Kidney tumor - cysts, children, adult benign
Childhood neoplasms
Rhabdoid tumor

Author: Mandolin Ziadie, M.D. (see Authors page)

Revised: 17 January 2017, last major update January 2012

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

PubMed Search: Rhabdoid tumor [title] kidney

Cite this page: Rhabdoid tumor. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/kidneytumorrhabdoid.html. Accessed October 21st, 2017.
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

Images hosted on PathOut server:

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

Images hosted on PathOut server:

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

Images hosted on PathOut server:

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


Rhabdoid and medullary tumors, H&E and INI1

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