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Oncocytic Papillary Renal Cell Carcinoma

Definition

  • Carcinoma of the kidney exhibiting papillary architecture with oncocytic lining cells

Diagnostic Criteria

  • Note: it is not presently clear if oncocytic papillary renal cell carcinoma is an independent type of carcinoma or if it represents a subtype of papillary renal cell carcinoma (PRCC)
    • Limited data available on behavior
      • Clearly differs from oncocytoma as some cases metastasize
      • May be less aggressive than type 2 PRCC
    • About half of reported cases share genetic abnormalities of PRCC
    • Immunophenotype reports are variable but generally are similar to PRCC
  • Predominantly papillary architecture in most cases
    • Thin fibrovascular cores
    • Rare cases are predominantly solid
      • Recognized based on other features below
    • (Note that rare oncocytomas may have focal papillary features, see Differential Diagnosis at left)
  • Lined by a single layer of oncocytic cells
    • Cuboidal to columnar cells
    • Abundant granular eosinophilic cytoplasm
    • Nuclei generally round and uniform
      • Most are Fuhrman grade 1 or 2
    • Nuclei frequently apical
  • Occasional features
    • Clusters of foamy macrophages
    • Tumor cell necrosis
    • Psammoma bodies
  • Racemase/AMACR/P504s positive

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

Immunohistology

  • The only consistent immunohistologic finding appears to be racemase positivity
  • The remainder of the results are highly variable from report to report including
    • CK7, cKIT (CD117), EMA, CD10, RCC membrane antigen, vimentin

Genetic studies

  • About half of reported series find abnormalities consistent with papillary renal cell carcinoma
    • Trisomy 7 and 17
    • Loss of Y chromosome
    • Some reports, however, do not find these abnormalities

 

Differential Diagnosis

Renal Oncocytoma Oncocytic Papillary Renal Cell Carcinoma
Solid, with minimal, poorly developed papillary change Usually predominantly papillary
At most, rare, focal foamy macrophages May have clusters of foamy macrophages
Necrosis is rare and only microscopic foci May have tumor cell necrosis
AMACR/racemase/P504S usually negative AMACR/racemase/P504S positive
Occasional oncocytic papillary renal cell carcinomas may be largely solid; the bottom three criteria are more important in such cases

Type 2 Papillary Renal Cell Carcinoma Oncocytic Papillary Renal Cell Carcinoma
Nuclei usually pseudostratified Nuclei usually linear, frequently apical
Cytoplasm eosinophilic but not abundant and granular Abundant granular eosinophilic cytoplasm
The distinction might be significant since oncocytic papillary carcinomas may have a better prognosis, but more data is needed.

Grading / Staging

Grading

  • Use Fuhrman criteria but clinical correlation is not clear (see below)

Staging

  • Use TNM for renal cell carcinoma

  • Most commonly cited grading scheme is that of Fuhrman
    • Requires simultaneous assessment of three features: nuclear size, shape and nucleoli
      • Nuclear size may be subject to fixation variables and difficult to measure
      • No provision for cases with discrepant grade features
      • Poor interobserver agreement
  • We and others find a simplified version based primarily on nucleolar prominence to be more practical
    • This approach has been shown to have predictive value for clear cell and papillary carcinomas (Lohse; Sika-Paotonu; Delahunt)
    • Grade based on worst high powered field
    • Does not apply to chromophobe carcinoma or oncocytomas
    • Is provisionally applied to various other types and variants of RCC but has not been validated
      • Complete Fuhrman grading has not been validated on other types either
    Simplified Fuhrman Grading
    • Grade 1 Small, round, dark lymphocyte-like nuclei with without visible nucleoli
    Grade 2 Inconspicuous nucleoli, visible only at 200-400X (nuclei usually small and uniform with open, finely granular chromatin)
    Grade 3 Prominent nucleoli, easily visible at 100X (nuclei usually mildly to moderately pleomorphic)
    Grade 4 Markedly pleomorphic, bizarre nuclei, giant cells, multiple nucleoli
    • 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)

    Bibliography (for Grading)

