Ross J. Rapid development of metastatic Xp11 translocation renal cell carcinoma in a girl treated for neuroblastoma. J Pediatr Hematol Oncol. 2009 Aug;31(8):602-4.
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