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Anaplastic sarcoma of the kidney

MedGen UID:
1661979
•Concept ID:
C4722276
•
Neoplastic Process
Synonyms: Anaplastic Sarcoma of the Kidney; anaplastic sarcoma of the kidney
 
Monarch Initiative: MONDO:0958165

Definition

A rare tumor, usually occurring in young adults (mean age 12 years) with slight female predominance. It is characterized by a proliferation of anaplastic spindle cells with bizarre, pleomorphic nuclei and atypical mitotic figures. Most cases show chondroid differentiation. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAnaplastic sarcoma of the kidney

Recent clinical studies

Etiology

Anaplastic sarcoma of the kidney (DICER1-sarcoma of the kidney): A report from the International Pleuropulmonary Blastoma/DICER1 Registry.
Schoettler PJ, Smith CC, Nishitani M, Harris AK, Nelson AT, Watson DA, Kamihara J, Mullen EA, Hill DA, Messinger YH, Fair DB, Kumar KA, Dehner LP, Ash S, Chen KS, Schultz KAP
Pediatr Blood Cancer 2024 Aug;71(8):e31090. Epub 2024 May 28 doi: 10.1002/pbc.31090. PMID: 38807260
Metachronous anaplastic sarcoma of the kidney and thyroid follicular carcinoma as manifestations of DICER1 abnormalities.
Yoshida M, Hamanoue S, Seki M, Tanaka M, Yoshida K, Goto H, Ogawa S, Takita J, Tanaka Y
Hum Pathol 2017 Mar;61:205-209. Epub 2016 Sep 30 doi: 10.1016/j.humpath.2016.06.024. PMID: 27697588
Anaplastic sarcoma of the kidney.
Labanaris A, Zugor V, Smiszek R, Nützel R, Kühn R
ScientificWorldJournal 2009 Feb 15;9:97-101. doi: 10.1100/tsw.2009.15. PMID: 19219373Free PMC Article
Anaplastic sarcoma of the kidney: a clinicopathologic study of 20 cases of a new entity with polyphenotypic features.
Vujanić GM, Kelsey A, Perlman EJ, Sandstedt B, Beckwith JB
Am J Surg Pathol 2007 Oct;31(10):1459-68. doi: 10.1097/PAS.0b013e31804d43a4. PMID: 17895746

Diagnosis

Anaplastic Sarcoma of the Kidney With Heterologous Ganglioneuroblastic Differentiation: Another DICER1-Associated Tumor.
Kroll-Wheeler L, Heider A
Pediatr Dev Pathol 2022 Mar-Apr;25(2):186-191. Epub 2021 Sep 13 doi: 10.1177/10935266211043861. PMID: 34515577
Metachronous anaplastic sarcoma of the kidney and thyroid follicular carcinoma as manifestations of DICER1 abnormalities.
Yoshida M, Hamanoue S, Seki M, Tanaka M, Yoshida K, Goto H, Ogawa S, Takita J, Tanaka Y
Hum Pathol 2017 Mar;61:205-209. Epub 2016 Sep 30 doi: 10.1016/j.humpath.2016.06.024. PMID: 27697588
Tumor progression in DICER1-mutated cystic nephroma-witnessing the genesis of anaplastic sarcoma of the kidney.
Wu MK, Cotter MB, Pears J, McDermott MB, Fabian MR, Foulkes WD, O'Sullivan MJ
Hum Pathol 2016 Jul;53:114-20. Epub 2016 Mar 29 doi: 10.1016/j.humpath.2016.03.002. PMID: 27036314
Anaplastic sarcoma of the kidney: case report and literature review.
Watanabe N, Omagari D, Yamada T, Nemoto N, Furuya T, Sugito K, Koshinaga T, Yagasaki H, Sugitani M
Pediatr Int 2013 Oct;55(5):e129-32. doi: 10.1111/ped.12167. PMID: 24134767
Anaplastic sarcoma of the kidney with chromosomal abnormality: first report on cytogenetic findings.
Gomi K, Hamanoue S, Tanaka M, Matsumoto M, Kitagawa N, Niwa T, Aida N, Kigasawa H, Tanaka Y
Hum Pathol 2010 Oct;41(10):1495-9. Epub 2010 Jul 24 doi: 10.1016/j.humpath.2010.03.008. PMID: 20656319

Therapy

Anaplastic sarcoma of the kidney.
Labanaris A, Zugor V, Smiszek R, Nützel R, Kühn R
ScientificWorldJournal 2009 Feb 15;9:97-101. doi: 10.1100/tsw.2009.15. PMID: 19219373Free PMC Article

Prognosis

Anaplastic sarcoma of the kidney (DICER1-sarcoma of the kidney): A report from the International Pleuropulmonary Blastoma/DICER1 Registry.
Schoettler PJ, Smith CC, Nishitani M, Harris AK, Nelson AT, Watson DA, Kamihara J, Mullen EA, Hill DA, Messinger YH, Fair DB, Kumar KA, Dehner LP, Ash S, Chen KS, Schultz KAP
Pediatr Blood Cancer 2024 Aug;71(8):e31090. Epub 2024 May 28 doi: 10.1002/pbc.31090. PMID: 38807260
Anaplastic sarcoma of the kidney: case report and literature review.
Watanabe N, Omagari D, Yamada T, Nemoto N, Furuya T, Sugito K, Koshinaga T, Yagasaki H, Sugitani M
Pediatr Int 2013 Oct;55(5):e129-32. doi: 10.1111/ped.12167. PMID: 24134767
Anaplastic sarcoma of the kidney.
Labanaris A, Zugor V, Smiszek R, Nützel R, Kühn R
ScientificWorldJournal 2009 Feb 15;9:97-101. doi: 10.1100/tsw.2009.15. PMID: 19219373Free PMC Article

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