Kidney tumor
Renal pelvic tumors
Urothelial carcinoma of renal pelvis

Author: Nicole K. Andeen, M.D.
Editor: Maria Tretiakova, M.D.
Deputy Editor Review: Debra Zynger, M.D.

Revised: 25 September 2018, last major update October 2015

Copyright: (c) 2002-2018, PathologyOutlines.com, Inc.

PubMed Search: Urothelial carcinoma [title] renal pelvis kidney

See also High grade variant, Low grade variant, Lymphoepithelioma-like variant, Microcystic variant, Micropapillary variant, Nested variant, Plasmacytoid variant, Sarcomatoid variant, With glandular differentiation
Cite this page: Andeen, N.K. Urothelial carcinoma of renal pelvis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneytumormalignanturothelialcarcinoma.html. Accessed December 13th, 2018.
Definition / general
Terminology
  • Formerly called transitional cell carcinoma
Epidemiology
Sites
Clinical features
  • 64% men, mean age 67 years (Am J Surg Pathol 2004;28:1545)
  • Risk factors: tobacco use, phenacetin use, industrial carcinogen exposure (coal, asphalt, petrochemicals, tar), thorium containing radiologic contrast material, Balkan endemic nephropathy
  • Present with hematuria, flank pain
  • Outcomes for urothelial carcinomas have not changed significantly in the past 3 decades (Int J Urol 2012;19:1060)
Radiology description
  • Papillary tumors may be seen as soft tissue density filling defects in renal pelvis; large ones may lead to hydronephrosis
  • Infiltrative urothelial carcinoma preserves normal renal outline, unlike renal cell carcinoma (Radiopaedia - Transitional cell carcinoma)
Radiology images

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Retrograde pyelogram

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Pyeloureteral transitional cell

Prognostic factors
Case reports
Treatment
  • Nephroureterectomy, fulguration, chemotherapy
Gross description
  • Mean 4 cm, 28% multifocal
  • Recommended to fix specimen prior to grossing for accurate staging (Am J Surg Pathol 2004;28:1545)
  • Recommended to stage renal caliceal tumors based on the extent of invasion relative to the corticomedullary junction (Hum Pathol 2007;38:1639)
Gross images

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Contributed by Nicole K. Andeen, M.D. and Maria Tretiakova, M.D.

Friable, exophytic,
papillary mass,
renal pelvis



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Multiple masses

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Large invasive tumor

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Tumor of the renal pelvis infiltrates renal pelvic fat and kidney


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Papillary tumor, renal pelvis

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Necrotic and infiltrative tumor

Bulbous expansion of proximal ureter

Exophytic mass

Microscopic (histologic) description
  • Similar to urothelial neoplasia in bladder: nests, clusters and single neoplastic cells with or without papillary cores or desmoplastic response
  • Pitfalls in staging:
    • Due to variations in microanatomy in renal pelvis, there may be a paucity of subepithelium, and the muscularis may be absent or indiscernible from the calyceal connective tissue (Adv Anat Pathol 2008;15:127)
    • Must distinguish between renal parenchymal invasion (pT3) and spread within renal tubules or von Brunn nests (pTa / Tis) (Adv Anat Pathol 2008;15:127)
  • Grading for papillary neoplasms (same as in bladder, Am J Surg Pathol 1998;22:1435):
    • May have spectrum; report highest grade area
    • Low grade: predominantly orderly at low power with mild but definitive cytologic atypia with regard to polarity, nuclear size, shape and chromatin texture; occasional mitotic figures
    • High grade: predominantly disorderly appearance at low power due to both architectural irregularities and cytologic pleomorphism (size, shape, nuclear chromatin pattern, hyperchromasia, nucleolar prominence); frequent mitotic figures
Microscopic (histologic) images

Images hosted on PathOut server:

Contributed by Nicole K. Andeen, M.D. and Maria Tretiakova, M.D.

Noninvasive papillary urothelial carcinoma, low grade (pTa)

Noninvasive papillary urothelial carcinoma, low grade (pTa)

Papillary urothelial
carcinoma, muscularis
is absent


Subepithelial invasion characterized by jagged, irregular nests and a desmoplastic response (pT1)

Invasion into muscularis (pT2)

Invasion of renal parenchyma (pT3)

Artifact / carryover
of tumor into adipose
(not invasion)



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Clear cell change

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Vascular invasion

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Perineural invasion

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Forming glands with mucus

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Alcian blue stains mucus

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Diffuse infiltration into perirenal fat

Cytology description
  • Cellular, atypical urothelial cells present individually and in clusters with enlarged nuclei, high nuclear to cytoplasmic ratios, coarse chromatin and irregular nuclear contours
Cytology images

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Contributed by Vanya Jaitly, M.D. and Songlin Zhang, M.D.

Metastatic micropapillary carcinoma, consistent with renal pelvic primary

Diff - Quik

PAP

Cell block



CK7

CK20

CA125

GATA3

HER2




Images hosted on other servers:

Contributed by Nicole K. Andeen, M.D. and Maria Tretiakova, M.D.

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Highly atypical cells, adjacent to a sheet of small bland urothelial cells (right)

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Large nuclei with high nuclear to cytoplasmic ratios, coarse chromatin, and irregular nuclear contours (Papanicolaou & DiffQuik)

Negative stains
Electron microscopy description
  • Papillary urothelial carcinoma of urinary bladder: well differentiated neoplasm has zonula occludens (tight junctions) and well developed macula adherens (desmosomes), with fewer surface vesicles than normal epithelium
  • High grade urothelial carcinoma shows loss of specialization of luminal membrane and loss of characteristic epithelial zonula occludens and macula adherens (Cancer 1971;27:71)
Molecular / cytogenetics description
  • Aberrations of p53, chromosome 9
  • Microsatellite instability in 20 - 30%, associated with inverted growth patterns and hereditary nonpolyposis colorectal cancer syndrome (Hum Pathol 2003;34:222, Adv Anat Pathol 2008;15:127)
  • Fluorescent in situ hybridization (FISH, UroVysion) studies for chromosomes 3, 7, 17, and 9p21 (p16 tumor suppressor gene) may be useful, but require additional study in upper tract urothelial neoplasms (Adv Anat Pathol 2008;15:279)
Molecular / cytogenetics images

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Contributed by Dr. Nikole K. Andeen and Dr. Maria Tretiakova:
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FISH, loss of 9p, normal disomy chromosomes 3, 7, 17

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FISH, polyploidy chromosomes 3, 7, 17; 9p preserved

Differential diagnosis
Additional references
Board review question #1
Which of the following is true about micropapillary carcinoma of the bladder?

A. A panel of immunostains is typically necessary to determine the site of an unknown primary.
B. The micropapillary component only needs to be reported if 50% or more.
C. Treatment response is comparable to other urothelial carcinoma.
D. Tumor cells appear in small clusters with a vascular axis.
Board review answer #1
A. A panel of immunostains is typically necessary to determine the site of an unknown primary.