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Pigmented dermatofibrosarcoma protuberans

MedGen UID:
90786
•Concept ID:
C0334464
•
Neoplastic Process
Synonym: Bednar's tumor
SNOMED CT: Pigmented dermatofibrosarcoma protuberans (62621002); Bednar tumor (62621002); Pigmented dermatofibrosarcoma protuberans of skin (398670003); Bednar tumor (398670003); Pigmented storiform neurofibroma (398670003); Pigmented dermatofibrosarcoma (398670003)
 
Monarch Initiative: MONDO:0023273

Definition

A morphologic variant of dermatofibrosarcoma protuberans characterized by the presence of melanin-pigmented dendritic cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPigmented dermatofibrosarcoma protuberans

Professional guidelines

PubMed

Management of dermatofibrosarcoma protuberans.
Badhey AK, Tikhtman R, Tang AL
Curr Opin Otolaryngol Head Neck Surg 2021 Aug 1;29(4):278-282. doi: 10.1097/MOO.0000000000000721. PMID: 33993132
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
A risk-based treatment strategy for non-rhabdomyosarcoma soft-tissue sarcomas in patients younger than 30 years (ARST0332): a Children's Oncology Group prospective study.
Spunt SL, Million L, Chi YY, Anderson J, Tian J, Hibbitts E, Coffin C, McCarville MB, Randall RL, Parham DM, Black JO, Kao SC, Hayes-Jordan A, Wolden S, Laurie F, Speights R, Kawashima E, Skapek SX, Meyer W, Pappo AS, Hawkins DS
Lancet Oncol 2020 Jan;21(1):145-161. Epub 2019 Nov 27 doi: 10.1016/S1470-2045(19)30672-2. PMID: 31786124Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Dermatofibrosarcoma Protuberans, 2022

Recent clinical studies

Etiology

Pigmented Dermatofibrosarcoma Protuberans: Description of a pediatric case.
Nieto-Benito LM, Berenguer-Fröhner B, Parra-Blanco V, Campos-Domínguez M
Rev Chil Pediatr 2020 Feb;91(1):99-104. Epub 2019 Dec 3 doi: 10.32641/rchped.v91i1.1303. PMID: 32730419
Two childhood cases of pigmented dermatofibrosarcoma protuberans with atrophic change.
Zhang Y, Chen H, Sun J
Eur J Dermatol 2018 Apr 1;28(2):225-226. doi: 10.1684/ejd.2017.3198. PMID: 29336320
Tumoral, quasitumoral and pseudotumoral lesions of the superficial and somatic soft tissue: new entities and new variants of old entities recorded during the last 25 years. Part XII: appendix.
Bisceglia M, Spagnolo D, Galliani C, Fisher C, Suster S, Kazakov DV, Cooper K, Michal M
Pathologica 2006 Aug;98(4):239-98. PMID: 17175794
Pediatric pigmented dermatofibrosarcoma protuberans (Bednár tumor): case report and review of the literature with emphasis on the differential diagnosis.
Reis-Filho JS, Milanezi F, Ferro J, Schmitt FC
Pathol Res Pract 2002;198(9):621-6. doi: 10.1078/0344-0338-00311. PMID: 12440785

Diagnosis

Pigmented dermatofibrosarcoma (Bednar tumour) of scalp.
Agarwal A, Rana C
BMJ Case Rep 2023 Sep 27;16(9) doi: 10.1136/bcr-2022-254473. PMID: 37758662Free PMC Article
LMNA-NTRK1 rearranged mesenchymal tumor (lipofibromatosis-like neural tumor) mimicking pigmented dermatofibrosarcoma protuberans.
Panse G, Reisenbichler E, Snuderl M, Wang WL, Laskin W, Jour G
J Cutan Pathol 2021 Feb;48(2):290-294. Epub 2020 Jul 6 doi: 10.1111/cup.13772. PMID: 32519338
Pigmented Dermatofibrosarcoma Protuberans: Description of a pediatric case.
Nieto-Benito LM, Berenguer-Fröhner B, Parra-Blanco V, Campos-Domínguez M
Rev Chil Pediatr 2020 Feb;91(1):99-104. Epub 2019 Dec 3 doi: 10.32641/rchped.v91i1.1303. PMID: 32730419
Pigmented Dermatofibrosarcoma Protuberans and Blue Naevi with Similar Dermoscopy: A Case Report.
Ehara Y, Yoshida Y, Shiomi T, Yamamoto O
Acta Derm Venereol 2016 Feb;96(2):272-3. doi: 10.2340/00015555-2204. PMID: 26258458
Congenital myxoid and pigmented dermatofibrosarcoma protuberans: a case report.
El Hachem M, Diociaiuti A, Latella E, Zama M, Lambiase C, Giraldi L, Surrenti T, Callea F
Pediatr Dermatol 2013 Sep-Oct;30(5):e74-7. Epub 2013 Mar 28 doi: 10.1111/pde.12131. PMID: 23534369

