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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 | Micro Description | Micro Images | Cytology Description | Cytology Images | Positive Stains | Negative Stains | Electron Microscopy Description | Molecular / Cytogenetics Description | Molecular / Cytogenetics Images | Differential Diagnosis | Additional ReferencesDefinition / 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 non-invasive (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
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 Ki-67 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)
- 75 year old man with high grade urothelial carcinoma, plasmacytoid variant (Pol J Pathol 2014;65:237)
- 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
Flickr image courtesy of Nikole 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
Papillary tumor, renal pelvis
Tumor of the renal pelvis infiltrates renal pelvic fat and kidney
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
Flickr images courtesy of Nikole K. Andeen, M.D. and Maria Tretiakova, M.D.:
Low grade papillary urothelial carcinoma: predominantly orderly at low power with mild but definitive cytologic atypia with regard to polarity, nuclear size, shape and chromatin texture
High grade papillary urothelial carcinoma, disordered architecture and cytologic pleomorphism
Invasion into lamina propria characterized by jagged, irregular nests and a desmoplastic response
Invasion into muscularis propria
Adipose tissue represented artifact/carryover and not invasion
Pap urothelial carc, muscularis propria is absent
Invasion of renal parenchyma (pT3)
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
Nature images:
Invasion into renal parenchyma
Invasion in lymphatic vessels
Clear cell change
'Rhabdoid' features
Pseudosarcomatous stroma
Invasion into collecting tubules
Uniform distribution of osteoclast-like giant cells
Giant cells concentrate
CK7
Mononuclear cells
Increased pleomorphism
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
Flickr images courtesy of Nikole 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, thombomodulin, 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
Flickr images courtesy of Nikole K. Andeen, M.D. and Maria Tretiakova, M.D.:
FISH, loss of 9p, normal disomy chromosomes 3, 7, 17
FISH, polyploidy chromosomes 3, 7, 17; 9p preserved
Differential Diagnosis
- 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)
- Renal medullary carcinoma: is associated with sickle cell trait, has loss of INI-1 (Am J Surg Pathol 2014;38:e35)
- 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)
- Any high grade neoplasm in renal pelvis with unusual morphologic features (Mod Pathol 2006;19:494)
- 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
Additional References
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