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Tubulocystic Carcinoma of the Kidney

Definition

  • Cytologically high grade renal neoplasm composed of densely packed cysts and tubules

Alternate/Historical Names

Diagnostic Criteria

  • Closely packed variably sized tubules and cysts
    • 0.05-2 mm diameter
    • Separated by bland fibrous stroma
      • No desmoplasia or ovarian stromal features
  • Lined by cuboidal to flattened and hobnail cells
    • Abundant eosinophilic or amphophilic cytoplasm
      • Cells resemble those of oncocytoma but show uniform prominent nucleoli
    • Focal clear cell or papillary features are seen in rare cases
    • No foamy macrophages or psammoma bodies
  • High grade nuclear features
    • Large hyperchromatic nuclei
    • Prominent round nucleoli
    • Mitotic figures and necrosis rare
  • Sharply circumscribed and spongy gross appearance

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

Low MW keratin pos
CK7 weak, focal pos
AMACR/racemase/P504S >75%
CD10 variable
PAX2 40%
PAX8 pos
High MW keratin

18%

Differential Diagnosis

Tubulocystic Carcinoma of the Kidney Collecting Duct Carcinoma
Lacks invasion and desmoplastic stroma Prominent invasion and desmoplastic stroma
Prominently dilated, cystic spaces Predominantly tubules
Lacks necrosis and mitotic figures Frequent necrosis and mitotic figures
Infrequent metastases Frequent metastases at presentation

Tubulocystic Carcinoma of the Kidney Multilocular Cystic Renal Cell Carcinoma
Lining cells have abundant eosinophilic cytoplasm Clear cells line cysts
No nests of clear cells Nests of clear cells in stroma
High grade nuclear features Low grade nuclear features

Tubulocystic Carcinoma of the Kidney Mixed Epithelial and Stromal Neoplasm
Overwhelmingly cystic Cysts common but stroma is prominent
Fibrotic stroma Cellular stroma
Large irregular nuclei Bland nuclei

Tubulocystic Carcinoma of the Kidney Renal Oncocytoma with Cystic Features
Overwhelmingly cystic Usually solid nests with focal cystic dilation
Fibrotic stroma Loose hypocellular stroma
Large irregular nuclei Large round nuclei
Consistent prominent nucleoli Nucleoli variably present
Racemase and CD10 positive Racemase and CD10 usually negative

Tubulocystic Carcinoma of the Kidney Cystic Nephroma
7:1 M:F Nearly all female
High grade nuclear features Low grade nuclear features
Lacks ovarian type stroma Ovarian type stroma

Grading / Staging

Grading

  • Definitionally cytologically high grade 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 60
      • Range 30-94
    • M:F = 7:1
    • Behavior uncertain
      • 2 of 31 cases reported with metastases

    Classification / Lists

    Renal epithelial neoplasms

    Bibliography

    • 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
    • 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.
    • Moch H. Cystic renal tumors: new entities and novel concepts. Adv Anat Pathol. 2010 May;17(3):209-14.
    • Zhou M, Yang XJ, Lopez JI, Shah RB, Hes O, Shen SS, Li R, Yang Y, Lin F, Elson P, Sercia L, Magi-Galluzzi C, Tubbs R. Renal tubulocystic carcinoma is closely related to papillary renal cell carcinoma: implications for pathologic classification. Am J Surg Pathol. 2009 Dec;33(12):1840-9.
    • Amin MB, MacLennan GT, Gupta R, Grignon D, Paraf F, Vieillefond A, Paner GP, Stovsky M, Young AN, Srigley JR, Cheville JC. Tubulocystic carcinoma of the kidney: clinicopathologic analysis of 31 cases of a distinctive rare subtype of renal cell carcinoma. Am J Surg Pathol. 2009 Mar;33(3):384-92.
    • Yang XJ, Zhou M, Hes O, Shen S, Li R, Lopez J, Shah RB, Yang Y, Chuang ST, Lin F, Tretiakova MM, Kort EJ, Teh BT. Tubulocystic carcinoma of the kidney: clinicopathologic and molecular characterization. Am J Surg Pathol. 2008 Feb;32(2):177-87.
    • MacLennan GT, Farrow GM, Bostwick DG. Low-grade collecting duct carcinoma of the kidney: report of 13 cases of low-grade mucinous tubulocystic renal carcinoma of possible collecting duct origin. Urology. 1997 Nov;50(5):679-84.
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