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

Author: Daniel Anderson, M.D., MBA
Editor: Maria Tretiakova, M.D., Ph.D.

Revised: 14 December 2017, last major update December 2017

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

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

Cite this page: Anderson, D. Collecting duct carcinoma. PathologyOutlines.com website. http://pathologyoutlines.com/topic/kidneytumormalignantcollectingduct.html. Accessed December 15th, 2017.
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
Terminology
ICD-10 coding
  • C64: malignant neoplasm of kidney, except renal pelvis
Epidemiology
Sites
  • Arising from renal medulla but often involving both the cortex and medulla because of large size
Clinical features
  • 2/3 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
Treatment
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

Images hosted on PathOut server:

Images contributed by Daniel Anderson, M.D., MBA

Involving kidney and pelvis

Partially necrotic



Images from AFIP

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

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

Images hosted on PathOut server:

Images contributed by Daniel Anderson, M.D., MBA

4x

10x

20x

40x



Images from AFIP

Tumor composed
of dilated tubules
and papillary
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

Minimal morphologic
features of malignancy
and resembles primary



Images hosted on other servers:

Tumor with sarcomatoid features

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 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. CDC is known to have a wide age of presentation, male predominance and is often advanced at the time of diagnosis
    3. CDC is 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 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