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Table of Contents
Definition / general | Terminology | Epidemiology | Sites | Clinical features | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Positive stains | Negative stains | Electron microscopy description | Molecular / cytogenetics description | Molecular / cytogenetics images | Differential diagnosis | Additional references | Board review question #1 | Board review answer #1Cite this page: Andeen, N.K. Urothelial carcinoma of renal pelvis. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneytumormalignanturothelialcarcinoma.html. Accessed February 18th, 2019.
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
Images hosted on PathOut server:
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
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)
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
Images hosted on PathOut server:
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.
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 & 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
Images hosted on other servers:
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
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.
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.
