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Dedifferentiated Dermatofibrosarcoma Protuberans

MedGen UID:
232420
•Concept ID:
C1333264
•
Neoplastic Process

Definition

A morphologic variant of dermatofibrosarcoma protuberans characterized by the presence of poorly differentiated sarcomatous components. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVDedifferentiated Dermatofibrosarcoma Protuberans

Professional guidelines

PubMed

The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis.
Lai JP, Robbins PF, Raffeld M, Aung PP, Tsokos M, Rosenberg SA, Miettinen MM, Lee CC
Mod Pathol 2012 Jun;25(6):854-8. Epub 2012 Mar 2 doi: 10.1038/modpathol.2012.31. PMID: 22388761Free PMC Article
Collagen triple helix repeat containing-1 in the differential diagnosis of dermatofibrosarcoma protuberans and dermatofibroma.
Wang L, Xiang YN, Zhang YH, Tu YT, Chen HX
Br J Dermatol 2011 Jan;164(1):135-40. Epub 2010 Nov 11 doi: 10.1111/j.1365-2133.2010.10050.x. PMID: 20849518

Recent clinical studies

Etiology

Loss of dimethylated H3K27 (H3K27me2) expression is not a specific marker of malignant peripheral nerve sheath tumor (MPNST): An immunohistochemical study of 137 cases, with emphasis on MPNST and melanocytic tumors.
Thangaiah JJ, Westling BE, Roden AC, Giannini C, Tetzlaff M, Cho WC, Folpe AL
Ann Diagn Pathol 2022 Aug;59:151967. Epub 2022 May 6 doi: 10.1016/j.anndiagpath.2022.151967. PMID: 35567887
Novel Aspects of Genetics, Molecular Biology and Clinical Oncology of Sarcomas.
Houfková K, Hatina J
Klin Onkol 2020 Winter;33(1):66-78. doi: 10.14735/amko202066. PMID: 32075391
Atypical Spindle Cell Lipomatous Tumor: Clinicopathologic Characterization of 232 Cases Demonstrating a Morphologic Spectrum.
Mariño-Enriquez A, Nascimento AF, Ligon AH, Liang C, Fletcher CD
Am J Surg Pathol 2017 Feb;41(2):234-244. doi: 10.1097/PAS.0000000000000770. PMID: 27879515
Dermatofibrosarcoma Protuberans: A comprehensive review on the spectrum of clinico-radiological presentations.
Sung TH, Tam AC, Khoo JL
J Med Imaging Radiat Oncol 2017 Feb;61(1):9-17. Epub 2016 Oct 18 doi: 10.1111/1754-9485.12549. PMID: 27753236
Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study.
Italiano A, Di Mauro I, Rapp J, Pierron G, Auger N, Alberti L, Chibon F, Escande F, Voegeli AC, Ghnassia JP, Keslair F, Laé M, Ranchère-Vince D, Terrier P, Baffert S, Coindre JM, Pedeutour F
Lancet Oncol 2016 Apr;17(4):532-538. Epub 2016 Mar 10 doi: 10.1016/S1470-2045(15)00583-5. PMID: 26970672

Diagnosis

PRAME immunohistochemistry in soft tissue tumors and mimics: a study of 350 cases highlighting its imperfect specificity but potentially useful diagnostic applications.
Cammareri C, Beltzung F, Michal M, Vanhersecke L, Coindre JM, Velasco V, Le Loarer F, Vergier B, Perret R
Virchows Arch 2023 Aug;483(2):145-156. Epub 2023 Jul 21 doi: 10.1007/s00428-023-03606-6. PMID: 37477762
The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
The Diagnostic and Clinical Significance of TFE3 Immunohistochemical Nuclear Expression in Solitary Fibrous Tumour.
Zhou L, Wang A, Yu G, Zhou J, Xu H, Wang C
Anal Cell Pathol (Amst) 2020;2020:8232803. Epub 2020 May 28 doi: 10.1155/2020/8232803. PMID: 32566457Free PMC Article
Dermatofibrosarcoma Protuberans: A comprehensive review on the spectrum of clinico-radiological presentations.
Sung TH, Tam AC, Khoo JL
J Med Imaging Radiat Oncol 2017 Feb;61(1):9-17. Epub 2016 Oct 18 doi: 10.1111/1754-9485.12549. PMID: 27753236
Collagen triple helix repeat containing-1 in the differential diagnosis of dermatofibrosarcoma protuberans and dermatofibroma.
Wang L, Xiang YN, Zhang YH, Tu YT, Chen HX
Br J Dermatol 2011 Jan;164(1):135-40. Epub 2010 Nov 11 doi: 10.1111/j.1365-2133.2010.10050.x. PMID: 20849518

