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Carcinoma of the Collecting Ducts of Bellini

Definition

  • High grade renal adenocarcinoma arising in medulla of the kidney, with a predominantly invasive tubular growth pattern
  • Clearly overlaps morphologically and immunophenotypically with medullary carcinoma but the latter has a distinctive clinicopathologic setting

Alternate/Historical Names

  • Bellini duct carcinoma
  • Collecting duct carcinoma

Diagnostic Criteria

  • Metastatic adenocarcinoma must be ruled out in every case
    • Collecting duct carcinoma should be considered a diagnosis of exclusion
  • Firm mass centered on the renal medulla
    • Frequently extends into renal cortex and/or pelvis
  • Infiltrating tubular or tubulopapillary pattern
    • Occasionally solid or microcystic
    • Usually single layer of lining cells with hobnail nuclei
    • Extra-renal and vascular invasion frequent
    • Cytoplasmic mucin may be present
      • Signet ring cells seen in rare cases
    • Rare sarcomatoid cases
      • Defined by presence of a distinct spindle cell component occupying at least one microscopic low-power field (×40)
  • Prominent stromal desmoplasia
    • Frequent mixed acute and chronic inflammation
  • High grade nuclear atypia
    • Equivalent to Fuhrman 3 or 4
    • Frequent mitotic figures
    • Dysplastic epithelial lining frequently seen in adjacent collecting duct
  • Cases described as low grade collecting duct carcinomas in the past are better classified as one of the following
  • Renal medullary carcinoma is considered a separate entity
    • Considered by some reports to be a variant of collecting duct carcinoma
    • Overlaps histopathologically with collecting duct carcinoma but is separated based on the following features
      • Occurrence in young patients
      • Association with sickle cell trait
      • Lack of INI1 expression (Cheng 2008) vs retention in 85% of collecting duct carcinoma (Elwood 2011)

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

Original posting/updates: 1/24/11, 12/29/12

Supplemental studies

Immunohistology

High & low MW keratin variable
PAX8 pos
cKIT variable
E-cadherin pos
EMA, CD15 variable
p63

14%

CD10 25%
AMACR (racemase) neg
PN15/gp200 neg
CK20 neg
Ulex & peanut agglutinin lectins pos

Differential Diagnosis

Metastatic adenocarcinoma must be ruled out clinically and by immunohistochemistry when possible

Papillary Renal Cell Carcinoma Collecting Duct Carcinoma
Lacks desmoplasia Prominent desmoplasia
Circumscribed Infiltrative
Frequently low grade nuclear features High grade nuclear features
CD10, AMACR/Racemase, PN15/gp200 positive CD10, AMACR/Racemase, PN15/gp200 negative

Gland-forming Urothelial Carcinoma Collecting Duct Carcinoma
Adjacent usual urothelial carcinoma or carcinoma in situ frequently present Lacks urothelial components
CK20+, p63+, PAX8 infrequent CK20-, p63 14%, PAX8+

Medullary Carcinoma of the Kidney Collecting Duct Carcinoma
Frequent solid and/or reticular areas Predominantly tubular and papillary
Mean age 20-24 Mean age 50-55
Associated with sickle cell trait/disease No association with sickle cell
INI negative INI positive in 85%
OCT3/4 69% positive OCT3/4 negative
The distinction may be largely clinical as there is considerable morphologic overlap

Mucinous Tubular and Spindle Cell Carcinoma of the Kidney Collecting Duct Carcinoma
Cytologically bland tubules Cytologically highly atypical tubules
Mucinous stroma Desmoplastic stroma
Circumscribed Aggressively invasive

Papillary Renal Cell Carcinoma Collecting Duct Carcinoma
Lacks desmoplasia Prominent desmoplasia
Circumscribed Infiltrative
Frequently low grade nuclear features High grade nuclear features
CD10, AMACR/Racemase, PN15/gp200 positive CD10, AMACR/Racemase, PN15/gp200 negative

Grading / Staging

Grading

  • Definitionally high grade (Fuhrman grade 3 or 4)
  • Cases described as low grade collecting duct carcinomas are better classified as one of the following
    • Mucinous tubular and spindle cell carcinoma
    • Tubulocystic carcinoma

Staging

  • Use TNM
  • Most present with high stage

Clinical

  • Mean age 50-55
    • Range 13-83
  • Poor prognosis
    • 50% node positive at presentation
    • 20-40% have distant metastases at presentation
    • 14% 10 year survival
      • One report that found same behavior in usual RCC when matched for grade and stage (Karakiewicz)

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.
  • Srigley JR, Eble JN. Collecting duct carcinoma of kidney. Semin Diagn Pathol. 1998 Feb;15(1):54-67.
  • Karakiewicz PI, Trinh QD, Rioux-Leclercq N, de la Taille A, Novara G, Tostain J, Cindolo L, Ficarra V, Artibani W, Schips L, Zigeuner R, Mulders PF, Lechevallier E, Coulange C, Valeri A, Descotes JL, Rambeaud JJ, Abbou CC, Lang H, Jacqmin D, Mejean A, Patard JJ. Collecting duct renal cell carcinoma: a matched analysis of 41 cases. Eur Urol. 2007 Oct;52(4):1140-5.
  • Tokuda N, Naito S, Matsuzaki O, Nagashima Y, Ozono S, Igarashi T; Japanese Society of Renal Cancer. Collecting duct (Bellini duct) renal cell carcinoma: a nationwide survey in Japan. J Urol. 2006 Jul;176(1):40-3.
  • Albadine R, Schultz L, Illei P, Ertoy D, Hicks J, Sharma R, Epstein JI, Netto GJ. PAX8 (+)/p63 (-) immunostaining pattern in renal collecting duct carcinoma (CDC): a useful immunoprofile in the differential diagnosis of CDC versus urothelial carcinoma of upper urinary tract. Am J Surg Pathol. 2010 Jul;34(7):965-9.
  • Kobayashi N, Matsuzaki O, Shirai S, Aoki I, Yao M, Nagashima Y. Collecting duct carcinoma of the kidney: an immunohistochemical evaluation of the use of antibodies for differential diagnosis. Hum Pathol. 2008 Sep;39(9):1350-9.
  • Cheng JX, Tretiakova M, Gong C, Mandal S, Krausz T, Taxy JB. Renal medullary carcinoma: rhabdoid features and the absence of INI1 expression as markers of aggressive behavior. Mod Pathol. 2008 Jun;21(6):647-52.
  • Elwood H, Chaux A, Schultz L, Illei PB, Baydar DE, Billis A, Sharma R, Argani P, Epstein JI, Netto GJ. Immunohistochemical analysis of SMARCB1/INI-1 expression in collecting duct carcinoma. Urology. 2011 Aug;78(2):474.e1-5.
  • Gupta R, Billis A, Shah RB, Moch H, Osunkoya AO, Jochum W, Hes O, Bacchi CE, de Castro MG, Hansel DE, Zhou M, Vankalakunti M, Salles PG, Cabrera RA, Gown AM, Amin MB. Carcinoma of the collecting ducts of Bellini and renal medullary carcinoma: clinicopathologic analysis of 52 cases of rare aggressive subtypes of renal cell carcinoma with a focus on their interrelationship. Am J Surg Pathol. 2012 Sep;36(9):1265-78.
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