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
Bednar tumor (pigmented dermatofibrosarcoma protuberans) occurring in a site of prior immunization: immunochemical findings and therapy.
Elgart GW, Hanly A, Busso M, Spencer JM
J Am Acad Dermatol 1999 Feb;40(2 Pt 2):315-7. doi: 10.1016/s0190-9622(99)70474-3. PMID: 10025857

Prognosis

Pigmented Dermatofibrosarcoma Protuberans: Description of a pediatric case.
Nieto-Benito LM, Berenguer-Fröhner B, Parra-Blanco V, Campos-Domínguez M
Rev Chil Pediatr 2020 Feb;91(1):99-104. Epub 2019 Dec 3 doi: 10.32641/rchped.v91i1.1303. PMID: 32730419
Two childhood cases of pigmented dermatofibrosarcoma protuberans with atrophic change.
Zhang Y, Chen H, Sun J
Eur J Dermatol 2018 Apr 1;28(2):225-226. doi: 10.1684/ejd.2017.3198. PMID: 29336320
Pigmented Dermatofibrosarcoma Protuberans and Blue Naevi with Similar Dermoscopy: A Case Report.
Ehara Y, Yoshida Y, Shiomi T, Yamamoto O
Acta Derm Venereol 2016 Feb;96(2):272-3. doi: 10.2340/00015555-2204. PMID: 26258458
Fibrosarcomatous Bednar tumor with distant metastases--a case report.
Kini H, Raghuveer CV, Pai MR, S K
Indian J Pathol Microbiol 2004 Jan;47(1):26-9. PMID: 15471119
Pigmented dermatofibrosarcoma protuberans (Bednár tumor). An autopsy case with systemic metastasis.
Onoda N, Tsutsumi Y, Kakudo K, Ozawa A, Niizuma K, Ohkido M, Osamura RY
Acta Pathol Jpn 1990 Dec;40(12):935-40. PMID: 2096596

Clinical prediction guides

Pigmented Dermatofibrosarcoma Protuberans and Blue Naevi with Similar Dermoscopy: A Case Report.
Ehara Y, Yoshida Y, Shiomi T, Yamamoto O
Acta Derm Venereol 2016 Feb;96(2):272-3. doi: 10.2340/00015555-2204. PMID: 26258458
Congenital myxoid and pigmented dermatofibrosarcoma protuberans: a case report.
El Hachem M, Diociaiuti A, Latella E, Zama M, Lambiase C, Giraldi L, Surrenti T, Callea F
Pediatr Dermatol 2013 Sep-Oct;30(5):e74-7. Epub 2013 Mar 28 doi: 10.1111/pde.12131. PMID: 23534369
COL1A1-PDGFB fusion in a pediatric Bednar tumor with 2 copies of a der(22)t(17;22).
Craver R, Dewenter T, Ebran N, Pedeutour F
Cancer Genet Cytogenet 2006 Jul 15;168(2):155-7. doi: 10.1016/j.cancergencyto.2006.02.005. PMID: 16843106
Bednár tumor associated with dermal melanocytosis: melanocytic colonization or neuroectodermal multidirectional differentiation?
Goncharuk V, Mulvaney M, Carlson JA
J Cutan Pathol 2003 Feb;30(2):147-51. doi: 10.1034/j.1600-0560.2003.00030.x. PMID: 12641795
Pigmented dermatofibrosarcoma protuberans (Bednar tumor). A pathologic, ultrastructural, and immunohistochemical study.
Dupree WB, Langloss JM, Weiss SW
Am J Surg Pathol 1985 Sep;9(9):630-9. doi: 10.1097/00000478-198509000-00002. PMID: 3901787

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    Curated

    • NCCN, 2022
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Dermatofibrosarcoma Protuberans, 2022

    Reviews

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