Therapy

Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study.
Italiano A, Di Mauro I, Rapp J, Pierron G, Auger N, Alberti L, Chibon F, Escande F, Voegeli AC, Ghnassia JP, Keslair F, Laé M, Ranchère-Vince D, Terrier P, Baffert S, Coindre JM, Pedeutour F
Lancet Oncol 2016 Apr;17(4):532-538. Epub 2016 Mar 10 doi: 10.1016/S1470-2045(15)00583-5. PMID: 26970672
NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis.
Lai JP, Robbins PF, Raffeld M, Aung PP, Tsokos M, Rosenberg SA, Miettinen MM, Lee CC
Mod Pathol 2012 Jun;25(6):854-8. Epub 2012 Mar 2 doi: 10.1038/modpathol.2012.31. PMID: 22388761Free PMC Article

Prognosis

The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
The Diagnostic and Clinical Significance of TFE3 Immunohistochemical Nuclear Expression in Solitary Fibrous Tumour.
Zhou L, Wang A, Yu G, Zhou J, Xu H, Wang C
Anal Cell Pathol (Amst) 2020;2020:8232803. Epub 2020 May 28 doi: 10.1155/2020/8232803. PMID: 32566457Free PMC Article
Novel Aspects of Genetics, Molecular Biology and Clinical Oncology of Sarcomas.
Houfková K, Hatina J
Klin Onkol 2020 Winter;33(1):66-78. doi: 10.14735/amko202066. PMID: 32075391
Dermatofibrosarcoma Protuberans: A comprehensive review on the spectrum of clinico-radiological presentations.
Sung TH, Tam AC, Khoo JL
J Med Imaging Radiat Oncol 2017 Feb;61(1):9-17. Epub 2016 Oct 18 doi: 10.1111/1754-9485.12549. PMID: 27753236
Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study.
Italiano A, Di Mauro I, Rapp J, Pierron G, Auger N, Alberti L, Chibon F, Escande F, Voegeli AC, Ghnassia JP, Keslair F, Laé M, Ranchère-Vince D, Terrier P, Baffert S, Coindre JM, Pedeutour F
Lancet Oncol 2016 Apr;17(4):532-538. Epub 2016 Mar 10 doi: 10.1016/S1470-2045(15)00583-5. PMID: 26970672

Clinical prediction guides

The many faces of solitary fibrous tumor; diversity of histological features, differential diagnosis and role of molecular studies and surrogate markers in avoiding misdiagnosis and predicting the behavior.
Tariq MU, Din NU, Abdul-Ghafar J, Park YK
Diagn Pathol 2021 Apr 20;16(1):32. doi: 10.1186/s13000-021-01095-2. PMID: 33879215Free PMC Article
Dermatofibrosarcoma Protuberans: A comprehensive review on the spectrum of clinico-radiological presentations.
Sung TH, Tam AC, Khoo JL
J Med Imaging Radiat Oncol 2017 Feb;61(1):9-17. Epub 2016 Oct 18 doi: 10.1111/1754-9485.12549. PMID: 27753236
Clinical effect of molecular methods in sarcoma diagnosis (GENSARC): a prospective, multicentre, observational study.
Italiano A, Di Mauro I, Rapp J, Pierron G, Auger N, Alberti L, Chibon F, Escande F, Voegeli AC, Ghnassia JP, Keslair F, Laé M, Ranchère-Vince D, Terrier P, Baffert S, Coindre JM, Pedeutour F
Lancet Oncol 2016 Apr;17(4):532-538. Epub 2016 Mar 10 doi: 10.1016/S1470-2045(15)00583-5. PMID: 26970672
Loss of H3K27 trimethylation distinguishes malignant peripheral nerve sheath tumors from histologic mimics.
Schaefer IM, Fletcher CD, Hornick JL
Mod Pathol 2016 Jan;29(1):4-13. Epub 2015 Nov 20 doi: 10.1038/modpathol.2015.134. PMID: 26585554
Epidemiology and survivorship of soft tissue sarcomas in adults: a national cancer database report.
Corey RM, Swett K, Ward WG
Cancer Med 2014 Oct;3(5):1404-15. Epub 2014 Jul 8 doi: 10.1002/cam4.288. PMID: 25044961Free PMC Article

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