Kidney tumor - adult malignancies
Adult renal cell carcinoma
Urothelial carcinoma of renal pelvis

Author: Nicole K. Andeen, M.D. (see Authors page)
Editor: Maria Tretiakova, M.D.

Revised: 30 December 2015, last major update October 2015

Copyright: (c) 2003-2015, PathologyOutlines.com, Inc.

PubMed Search: Urothelial carcinoma [title] renal pelvis

Variants: High grade, Low grade, Lymphoepithelioma like, Microcystic, Micropapillary, Nested variant, Plasmacytoid, Sarcomatoid, With glandular differentiation

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 (see Kidney International 2006; 69: 644)
  • Present with hematuria, flank pain
Radiology Images
  • Papillary tumors may be seen as soft tissue density filling defects in renal pelvis; large ones may lead to hydronephrosis
  • Infiltrative urothelial carcinomas preserve normal renal outline, unlike RCCs (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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Friable, exophytic, papillary mass, renal pelvis

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Multiple masses

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Large invasive tumor

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Papillary tumor, renal pelvis

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Tumor of the renal pelvis infiltrates renal pelvic fat and kidney

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Necrotic and infiltrative tumor

Micro 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 lamina propria, 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
Micro Images
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High grade papillary urothelial carcinoma, disordered architecture and cytologic pleomorphism

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Invasion into lamina propria characterized by jagged, irregular nests and a desmoplastic response

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Invasion into muscularis propria

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Adipose tissue represented artifact/carryover and not invasion

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Pap urothelial carc, muscularis propria is absent

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Invasion of renal parenchyma (pT3)

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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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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, 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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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