Kidney tumor
Childhood neoplasms
Rhabdoid tumor

Topic Completed: 1 January 2012

Revised: 12 February 2019

Copyright: 2003-2019,, Inc.

PubMed Search: Rhabdoid tumor [title] kidney

Mandolin S. Ziadie, M.D.
Page views in 2018: 2,366
Page views in 2019 to date: 1,834
Cite this page: Ziadie MS. Rhabdoid tumor. website. Accessed August 19th, 2019.
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


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


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


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

Images hosted on other servers:

38 year old woman - various images

10 year old girl
Fig 1: CT shows large calcified mass in pelvic brim
fig 2 / 3: nodules of large, cells with well defined cell borders,
abundant eosinophilic cytoplasm pushing nuclei to periphery,
karyotype shows +X, +2 +7 plus other changes

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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