Kidney tumor
Childhood neoplasms
Cystic partially differentiated nephroblastoma

Author: Maxwell Rollins, M.D. (see Authors page)
Editor: Dr. Carla Ellis M.D., MS, FCAP

Revised: 29 June 2017, last major update June 2017

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

PubMed Search: Cystic partially differentiated nephroblastoma [title]

Related topics: Wilms tumor of children , Teratoid Wilms tumor
Cite this page: Cystic partially differentiated nephroblastoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneytumorcysticnephroblastoma.html. Accessed October 23rd, 2017.
Definition / general
Essential features
  • Entirely multilocular cystic tumor (no expansile masses or solid areas within the tumor)
Epidemiology
Radiology images

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Ultrasonography of a large multi-cystic lesion

CT showing abdominal distension

T2 weighted image of a renal mass

Case reports
Treatment
Gross description
  • Typically a large multicystic tumor (mean diameter of 10 cm), with no expansile or solid areas
Gross images

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Numerous thin walled cysts

Cystic nephroma specimen

well circumscribed multicystic lesion

multicystic tumor

Microscopic (histologic) description
  • Cysts lined with flat / cuboidal / hobnail cells (or are denuded)
  • Epithelial elements consist mainly of mature and immature tubules and small papillae resembling immature glomeruli
  • Key histological findings of the variably cellular septa include:
    • Nephroblastomatous epithelial elements (J Urol 2010;183:1585)
    • Islands of undifferentiated blastema and differentiated mesenchymal elements (skeletal muscle and less often cartilage and fat, J Urol 2010;183:1585)
    • Focally, the septal elements may protrude into the cystic spaces in microscopic papillary folds
Microscopic (histologic) images

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Embryonal elements are present



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Cysts with hobnail epithelium

Various images

H&E

WT1

Positive stains
  • WT1: blastemal cells
Molecular / cytogenetics description
  • Hyperdiploidy and trisomy 12: trisomy 12 was the only chromosomal anomaly common to all four studied cases (Hum Pathol 1996;27:980)
Differential diagnosis
  • Cystic renal dysplasia:
    • Usually diffuse and bilateral, typically obstructive symptoms are present clinically, presence of primitive ducts on histology (Arch Pathol Lab Med 2015;139:547) 

  • Pediatric cystic nephroma:
    • Exclusively cystic renal neoplasm, however the septae do not contain nephroblastomatous elements
    • Pediatric cystic nephroma does not contain ovarian type stroma and is associated with DICER1 mutations, not found in cystic partially differentiated nephroblastoma
  • Wilms Tumor:
    • Solid Wilms tumor with multifocal cystic change has predominately solid areas containing focally cystic areas, and has malignant behavior
    • In contrast, cystic partially differentiated nephroblastoma is predominantly cystic with blastemal or other embryonal cells in the septa of the cysts; it lacks nodular solid regions
    • Both are WT1+