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Fibrosarcomatous Dermatofibrosarcoma Protuberans

MedGen UID:
811315
•Concept ID:
C3665732
•
Neoplastic Process
Synonyms: Dermatofibrosarcoma Protuberan, Fibrosarcomatous; Dermatofibrosarcoma Protuberan, Metastatic; Dermatofibrosarcoma Protuberans, Fibrosarcomatous; Dermatofibrosarcoma Protuberans, Metastatic; DFSP, Fibrosarcomatous; DFSPs, Fibrosarcomatous; Fibrosarcomatous Dermatofibrosarcoma Protuberan; Fibrosarcomatous DFSP; Fibrosarcomatous DFSPs; FS-DFSP; Metastatic Dermatofibrosarcoma Protuberan; Metastatic Dermatofibrosarcoma Protuberans; Protuberan, Fibrosarcomatous Dermatofibrosarcoma; Protuberan, Metastatic Dermatofibrosarcoma; Protuberans, Fibrosarcomatous Dermatofibrosarcoma; Protuberans, Metastatic Dermatofibrosarcoma
SNOMED CT: Fibrosarcomatous dermatofibrosarcoma protuberans (733859002); Sarcomatous dermatofibrosarcoma (733859002)

Definition

A morphologic variant of dermatofibrosarcoma protuberans characterized by the presence of a fibrosarcomatous component. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Adaptive Immunity in Fibrosarcomatous Dermatofibrosarcoma Protuberans and Response to Imatinib Treatment.
Tazzari M, Indio V, Vergani B, De Cecco L, Rini F, Negri T, Camisaschi C, Fiore M, Stacchiotti S, Dagrada GP, Casali PG, Gronchi A, Astolfi A, Pantaleo MA, Villa A, Lombardo C, Arienti F, Pilotti S, Rivoltini L, Castelli C
J Invest Dermatol 2017 Feb;137(2):484-493. Epub 2016 Sep 5 doi: 10.1016/j.jid.2016.06.634. PMID: 27608549

Recent clinical studies

Etiology

Successful treatment of metastatic fibrosarcomatous dermatofibrosarcoma protuberans with imatinib mesylate.
Kambayashi Y, Kasahara Y, Ohuchi K, Amagai R, Hashimoto A, Asano Y, Fujimura T
Dermatol Ther 2022 Oct;35(10):e15736. Epub 2022 Aug 8 doi: 10.1111/dth.15736. PMID: 35898161
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 TNC-PDGFD fusion in fibrosarcomatous dermatofibrosarcoma protuberans: a case report.
Chen Y, Shi YZ, Feng XH, Wang XT, He XL, Zhao M
Diagn Pathol 2021 Jul 13;16(1):63. doi: 10.1186/s13000-021-01123-1. PMID: 34256767Free PMC Article
Clinicopathological features of fibrosarcomatous dermatofibrosarcoma protuberans and the construction of a back-propagation neural network recognition model.
Li Y, Liang J, Xu X, Jiang X, Wang C, Chen S, Xiang B, Ji Y
Orphanet J Rare Dis 2021 Jan 26;16(1):48. doi: 10.1186/s13023-021-01698-4. PMID: 33499900Free PMC Article
A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change.
Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD
J Am Acad Dermatol 2014 Oct;71(4):781-6. Epub 2014 Apr 19 doi: 10.1016/j.jaad.2014.03.018. PMID: 24755121

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
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 TNC-PDGFD fusion in fibrosarcomatous dermatofibrosarcoma protuberans: a case report.
Chen Y, Shi YZ, Feng XH, Wang XT, He XL, Zhao M
Diagn Pathol 2021 Jul 13;16(1):63. doi: 10.1186/s13000-021-01123-1. PMID: 34256767Free PMC Article
Clinicopathological features of fibrosarcomatous dermatofibrosarcoma protuberans and the construction of a back-propagation neural network recognition model.
Li Y, Liang J, Xu X, Jiang X, Wang C, Chen S, Xiang B, Ji Y
Orphanet J Rare Dis 2021 Jan 26;16(1):48. doi: 10.1186/s13023-021-01698-4. PMID: 33499900Free PMC Article
Adaptive Immunity in Fibrosarcomatous Dermatofibrosarcoma Protuberans and Response to Imatinib Treatment.
Tazzari M, Indio V, Vergani B, De Cecco L, Rini F, Negri T, Camisaschi C, Fiore M, Stacchiotti S, Dagrada GP, Casali PG, Gronchi A, Astolfi A, Pantaleo MA, Villa A, Lombardo C, Arienti F, Pilotti S, Rivoltini L, Castelli C
J Invest Dermatol 2017 Feb;137(2):484-493. Epub 2016 Sep 5 doi: 10.1016/j.jid.2016.06.634. PMID: 27608549

