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Multilocular Cystic Renal Neoplasm of Low Malignant Potential

Definition

  • Neoplasm of the kidney composed entirely of cysts with small groups of non-expansile clear cells in the septa
    • These tumors have been named carcinomas (probably not very sensibly) because they are composed of cells indistinguishable from conventional clear cell carcinoma cells

Alternate/Historical Names

  • Multilocular renal cell carcinoma
  • Multilocular cystic renal cell carcinoma

Diagnostic Criteria

  • All of the following criteria must be met to make this diagnosis
    • Circumscribed nodule composed entirely of cysts and septa
      • Cysts are non-communicating
    • Cysts lined by single layer of low grade clear cells
      • May be largely denuded
      • No papillary growth pattern
    • Cysts separated by fibrous septa
      • May calcify or ossify
      • Lacks ovarian type cellular stroma
    • Groups of low grade clear cells are permitted within the septa
      • Must not be expansile nodules or show infiltrative growth
      • Cells indistinguishable from conventional clear cell carcinoma cells
      • Nuclear/nucleolar grade 1 or 2
      • May exhibit increased vascularity
      • May be infrequent
      • Must be distinguished from histiocytes and lymphocytes
      • Keratin or EMA stain may aid in identification
    • Features not allowed:
      • Necrosis
      • Vascular invasioin
      • Sarcomatous differentiation
    • The tumor must be extensively, or preferably entirely, sampled to make this diagnosis
      • The presence of any expansile nodules of clear cells in the septa between the cysts indicate an ordinary, fully malignant, clear cell carcinoma with cystic change

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

Original posting / last update:: 1/24/11, 11/1/16

Supplemental studies

Immunohistology

  • PAX8 and carbonic anhydrase IX (CAIX) positive
  • Keratin or EMA stain may be useful for identification of nests in septa
  • Some differences compared to usual clear cell carcinoma but they are not great enough to be useful (Williamson 2012)
    • CK7 is more frequently positive (92 vs 38%)
    • Racemase (AMACR) is less frequently positive (21 vs 67%)

Genetic Study

  • VHL mutations in 25%
  • Most show 3p deletions
    • Similar to clear cell renal cell carcinoma

Differential Diagnosis

Conventional Clear Cell Renal Cell Carcinoma Multilocular Renal Cell Neoplasm of Low Malignant Potential
Both cystic and solid areas No solid areas
Nodules of clear cells are expansile and/or invasive Nests of clear cells are not expansile or invasive
May be low or high grade Must be low grade (1 or 2)

Multilocular Renal Cell Neoplasm of Low Malignant Potential Cystic Nephroma
3:1 M:F Nearly all female
Nests of clear cells in stroma No nests of clear cells in stroma
Lacks cellular ovarian type stroma Ovarian type stroma

Tubulocystic Carcinoma of the Kidney Multilocular Renal Cell Neoplasm of Low Malignant Potential
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

Grading / Staging

Grading

  • Does not apply as these are benign neoplasms
    • However, by definition they have grade 1 or 2 nuclei
    • Grade should be assigned based on the siingle high power field showing the greatest degre of nuclear pleomorphism
    • 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

       

Staging

  • Does not apply

Clinical

  • Benign if all criteria fulfilled
  • Only seen in adults
    • One reported case in an 8 year old child
      • Described as having clear cells infiltrating renal parenchyma - this feature rules out the diagnosis of multicystic renal cell carcinoma

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
  • Moch H, Humphrey PA, Ulbright TM, Reuter VE eds. World Health Organization Classification of Tumors. Pathology and genetics of tumors of the Urinary System and Male Genital Organs, 4th edition,. IARC Press: Lyon 2016.
  • Störkel S, Eble JN, Adlakha K, Amin M, Blute ML, Bostwick DG, Darson M, Delahunt B, Iczkowski K. Classification of renal cell carcinoma: Workgroup No. 1. Union Internationale Contre le Cancer (UICC) and the American Joint Committee on Cancer (AJCC). Cancer. 1997 Sep 1;80(5):987-9.
  • Kovacs G, Akhtar M, Beckwith BJ, Bugert P, Cooper CS, Delahunt B, Eble JN, Fleming S, Ljungberg B, Medeiros LJ, Moch H, Reuter VE, Ritz E, Roos G, Schmidt D, Srigley JR, Störkel S, van den Berg E, Zbar B. The Heidelberg classification of renal cell tumours. J Pathol. 1997 Oct;183(2):131-3.
  • Moch H. Cystic renal tumors: new entities and novel concepts. Adv Anat Pathol. 2010 May;17(3):209-14.
  • Halat S, Eble JN, Grignon DJ, Lopez-Beltran A, Montironi R, Tan PH, Wang M, Zhang S, MacLennan GT, Cheng L. Multilocular cystic renal cell carcinoma is a subtype of clear cell renal cell carcinoma. Mod Pathol. 2010 Jul;23(7):931-6.
  • Gong K, Zhang N, He Z, Zhou L, Lin G, Na Y. Multilocular cystic renal cell carcinoma: an experience of clinical management for 31 cases. J Cancer Res Clin Oncol. 2008 Apr;134(4):433-7.
  • Suzigan S, López-Beltrán A, Montironi R, Drut R, Romero A, Hayashi T, Gentili AL, Fonseca PS, deTorres I, Billis A, Japp LC, Bollito E, Algaba F, Requena-Tapias MJ. Multilocular cystic renal cell carcinoma : a report of 45 cases of a kidney tumor of low malignant potential. Am J Clin Pathol. 2006 Feb;125(2):217-22.
  • Menon P, Rao KL, Kakkar N, Saxena AK, Singh M. Multilocular cystic renal cell carcinoma in a child. J Pediatr Surg. 2004 Oct;39(10):e14-6. (Note: the clear cells are described as infiltrating the renal parenchyma - this is not multilocular cystic renal cell carcinoma. (rvr))
  • Williamson SR,  Halat S,  Eble JN,  Grignon DJ,  Lopez-Beltran A, Montironi R,  Tan P-H,  Wang M,  Zhang S, MacLennan GT,;  Baldridge LA,  Cheng L. Multilocular cystic renal cell carcinoma: similarities and differences in immunoprofile compared with clear cell renal cell carcinoma. American Journal of Surgical Pathology 36(10):1425-1433, October 2012.
  • WHO 2016
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