Kidney tumor

Childhood tumors

Cystic partially differentiated nephroblastoma


Editorial Board Member: Debra L. Zynger, M.D.
Deputy Editor-in-Chief: Maria Tretiakova, M.D., Ph.D.
Daniel Athanazio, M.D., Ph.D.

Last author update: 26 May 2023
Last staff update: 16 February 2024

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PubMed Search: Cystic partially differentiated nephroblastoma

See Also: Wilms tumor of children

Daniel Athanazio, M.D., Ph.D.
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Cite this page: Athanazio D. Cystic partially differentiated nephroblastoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/kidneytumorcysticnephroblastoma.html. Accessed March 18th, 2024.
Definition / general
  • WHO: multilocular, exclusively cystic neoplasm of very young children, containing nephroblastomatous tissue
  • The concept was introduced as a variant of nephroblastoma curable by surgery (Perspect Pediatr Pathol 1979;5:217)
Essential features
  • Entirely multilocular cystic tumor that lacks solid nodules on gross or microscopic examination; septa contain (undifferentiated) mesenchyme, blastemal and immature epithelial elements
ICD coding
  • ICD-O: 8959/1 - cystic partially differentiated nephroblastoma
  • ICD-11: 2C90.Y & XH1JB4 - other specified malignant neoplasms of kidney, except renal pelvis & cystic partially differentiated nephroblastoma
Epidemiology
Clinical features
  • Palpable abdominal mass (Cancers (Basel) 2021;13:997)
  • Bilateral disease in 4.4%, 3 cases with bilateral cystic partially differentiated nephroblastoma, 2 with nephroblastomatosis in the contralateral kidney and 1 with Wilms tumor in the contralateral kidney (Cancers (Basel) 2021;13:997)
Diagnosis
  • Requires microscopic examination of resected tumor
Radiology description
  • Large multilocular cystic mass with septa of varying thickness
  • No solid component
Radiology images

Images hosted on other servers:

Ultrasonography of multilocular cystic mass

MRI of multilocular cystic mass

Abdominal CT with bilateral CPDN

Prognostic factors
Case reports
Treatment
  • Surgery is almost always curative
  • Patients with stage I disease are cured by surgery without adjuvant chemotherapy
  • Questionable value of chemotherapy in cases of tumor rupture and spillage (Cancers (Basel) 2021;13:997)
Gross description
  • Typically, a large multilocular cystic tumor (mean diameter of 10 cm)
  • Thin or variable sized septa
  • Well circumscribed
  • No expansive nodules
  • Reference: J Postgrad Med 2006;52:45
Gross images

Contributed by Americo Brilhante, M.D. and Daniel Athanazio, M.D., Ph.D.
Multilocular cyst of the kidney Multilocular cyst of the kidney in a 11 month old boy Multilocular cyst of the kidney

Multilocular cyst of the kidney

Microscopic (histologic) description
  • Cysts lined with flat / cuboidal / hobnail cells (or are denuded)
  • Epithelial elements consist mainly of mature and immature / abortive tubules and small papillae resembling immature glomeruli
  • Key histological findings of the variably cellular septa include
    • Nephroblastomatous epithelial elements
    • 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

Contributed by Americo Brilhante, M.D. and Daniel Athanazio, M.D., Ph.D.
Multilocular cystic tumor

Multilocular cystic tumor

Nephroblastomatous elements Nephroblastomatous elements Nephroblastomatous elements

Nephroblastomatous elements

Transition between tumor and renal cortex

Transition between tumor and renal cortex

Fibrous septa and epithelial lining

Fibrous septa and epithelial lining


Small tubules with hobnail morphology Small tubules with hobnail morphology

Small tubules with hobnail morphology

Abortive / immature tubules Abortive / immature tubules

Abortive / immature tubules

Abortive / immature tubules Abortive / immature tubules

Abortive / immature tubules


Primitive blastema and tubules

Primitive blastema and tubules

AE1 / AE3

AE1 / AE3

WT1

WT1

Positive stains
Molecular / cytogenetics description
  • Cystic partially differentiated nephroblastoma is considered part of the nephroblastoma spectrum
  • Does not harbor DICER1 mutations, which are found in the morphologically similar tumor pediatric cystic nephroma (Cancers (Basel) 2021;13:997)
  • No specific genetic alterations have been characterized (Cancers (Basel) 2021;13:997)
Sample pathology report
  • Left kidney, nephrectomy:
    • Cystic partially differentiated nephroblastoma (see synoptic report)
Differential diagnosis
Board review style question #1

A large multilocular cystic renal mass was removed from an 18 month old boy. The differential diagnosis is pediatric cystic nephroma and cystic partially differentiated nephroblastoma. What finding within the septa favors cystic partially differentiated nephroblastoma?

  1. Fibrous tissue
  2. Nephroblastomatous elements
  3. Presence of mature renal cortex within the septa
  4. Tubules with flat and hobnail morphology
Board review style answer #1
B. Nephroblastomatous elements. The presence of nephroblastomatous elements within septa is the major criterion for distinction between cystic partially differentiated nephroblastoma and cystic nephroma. Answer D is incorrect because flat and hobnail lining do not distinguish both lesions. Answer C is incorrect because residual cortical parenchyma may be observed between cysts in autosomal dominant polycystic kidney disease. Answer A is incorrect because the presence of fibrous tissue within septa would not distinguish the entities in this differential.

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Reference: Cystic partially differentiated nephroblastoma
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