Therapy

Successful treatment of metastatic fibrosarcomatous dermatofibrosarcoma protuberans with imatinib mesylate.
Kambayashi Y, Kasahara Y, Ohuchi K, Amagai R, Hashimoto A, Asano Y, Fujimura T
Dermatol Ther 2022 Oct;35(10):e15736. Epub 2022 Aug 8 doi: 10.1111/dth.15736. PMID: 35898161
Dermatofibrosarcoma protuberans with fibrosarcomatous transformation arising at the site of breast implant.
Haghayeghi K, Dicomo JA, Edmonson D, Banet N
Breast J 2020 Sep;26(9):1811-1813. Epub 2020 Mar 29 doi: 10.1111/tbj.13823. PMID: 32223026
Adaptive Immunity in Fibrosarcomatous Dermatofibrosarcoma Protuberans and Response to Imatinib Treatment.
Tazzari M, Indio V, Vergani B, De Cecco L, Rini F, Negri T, Camisaschi C, Fiore M, Stacchiotti S, Dagrada GP, Casali PG, Gronchi A, Astolfi A, Pantaleo MA, Villa A, Lombardo C, Arienti F, Pilotti S, Rivoltini L, Castelli C
J Invest Dermatol 2017 Feb;137(2):484-493. Epub 2016 Sep 5 doi: 10.1016/j.jid.2016.06.634. PMID: 27608549

Prognosis

Successful treatment of metastatic fibrosarcomatous dermatofibrosarcoma protuberans with imatinib mesylate.
Kambayashi Y, Kasahara Y, Ohuchi K, Amagai R, Hashimoto A, Asano Y, Fujimura T
Dermatol Ther 2022 Oct;35(10):e15736. Epub 2022 Aug 8 doi: 10.1111/dth.15736. PMID: 35898161
A novel case of an aggressive superficial spindle cell sarcoma in an adult resembling fibrosarcomatous dermatofibrosarcoma protuberans and harboring an EML4-NTRK3 fusion.
Olson N, Rouhi O, Zhang L, Angeles C, Bridge J, Lopez-Terrada D, Royce T, Linos K
J Cutan Pathol 2018 Dec;45(12):933-939. Epub 2018 Sep 26 doi: 10.1111/cup.13348. PMID: 30175468
Significance of H3K27me3 loss in the diagnosis of malignant peripheral nerve sheath tumors.
Pekmezci M, Cuevas-Ocampo AK, Perry A, Horvai AE
Mod Pathol 2017 Dec;30(12):1710-1719. Epub 2017 Aug 4 doi: 10.1038/modpathol.2017.97. PMID: 28776579
Risk factors for distant metastasis of dermatofibrosarcoma protuberans.
Hayakawa K, Matsumoto S, Ae K, Tanizawa T, Gokita T, Funauchi Y, Motoi N
J Orthop Traumatol 2016 Sep;17(3):261-6. Epub 2016 Jun 11 doi: 10.1007/s10195-016-0415-x. PMID: 27289468Free PMC Article
A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change.
Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD
J Am Acad Dermatol 2014 Oct;71(4):781-6. Epub 2014 Apr 19 doi: 10.1016/j.jaad.2014.03.018. PMID: 24755121

Clinical prediction guides

Evaluation of pan-TRK immunohistochemistry in infantile fibrosarcoma, lipofibromatosis-like neural tumour and histological mimics.
Hung YP, Fletcher CDM, Hornick JL
Histopathology 2018 Oct;73(4):634-644. Epub 2018 Jul 5 doi: 10.1111/his.13666. PMID: 29863809
Significance of H3K27me3 loss in the diagnosis of malignant peripheral nerve sheath tumors.
Pekmezci M, Cuevas-Ocampo AK, Perry A, Horvai AE
Mod Pathol 2017 Dec;30(12):1710-1719. Epub 2017 Aug 4 doi: 10.1038/modpathol.2017.97. PMID: 28776579
Loss of H3K27 tri-methylation is a diagnostic marker for malignant peripheral nerve sheath tumors and an indicator for an inferior survival.
Cleven AH, Sannaa GA, Briaire-de Bruijn I, Ingram DR, van de Rijn M, Rubin BP, de Vries MW, Watson KL, Torres KE, Wang WL, van Duinen SG, Hogendoorn PC, Lazar AJ, Bovée JV
Mod Pathol 2016 Jun;29(6):582-90. Epub 2016 Mar 18 doi: 10.1038/modpathol.2016.45. PMID: 26990975Free PMC Article
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
Gains of COL1A1-PDGFB genomic copies occur in fibrosarcomatous transformation of dermatofibrosarcoma protuberans.
Abbott JJ, Erickson-Johnson M, Wang X, Nascimento AG, Oliveira AM
Mod Pathol 2006 Nov;19(11):1512-8. Epub 2006 Sep 15 doi: 10.1038/modpathol.3800695. PMID: 16980946

Recent systematic reviews

A systematic review of outcome data for dermatofibrosarcoma protuberans with and without fibrosarcomatous change.
Liang CA, Jambusaria-Pahlajani A, Karia PS, Elenitsas R, Zhang PD, Schmults CD
J Am Acad Dermatol 2014 Oct;71(4):781-6. Epub 2014 Apr 19 doi: 10.1016/j.jaad.2014.03.018. PMID: 24755121

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