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Kidney tumor - adult malignancies

Adult renal cell carcinoma

Collecting duct carcinoma or Bellini duct carcinoma


Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 12 December 2014, last major update June 2012
Copyright: (c) 2003-2014, PathologyOutlines.com, Inc.

General
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● Aggressive epithelial malignancy of renal medulla with irregular tubules, high-grade hobnail cells and marked desmoplasia
● < 1% of adult renal epithelial tumors, controversial - often a diagnosis of exclusion
Origin: likely from distal collecting (Belliniís) ducts

Epidemiology
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● 2/3 male, mean age 55 years, but often found in young adults; associated with analgesic nephropathy (Am J Surg Pathol 1980;4:565)

Clinical description
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● Presents with painless gross hematuria; 50% have coexisting bladder urothelial tumors
● Poorest prognosis of common subtypes, with death in months to a few years
● 35-50% have metastases at presentation to regional lymph nodes, bone, adrenal glands, lung, skin
Major criteria: involvement of medullary pyramid (small tumors), irregular tubular architecture, marked desmoplasia, high grade hobnail cells, positive for high molecular weight cytokeratin and Ulex europaeus, no urothelial carcinoma elsewhere
Minor criteria: central location (large tumors), papillary architecture with wide fibrous stalks and desmoplastic stroma, inflammatory stroma with neutrophils; extensive renal, extrarenal and vascular infiltration, mucin positive

Case reports
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● 26 year-old man with associated meningeal carcinomatosis (Arch Pathol Lab Med 1999;123:638)
● 80 year old man with tumor exhibiting sarcomatoid features (Arch Pathol Lab Med 1999;123:338)
● 2 cases with multiorgan metastases (J Med Case Reports 2008 Sep 17;2:304)

Treatment
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● Poor response to chemotherapy, but tyrosine kinase inhibitors such as sorafenib or sunitinib may be useful (Onkologie 2009;32:44)

Gross description
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● Infiltrative, firm, gray-white, mean 5 cm
● Originates in medulla (as do some clear cell carcinomas)
● May have intrarenal metastases; usually no hemorrhage

Gross images
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Multicystic tumor involves the cortex and medulla


Tumor resembles multilocular cyst


Sarcomatoid tumor has extensive renal, extrarenal and vascular infiltration

Infiltrative gray-white tumor

Tumor invades renal capsule and infiltrates adipose tissue

Micro description
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● Poorly circumscribed tubulopapillary tumor with infiltrative borders
● Irregular channels are lined by high grade hobnail cells with marked desmoplastic response, brisk neutrophilic infiltrate, mucin production
● May have microcystic change; may have atypical hyperplastic component (not urothelial CIS, Hum Pathol 1990;21:449)
● May have sarcomatoid dedifferentiation, adjacent tubules may show dysplasia (although sometimes seen in other types of RCC)

Micro images
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Tumor is composed of dilated tubules and papillary structures

Multinodular growth pattern with neutrophils, high-grade cytologic features, inset shows extensive tubal dysplasia


Dilated tubules lined by single layer of hobnail cells


Hobnail tumor cells


Atypical hyperplasia of collecting duct epithelium adjacent to the tumor


Tubules are less well formed and there is nuclear anaplasia

   
Tumor with sarcomatoid features


Intra-abdominal metastasis has minimal morphologic features of malignancy, and resembles primary


p53, p27 and bcl2

Cytology description
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● Ductal/tubular differentiation with benign, dysplastic and malignant features, prominent desmoplastic stroma, neutrophils (Acta Cytol 2004;48:843)
● Sarcomatoid features may be seen (Diagn Cytopathol 2010;38:603)

Positive stains
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Most commonly used:
● PAX8 (Am J Surg Pathol 2010;34:965)
● High molecular weight keratin, CK7, CK8/18, CK19, EMA, Ulex europaeus, lectins/peanut agglutinin, mucin (strong), vimentin

Other positive stains:
● c-kit / CD117, LeuM1, E-cadherin, lysozyme

Negative stains
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● p63 (Am J Surg Pathol 2010;34:965), glycogen, RCC-Ma

Electron microscopy description
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● Features of adenocarcinoma, including intracellular and extracellular lumina

Cytogenetics description
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● Associated with loss of 1q, 6p, 14, 15, 22
● NOT associated with loss of 3p

Differential diagnosis
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● Adenocarcinoma from urothelium of renal pelvis: usually mucinous, resembles colon, vimentin negative
● Metastatic carcinoma from GI or lung: usually well defined borders, multiple
Papillary renal cell carcinoma: not necessarily central, more circumscribed, often psammoma bodies and foamy macrophages within papillae, usually no angiolymphatic invasion, no desmoplasia or inflammation, no dysplasia of collecting duct epithelium, LeuM1+, mucin-, Ulex europaeus-, E-cadherin-, CD117-, trisomy 7 or 17 (Hum Pathol 2008;39:1350)
Renal medullary carcinoma: may be related (Semin Diagn Pathol 1998;15:54); occurs in blacks with sickle cell trait; usually yolk sac pattern
Urothelial carcinoma: p63+/PAX8- (Am J Surg Pathol 2010;34:965)


Collecting duct carcinoma variant with signet-ring features

Case reports
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● Large intracytoplasmic vacuoles with compression of nuclei, mucin and glycogen negative, due to intracellular edema (Mod Pathol 2001;14:623)

Gross images
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Tan-yellow tumor with necrosis

Micro images
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Mildly enlarged cells, some with cytoplasmic vacuoles


Various images


High molecular weight keratin+ (34betaE12)

Electron microscopy images
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Edematous cytoplasm with sparse organelles

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Collecting duct carcinoma or Bellini duct carcinoma


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