Kidney tumor - adult malignancies
Adult renal carcinoma
Lymphoepithelioma-like carcinoma

Author: Daniel Anderson, M.D., M.B.A.
Editor: Maria Tretiakova, M.D., Ph.D.

Revised: 14 March 2018, last major update February 2018

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Lymphoepithelioma-like carcinoma kidney

Cite this page: Anderson, D. Lymphoepithelioma-like carcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneytumormalignantLEL.html. Accessed December 14th, 2018.
Definition / general
  • Resembles undifferentiated carcinoma of the nasopharynx (Int J Urol 2007;14:1093)
  • Lymphoepithelioma-like carcinoma (LELC) is considered a rare variant of urothelial carcinoma in the renal pelvis (Mod Pathol 2006;19:494)
  • Given the rarity and similarities with LELC of the bladder, some information and pictures are extracted from bladder, where appropriate
Essential features
  • Rare variant of urothelial carcinoma resembling LELC of nasopharynx
  • Composed of sheets of undifferentiated, pleomorphic cells with syncytial appearance, admixed with obscuring inflammatory infiltrate
Epidemiology
Etiology
  • Derived from urothelium, often admixed with conventional urothelial component
  • Not associated with Epstein-Barr virus (EBV) (Urology 2005;66:1109)
Clinical features
Radiology description
  • May show enhancing soft tissue mass in the renal pelvis or ureter, involvement of the renal parenchyma or hilar fat
Radiology images

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Scan showing enhancing
lesion from right renal pelvis
Prognostic factors
Case reports
Treatment
  • There are no specific guidelines regarding the management of LELC of the renal pelvis due to its rarity
  • In bladder, pure LELC is reportedly more responsive to chemotherapy
Gross images

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Tumor present in renal
pelvis with extension
into adjacent parenchyma
and hilar fat tissue
Microscopic (histologic) description
  • Pleomorphic, undifferentiated round or spindle cell carcinoma with ill defined borders
  • Arranged in syncytial sheets, nests or individual cells with abundant mixed inflammatory cells often obscuring the neoplastic cells
  • Microscopically resembles undifferentiated nasopharyngeal carcinoma
  • May be pure or mixed
    • Consider recording proportion of LELC in tumor
  • Surface urothelium may demonstrate carcinoma in situ (CIS)
  • A study of LELC of the urinary tract described cases with focal glandular and clear cell differentiation
Microscopic (histologic) images

Images hosted on PathOut server:

Images contributed by Maria Tretiakova, M.D., Ph.D.

Lymphoepithelioma-like carcinoma of the prostate



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Lymphoepithelioma-like carcinoma of the renal pelvis


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Urothelial CA - Lymphoepithelioma-like

Negative stains
Differential diagnosis
  • Xanthogranulomatous pyelonephritis and other inflammatory lesions: foamy histiocytes; cytokeratin negative
  • Lymphoma: no high grade epithelial cells; cytokeratin negative; lymphocytes are clonal, LCA positive
  • Poorly differentiated squamous cell carcinoma: a pure squamous lesion with no in situ or invasive urothelial component
  • Typical urothelial carcinoma with prominent lymphocytic reaction: lacks syncytial growth pattern
Board review question #1
    All the following about LELC of the urinary tract is true except:

  1. GATA3 is expressed in the majority of LELC
  2. LELC is not associated with Epstein-Barr Virus in contrast to nasopharyngeal carcinoma.
  3. LELC is thought to derive from the kidney parenchyma
  4. The tumor cells are cytokeratin and vimentin positive
Board review answer #1
All were true about LELC of the urinary tract except C. The epithelial cells, which are thought to derive from the urothelium, are seen in nests and syncytial sheets in a dense inflammatory background.