Kidney tumor

Childhood tumors

Rhabdoid tumor

Topic Completed: 1 January 2012

Minor changes: 13 September 2021

Copyright: 2003-2019,, Inc.

PubMed Search: Rhabdoid tumor [title] kidney

Mandolin S. Ziadie, M.D.
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Cite this page: Ziadie MS. Rhabdoid tumor. website. Accessed October 15th, 2021.
Definition / general
  • Highly aggressive renal tumor of infancy
  • 1% - 2% of childhood renal tumors
  • 60% are < 1 year old, 30% are 1 - 3 years old, rare > 5 years
  • 15% are associated with PNET in the midline posterior fossa
Diagrams / tables

AFIP images

Age distribution

Clinical features
  • Very aggressive; 82% present with metastases; 90% die in 2 years
  • Usually stage 2 or higher; 9% are bilateral
Case reports
Gross description
  • Small, fleshy / hemorrhagic tumor with ill defined margins involves medullary region
  • Satellite lesions may be present
Gross images

AFIP images

Soft tan tumor

Images hosted on other servers:

round lobulated mass (fig 14)

Microscopic (histologic) description
  • Diffuse sheets, trabecula or alveolar pattern of large monomorphic cells with well defined cell borders
  • Aggressive invasive patern
  • Neoplastic cells have pink cytoplasm with eccentric nuclei due to intracytoplasmic inclusions of eosinophilic hyaline globules
  • Chromatin is clear / vesicular with central, large nucleoli
  • Occasionally cells are spindled; variable necrosis and mitoses
Microscopic (histologic) images

AFIP images

High grade tumor with abundant cytoplasm, perinuclear inclusions and prominent nucleoli

Rhabdoid tumor

Sheets of tumor cells surround glomerulus

Patterns (left to right): epithelioid, hyalinizing, chondroid

Positive stains
Negative stains
Electron microscopy description
  • Hyaline globules composed of tangled intermediate filaments
Molecular / cytogenetics description
Differential diagnosis
Additional references
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