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Conventional Dermatofibrosarcoma Protuberans

MedGen UID:
353564
•Concept ID:
C1707508
•
Neoplastic Process

Definition

A morphologic variant of dermatofibrosarcoma protuberans characterized by the presence of spindle-shaped fibroblastic cells, histiocytic cells, and a storiform growth pattern. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVConventional 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
Genetics of rare mesenchymal tumors: implications for targeted treatment in DFSP, ASPS, CCS, GCTB and PEComa.
Rutkowski P, Przybył J, Świtaj T
Int J Biochem Cell Biol 2014 Aug;53:466-74. Epub 2014 Apr 2 doi: 10.1016/j.biocel.2014.03.024. PMID: 24704529
The impact of advances in molecular genetic pathology on the classification, diagnosis and treatment of selected soft tissue tumors of the head and neck.
García JJ, Folpe AL
Head Neck Pathol 2010 Mar;4(1):70-6. Epub 2010 Jan 22 doi: 10.1007/s12105-009-0160-z. PMID: 20237992Free PMC Article

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
Expression of COL1A1-PDGFB fusion transcripts in superficial adult fibrosarcoma suggests a close relationship to dermatofibrosarcoma protuberans.
Sheng WQ, Hashimoto H, Okamoto S, Ishida T, Meis-Kindblom JM, Kindblom LG, Hisaoka M
J Pathol 2001 May;194(1):88-94. doi: 10.1002/path.839. PMID: 11329146

Diagnosis

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
A case of congenital pigmented dermatofibrosarcoma protuberans (Bednar tumor) in a patient with Fanconi anemia.
Lee DW, Yang JH, Won CH, Chang SE, Lee MW, Choi JH, Moon KC
Pediatr Dermatol 2011 Sep-Oct;28(5):583-5. Epub 2011 Mar 25 doi: 10.1111/j.1525-1470.2011.01350.x. PMID: 21438918
Bednar tumor of the foot: a case report.
Vandeweyer E, Deraemaecker R, Somerhausen ND, Geledan L, Gebhart M
Foot Ankle Int 2001 Apr;22(4):339-41. doi: 10.1177/107110070102200412. PMID: 11354449
Expression of COL1A1-PDGFB fusion transcripts in superficial adult fibrosarcoma suggests a close relationship to dermatofibrosarcoma protuberans.
Sheng WQ, Hashimoto H, Okamoto S, Ishida T, Meis-Kindblom JM, Kindblom LG, Hisaoka M
J Pathol 2001 May;194(1):88-94. doi: 10.1002/path.839. PMID: 11329146

Therapy

A case of congenital pigmented dermatofibrosarcoma protuberans (Bednar tumor) in a patient with Fanconi anemia.
Lee DW, Yang JH, Won CH, Chang SE, Lee MW, Choi JH, Moon KC
Pediatr Dermatol 2011 Sep-Oct;28(5):583-5. Epub 2011 Mar 25 doi: 10.1111/j.1525-1470.2011.01350.x. PMID: 21438918

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