    • Murphy WM, Grignon DJ, Perlman EJ. Tumors of the Kidney, Bladder and Related Urinary Structures, Atlas of Tumor Pathology, AFIP Fourth Series, Fascicle 1, 2004
    • Delahunt B. Advances and controversies in grading and staging of renal cell carcinoma. Mod Pathol. 2009 Jun;22 Suppl 2:S24-36.
    • Delahunt B, Sika-Paotonu D, Bethwaite PB, William Jordan T, Magi-Galluzzi C, Zhou M, Samaratunga H, Srigley JR. Grading of clear cell renal cell carcinoma should be based on nucleolar prominence. Am J Surg Pathol. 2011 Aug;35(8):1134-9.
    • Delahunt B, Bethwaite PB, Nacey JN. Outcome prediction for renal cell carcinoma: evaluation of prognostic factors for tumours divided according to histological subtype. Pathology. 2007 Oct;39(5):459-65.
    • Lohse CM, Blute ML, Zincke H, Weaver AL, Cheville JC. Comparison of standardized and nonstandardized nuclear grade of renal cell carcinoma to predict outcome among 2,042 patients. Am J Clin Pathol. 2002 Dec;118(6):877-86.
    • de Peralta-Venturina M, Moch H, Amin M, Tamboli P, Hailemariam S, Mihatsch M, Javidan J, Stricker H, Ro JY, Amin MB. Sarcomatoid differentiation in renal cell carcinoma: a study of 101 cases. Am J Surg Pathol. 2001 Mar;25(3):275-84.
    • Cheville JC, Lohse CM, Zincke H, Weaver AL, Leibovich BC, Frank I, Blute ML. Sarcomatoid renal cell carcinoma: an examination of underlying histologic subtype and an analysis of associations with patient outcome. Am J Surg Pathol. 2004 Apr;28(4):435-41.
    • Sika-Paotonu D, Bethwaite PB, McCredie MR, William Jordan T, Delahunt B. Nucleolar grade but not Fuhrman grade is applicable to papillary renal cell carcinoma. Am J Surg Pathol. 2006 Sep;30(9):1091-6.
    • Delahunt B, Sika-Paotonu D, Bethwaite PB, McCredie MR, Martignoni G, Eble JN, Jordan TW. Fuhrman grading is not appropriate for chromophobe renal cell carcinoma. Am J Surg Pathol. 2007 Jun;31(6):957-60..
    • Paner GP, Amin MB, Alvarado-Cabrero I, Young AN, Stricker HJ, Moch H, Lyles RH. A novel tumor grading scheme for chromophobe renal cell carcinoma: prognostic utility and comparison with Fuhrman nuclear grade. Am J Surg Pathol. 2010 Sep;34(9):1233-40.
    • Fuhrman SA, Lasky LC, Limas C. Prognostic significance of morphologic parameters in renal cell carcinoma. Am J Surg Pathol. 1982 Oct;6(7):655-63.

    Clinical

    • Mean age about 65
      • Only seen in adults, range 40-81
    • M:F = 9:1
    • Limited data available on behavior
      • Clearly differs from oncocytoma as some cases metastasize
      • May be less aggressive than type 2 papillary renal cell carcinoma

    Classification / Lists

    Renal epithelial neoplasms

    Bibliography

    • Srigley JR, Delahunt B. Uncommon and recently described renal carcinomas. Mod Pathol. 2009 Jun;22 Suppl 2:S2-S23.
    • Lefèvre M, Couturier J, Sibony M, Bazille C, Boyer K, Callard P, Vieillefond A, Allory Y. Adult papillary renal tumor with oncocytic cells: clinicopathologic, immunohistochemical, and cytogenetic features of 10 cases. Am J Surg Pathol. 2005 Dec;29(12):1576-81.
    • Okada A, Sasaki S, Fujiyoshi Y, Niimi K, Kurokawa S, Umemoto Y, Kohri K. A case of oncocytic papillary renal cell carcinoma. Int J Urol. 2009 Sep;16(9):765-7.
    • Park BH, Ro JY, Park WS, Jee KJ, Kim K, Gong G, Cho YM. Oncocytic papillary renal cell carcinoma with inverted nuclear pattern: distinct subtype with an indolent clinical course. Pathol Int. 2009 Mar;59(3):137-46.
    • Masuzawa N, Kishimoto M, Nishimura A, Shichiri Y, Yanagisawa A. Oncocytic renal cell carcinoma having papillotubular growth: rare morphological variant of papillary renal cell carcinoma. Pathol Int. 2008 May;58(5):300-5.
    • Mai KT, Kohler DM, Robertson SJ, Belanger EC, Marginean EC. Oncocytic papillary renal cell carcinoma with solid architecture: mimic of renal oncocytoma. Pathol Int. 2008 Mar;58(3):164-8.
    • Kunju LP, Wojno K, Wolf JS Jr, Cheng L, Shah RB. Papillary renal cell carcinoma with oncocytic cells and nonoverlapping low grade nuclei: expanding the morphologic spectrum with emphasis on clinicopathologic, immunohistochemical and molecular features. Hum Pathol. 2008 Jan;39(1):96-101.
    • Hes O, Brunelli M, Michal M, Cossu Rocca P, Hora M, Chilosi M, Mina M, Boudova L, Menestrina F, Martignoni G. Oncocytic papillary renal cell carcinoma: a clinicopathologic, immunohistochemical, ultrastructural, and interphase cytogenetic study of 12 cases. Ann Diagn Pathol. 2006 Jun;10(3):133-9.
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