Kidney tumor
Childhood neoplasms
Rhabdoid tumor


Topic Completed: 1 January 2012

Revised: 12 February 2019

Copyright: 2003-2019, PathologyOutlines.com, Inc.

PubMed Search: Rhabdoid tumor [title] kidney
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Cite this page: Ziadie MS. Rhabdoid tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneytumorrhabdoid.html. Accessed March 25th, 2019.
Definition / general
  • Highly aggressive renal tumor of infancy
Epidemiology
  • 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

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
Treatment
Gross description
  • Small, fleshy / hemorrhagic tumor with ill defined margins involves medullary region
  • Satellite lesions may be present
Gross images

AFIP

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

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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