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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®)

Anaplastic Sarcoma of the Kidney

General Information About Anaplastic Sarcoma of the Kidney

Anaplastic sarcoma of the kidney is a rare renal tumor that has been identified mainly in patients younger than 15 years.

Patients present with a renal mass, with the most common sites of metastases being the lung, liver, and bones. (Refer to the Wilms tumor Clinical Features and Diagnostic and Staging Evaluation sections of this summary for more information about the clinical features and diagnostic evaluation of childhood kidney tumors.)

Cytogenetic abnormalities such as rearrangement between 10q21 and 18p11.2 have been reported.[1] These tumors show pathologic features similar to pleuropulmonary blastoma of childhood (refer to the Pleuropulmonary Blastoma section in the PDQ summary on Unusual Cancers of Childhood for more information) and undifferentiated embryonal sarcoma of the liver (refer to the Treatment of Undifferentiated Embryonal Sarcoma of the Liver section in the PDQ summary on Childhood Liver Cancer for more information).

Standard Treatment Options for Anaplastic Sarcoma of the Kidney

There is no standard treatment for anaplastic sarcoma of the kidney. In the past, these tumors have been identified as anaplastic Wilms tumor and treated accordingly.[2]


  1. Gomi K, Hamanoue S, Tanaka M, et al.: Anaplastic sarcoma of the kidney with chromosomal abnormality: first report on cytogenetic findings. Hum Pathol 41 (10): 1495-9, 2010. [PUBMED Abstract]
  2. Vujanić GM, Kelsey A, Perlman EJ, et al.: Anaplastic sarcoma of the kidney: a clinicopathologic study of 20 cases of a new entity with polyphenotypic features. Am J Surg Pathol 31 (10): 1459-68, 2007. [PUBMED Abstract]
  • Updated: August 15, 2014