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

Author: Sean Williamson, M.D. (see Authors page)

Revised: 1 February 2017, last major update June 2012

Copyright: (c) 2003-2017, PathologyOutlines.com, Inc.

PubMed Search: "Collecting duct carcinoma" OR "Bellini duct carcinoma" kidney

Cite this page: Collecting duct carcinoma or Bellini duct carcinoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/kidneytumormalignantcollectingduct.html. Accessed October 17th, 2017.
Definition / general
  • 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 (Bellini's) ducts
Epidemiology
Clinical features
  • 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
Treatment
  • Poor response to chemotherapy but tyrosine kinase inhibitors such as sorafenib or sunitinib may be useful (Onkologie 2009;32:44)
Gross description
  • Infiltrative, firm, grayish white, mean 5 cm
  • Originates in medulla (as do some clear cell carcinomas)
  • May have intrarenal metastases; usually no hemorrhage
Gross images

Images hosted on PathOut server:

Multicystic tumor involves the cortex and medulla

Tumor resembles multilocular cyst



Images hosted on other servers:

Infiltrative gray white tumor

Tumor invades renal capsule and infiltrates adipose tissue

Sarcomatoid tumor
has extensive renal,
extrarenal and
vascular infiltration



Variant with signet ring features:

Tan yellow tumor with necrosis

Microscopic (histologic) description
  • 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)
Microscopic (histologic) images

Images hosted on PathOut server:

Tumor is com-
posed of dilated
tubules and papil-
lary structures

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

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



Images hosted on other servers:

Multinodular growth pattern with neutrophils

Tumor with sarcomatoid features

p53, p27 and bcl2



Variant with signet ring features:

Mildly enlarged cells, some with cytoplasmic vacuoles

Various images

High molecular weight keratin+ (34betaE12)

Cytology description
Positive stains
Most commonly used:
Other positive stains:
Negative stains
Electron microscopy description
  • Features of adenocarcinoma, including intracellular and extracellular lumina
Electron microscopy images

Images hosted on other servers:

Variant with signet ring features:

Edematous cytoplasm with sparse organelles

Molecular / cytogenetics description
  • Associated with loss of 1q, 6p, 14, 15, 22
  • NOT associated with loss of 3p
Differential diagnosis