Home   Chapter Home   Jobs   Conferences   Fellowships   Books


Kidney tumor - adult malignancies

Adult renal cell carcinoma

Tubulocystic carcinoma

Reviewers: Sean Williamson, M.D. (see Reviewers page)
Revised: 30 June 2012, last major update June 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.


● Mixture of tubules and micro/macro cysts with low-grade nuclear features
● Also called low-grade collecting duct carcinoma, Bellini duct carcinoma
● Not part of WHO 2004 classification
Origin: appears to derive from proximal convoluted tubule and distal nephron


● Rare; mean age 54 years, 85% male (Am J Surg Pathol 2009;33:384)

Clinical description

● Often an incidental finding, usually low stage
● Prognosis is usually excellent with recurrences but only rare distant metastases or death from disease

Case reports

● 56 year old man (Case of the Week #51)

Gross description

● Up to 17 cm, usually well circumscribed, gray-white and cystic (“bubble wrap” appearance), with a medullary location
● Often surrounded by rim of compressed fibrous tissue
● Minimal hemorrhage, necrosis or invasion of adjacent renal parenchyma

Gross images

Image 1

Micro description

● Mixture of closely packed tubules and micro/macro cysts of variable sizes with low grade nuclear features (Urology 1997;50:679)
● Tubules and cysts are lined by single layer of cuboidal or columnar cells with abundant eosinophilic cytoplasm, uniform nuclei with distinct nucleoli; often have hobnail appearance
● Overall low grade nuclear features
● Cysts are closely spaced with variable intervening fibrotic stroma
● 40% coexist with papillary renal cell carcinoma
● Minimal mitotic activity, no atypia, no desmoplasia

Micro images

Low power:

Various images





Positive stains

● Mucin, keratins (AE1-3, Cam 5.2 [CK8/18] and CK19, Am J Surg Pathol 2005;29:747)
● Vimentin, EMA, PAX2; variable 34betaE12, variable CK7

Molecular description

● Distinct molecular signature from other carcinomas
● Often gain of chromosome 7 and 17, loss of Y, suggesting a relationship to papillary RCC (Am J Surg Pathol 2009;33:1840, Am J Surg Pathol 2008;32:177)

Electron microscopy description

● Short microvilli with brush border organization

Differential diagnosis

Multilocular cystic renal cell carcinoma: aggregates of clear cells with atypia within the septae dividing cystic spaces
Multilocular renal cyst/cystic nephroma: children under 2 years old and women 40-69 years, architecture is predominantly cystic, not tubulocystic; multilocular cysts are lined by flattened or attenuated epithelium with indistinct nucleoli; occasionally hobnail morphology; hyalinized, fibrotic or ovarian-like stroma
Mucinous tubular and spindle cell neoplasm: predominantly females, typically long tubular profiles or cordlike growth pattern of uniform, low cuboidal cells with eosinophilic, focally vacuolated cytoplasm and spindling; stroma is myxoid and bubbly with abundant extracellular mucin

End of Kidney tumor - adult malignancies > Adult renal cell carcinoma > Tubulocystic carcinoma

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must be interpreted in the context of a patient's clinical data using reasonable medical judgment. This website should not be used as a substitute for the advice of a licensed physician.

All information on this website is protected by copyright of PathologyOutlines.com, Inc. Information from third parties may also be protected by copyright. Please contact us at [email protected] with any questions (click here for other contact information).