Urothelial carcinoma of renal pelvis

Kidney tumor
Renal pelvis: Urothelial carcinoma and variants
Urothelial carcinoma of renal pelvis

Authors: Nicole K. Andeen, M.D., Maria Tretiakova, M.D., Ph.D.

Deputy Editor-in-Chief: Debra Zynger, M.D.

Topic Completed: 1 October 2015

Revised: 13 December 2019

Copyright: 2002-2019, PathologyOutlines.com, Inc.

PubMed Search: Urothelial carcinoma [title] renal pelvis kidney

See also Lymphoepithelioma-like variant, Microcystic variant, Micropapillary variant, Nested variant, Plasmacytoid variant, Sarcomatoid variant, With glandular differentiation

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Table of Contents

Cite this page: Andeen NK, Tretiakova M. Urothelial carcinoma of renal pelvis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneytumormalignanturothelialcarcinoma.html. Accessed March 31st, 2020.

Definition / general

Urothelial neoplasm in the renal pelvis
85% are papillary; 65% of these are high grade
Vast majority (93%) of low grade papillary neoplasms are noninvasive (Mod Pathol 2005;18:11, Mod Pathol 2006;19:494)

Terminology

Formerly called transitional cell carcinoma

Epidemiology

7% of primary renal carcinomas (eMedicine - Urothelial Tumors)

Sites

Up to 50% have lower tract disease (Am J Surg Pathol 2004;28:1545)

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

Images hosted on other servers:

Retrograde pyelogram

Pyeloureteral transitional cell

Prognostic factors

Poor prognostic features: TNM stage (Am J Surg Pathol 2004;28:1545), vascular invasion (Mod Pathol 2006;19:272), older patient age, high tumor grade, nodal metastases, sessile architecture (Cancer 2009;115:1224), extensive tumor necrosis (defined as < 10% of tumor area, Eur Urol 2010;57:575)
High Ki67 may predict development of bladder tumors (Urol Int 2008;81:306)
Prognostic importance of high vs. low grade has not been well established
- Grade may be a factor in papillary pT1 neoplasms, but most pT2 and higher stage are nonpapillary and higher grade (Adv Anat Pathol 2008;15:127, Mod Pathol 2005;18:11)

Case reports

53 year old woman with IgG4 related kidney disease from the renal pelvis that mimicked urothelial carcinoma (BMC Urol 2015;15:44)
56 year old man with testicular metastasis (Case Rep Urol 2014;2014:759858)
60 year old man with urothelial carcinoma of the renal pelvis with choriocarcinomatous features (Hum Pathol 2002;33:1234)
64 year old woman with urothelial carcinoma of the upper urothelial tract (Arch Pathol Lab Med 2003;127:E60)
75 year old man with high grade urothelial carcinoma, plasmacytoid variant (Pol J Pathol 2014;65:237)
79 year old man presented with shortness of breath (Case of the Week #464)
80 year old woman with nested variant of urothelial carcinoma of renal pelvis (Pol J Pathol 2014;65:74)

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

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

Friable, exophytic,
papillary mass,
renal pelvis

Images hosted on other servers:

Multiple masses

Large invasive tumor

Tumor of the renal pelvis infiltrates renal pelvic fat and kidney

Papillary tumor, renal pelvis

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

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

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)

Images hosted on other servers:

Clear cell change

Vascular invasion

Perineural invasion

Forming glands with mucus

Alcian blue stains mucus

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

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

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

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

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

Positive stains

p63, GATA3, thrombomodulin, 34βE12, CK7, CK20; may express uroplakin II/III, CK5 / 6, CAIX and PAX8 (Am J Surg Pathol 2014;38:e35, Adv Anat Pathol 2008;15:127)

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

Contributed by Dr. Nikole K. Andeen and Dr. Maria Tretiakova:

FISH, loss of 9p, normal disomy chromosomes 3, 7, 17

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

Differential diagnosis

Any high grade neoplasm in renal pelvis with unusual morphologic features (Mod Pathol 2006;19:494)
Clear cell renal cell carcinoma: p63 negative, CK7 negative (Histopathology 2012;60:597) and GATA3 negative (Hum Pathol 2014;45:244, Am J Surg Pathol 2014;38:13)
Collecting duct carcinoma (CDC): both may have desmoplastic stroma, glandular differentiation. Immunohistochemistry may be useful, but has overlap: PAX8 is expressed in almost all CDCs, and 17 - 20% of upper tract urothelial CAs. Conversely, p63 is expressed in nearly all urothelial CAs, and 14% of CDCs (Am J Surg Pathol 2010;34:965, Am J Surg Pathol 2013;37:1469)

Flat urothelial carcinoma in situ vs. reactive atypia (Adv Anat Pathol 2002;9:222):

	CK20	CD44	p53
Normal urothelium	Limited to umbrella cells	Limited to basal and parabasal layers	Absent
Reactive urothelium	Limited to umbrella cells	May be full thickness	Absent
Urothelial carcinoma in situ	Full thickness staining	Absent	Present, full thickness staining

Renal medullary carcinoma: is associated with sickle cell trait, has loss of INI-1 (Am J Surg Pathol 2014;38:e35)

Additional references

Urology 2005;66:274

Board review style 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.

Reference: Kidney tumor - Urothelial carcinoma of renal pelvis

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