Kidney tumor

Adult renal cell carcinoma - rare

Collecting duct carcinoma

Topic Completed: 1 December 2017

Minor changes: 17 January 2022

Copyright: 2002-2022,, Inc.

PubMed Search: (Collecting duct carcinoma [title]) OR (Bellini duct carcinoma [title]) "loattrfree full text"[sb]

Daniel Anderson, M.D., M.B.A.
Maria Tretiakova, M.D., Ph.D.
Page views in 2021: 13,586
Page views in 2022 to date: 879
Cite this page: Anderson D, Tretiakova M. Collecting duct carcinoma. website. Accessed January 21st, 2022.
Definition / general
  • Rare (< 1% of kidney cancers) aggressive carcinoma of renal medulla arising from the principal cells of the distal collecting ducts of Bellini
  • Morphology: irregular, infiltrating tubules with high grade cells often containing mucin; marked stromal desmoplasia and inflammatory infiltrate
Essential features
  • Major diagnostic criteria
    • Medullary involvement
    • Predominantly tubular architecture
    • Marked desmoplasia
    • Cytologically high grade cuboidal or hobnail cells
    • Infiltrative growth pattern
    • No other renal cell or urothelial carcinoma
  • Minor diagnostic criteria
    • Central location (large tumors)
    • Tubulopapillary architecture
    • Inflammatory stroma with neutrophils
    • Extensive renal, extrarenal and vascular infiltration
    • Mucin positive
ICD coding
  • ICD-10: C64 - malignant neoplasm of kidney, except renal pelvis
  • Arising from renal medulla but often involving both the cortex and medulla because of large size
Clinical features
  • Approximately 67% of patients present with pain, gross hematuria, weight loss and flank mass
  • Has the poorest prognosis of common subtypes
  • Median survival: 7.6 - 11 months with most patients dying within the first 2 years (Can Urol Assoc J 2015;9:E589, Curr Oncol 2013;20:e223)
  • Distant metastases in most patients either at presentation or after nephrectomy; metastases are usually to lymph nodes, liver, lungs, bones or contralateral kidney (J Clin Oncol 2002;20:2376)
Radiology description
  • CT imaging shows good correlation with histopathologic findings
  • Mass in the renal medulla and involving renal sinus; 50% with cystic component
  • Lymphadenopathy and metastases noted in 56% and 33% of cases, respectively
  • Perinephric stranding and vascular invasion present in 56% and 28% cases, respectively
  • Infiltrative growth (67%) and preservation of the renal contour (61%) more common than expansile growth (33%) and exophytic configuration (39%) (Eur J Radiol 2006;57:453)
Case reports
Gross description
  • Infiltrative, firm gray or white mass
  • Centered in the medulla but often involving both cortex and medulla
  • Tumor size range from 2.5 - 12 cm (mean 5 cm)
  • Hemorrhage, necrosis and cystic changes are common
  • May have satellite nodules and renal vein invasion
  • Often infiltrates perirenal and renal sinus fat
Gross images

Contributed by Daniel Anderson, M.D., M.B.A.

Involving kidney and pelvis

Partially necrotic

Images hosted on other servers:
Infiltrative gray white tumor

Infiltrative gray white tumor

Tumor invades renal capsule and infiltrates adipose tissue

Microscopic (histologic) description
  • Complex, infiltrative, poorly circumscribed tumor
  • Composed of cords, tubules, tubulopapillary or tubulocystic structures embedded into inflamed desmoplastic stroma
  • Irregular channels are lined by high grade cuboidal to hobnail cells with eosinophilic cytoplasm
  • Nuclei are large, pleomorphic with prominent nucleoli and coarse chromatin
  • Numerous mitotic figures
  • Intracytoplasmic and intraluminal mucin may be present
  • May have microcystic changes from dilation of the tubular structures
  • May have sarcomatoid dedifferentiation
  • Adjacent to the tumor, tubular epithelium lining collecting ducts may appear dysplastic
Microscopic (histologic) images

Contributed by Daniel Anderson, M.D., M.B.A.





Images hosted on other servers:

Multinodular growth pattern with neutrophils

p53, p27 and BCL2

Cytology description
  • Aspirate may contain cohesive nests of tumor cells with glandular features or individual cells
  • Eosinophilic, vacuolated cells with intracytoplasmic mucin; nuclei are large irregular hyperchromatic with vesicular chromatin and large nucleoli
  • 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)
Negative stains
Electron microscopy description
  • Features of adenocarcinoma, including intracellular and extracellular lumina
  • Well formed cell junctions, prominent basal lamina and short apical microvilli
Molecular / cytogenetics description
  • NOT associated with loss of 3p
  • NOT associated with trisomies 7 and 17
  • HER2/neu amplifications have been reported in 45% of cases (J Urol 1997;158:245)
  • Genomic alterations in NF2 (29%), SETD2 (24%), SMARCB1 (18%) and CDKN2A (12%) (Eur Urol 2016;70:516)
  • Whole exome sequencing and transcriptome sequencing (RNASeq) showed recurrent somatic single nucleotide variants (SNV) in MLL in 2 samples; somatically mutated SNVs identified in ATM, CREBBP, PRDM1, CBFB, FBXW7, IKZF1, KDR, KRAS, NACA, NF2, NUP98, SS18, TP53 and ZNF521
  • SNP array identified a CDKN2A homozygous deletion and SNV analysis showed a nonsense mutation of the CDKN2A gene with unknown somatic status
  • SLC7A11 (cystine transporter, xCT), a cisplatin resistance associated gene, was found to be overexpressed in 80% of cases (Oncotarget 2016;7:29901)
Differential diagnosis
Board review style question #1
Collecting duct carcinoma (CDC) has all the following features except:

  1. As opposed to urothelial carcinoma with glandular differentiation, CDC is often PAX8+ / p63- / GATA3-
  2. Known to have a wide age of presentation, male predominance and is often advanced at the time of diagnosis
  3. Thought to derive from the principal cells of the distal collecting ducts of Bellini and often centered in the cortex
  4. Prominent stromal desmoplasia and infiltrative growth pattern are typical for CDC
Board review style answer #1
C. All the preceding were true except collecting duct carcinoma is centered in the renal medulla not the cortex as it is derived from the collecting duct epithelium

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Reference: Collecting duct carcinoma
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