Skip to content
How It Works
Explore
Resources Ask a Question
Who It's For
Patients Caregivers Providers
Log In Ask a Question
PubMed This is a summary of 23 peer-reviewed journal articles Updated
Oncology

Subtypes and Risk: Understanding Your Specific DFSP Type

At a Glance

Dermatofibrosarcoma protuberans (DFSP) is classified into different subtypes that determine its risk level. Classic DFSP is low-risk and rarely spreads, while fibrosarcomatous DFSP (FS-DFSP) is more aggressive and has a higher risk of returning or spreading, requiring closer monitoring.

While all cases of Dermatofibrosarcoma protuberans (DFSP) share a similar genetic origin, they are not all the same. Pathologists look at the way the cells are arranged to “risk-stratify” your diagnosis—meaning they determine how likely the tumor is to grow back or spread [1][2].

Classic DFSP (Low Risk)

The majority of patients have Classic DFSP. Under a microscope, these cells form a “storiform” or wagon-wheel pattern [3].

  • Behavior: This version is low-grade. It is locally aggressive, meaning it likes to grow into nearby skin and fat, but it very rarely spreads to distant organs [4][5].
  • Markers: These tumors usually show high levels of the CD34 protein, which helps doctors confirm the diagnosis [3][6].

Fibrosarcomatous DFSP (FS-DFSP) (Higher Risk)

In some cases, a portion of the tumor changes and becomes more aggressive. This is called fibrosarcomatous transformation (FS-DFSP) [2][7].

  • Appearance: Pathologists describe this as a “herringbone” pattern, resembling the skeleton of a fish [8][2].
  • Behavior: FS-DFSP is considered high-grade. Compared to the classic version, it has a higher risk of local recurrence (growing back after surgery) and a greater chance of spreading to other parts of the body, such as the lungs [1][2][7].
  • Markers: In these aggressive areas, the tumor often loses its CD34 marker, which can make it harder to identify without genetic testing [9][10].

Rare Variants

You may see other names on your pathology report that describe rare “flavors” of DFSP:

  • Bednar Tumor (Pigmented DFSP): This variant contains melanin-producing cells, giving the tumor a darker color [11][12]. Despite its unique look, its behavior and prognosis are generally the same as classic DFSP [13][14].
  • Giant Cell Fibroblastoma (GCF): This is often called the “juvenile” version of DFSP because it most commonly occurs in children [15][16]. It shares the same genetic “glitch” as adult DFSP and is treated with similar surgical care to prevent recurrence [15][17].

Comparison of Subtypes

Feature Classic DFSP Fibrosarcomatous (FS-DFSP)
Growth Pattern Storiform (Wagon-wheel) Herringbone (Fishbone)
CD34 Protein Strong / High [3] Low / Absent [9]
Recurrence Risk Moderate (Local only) [4] High (Local & Systemic) [7]
Metastatic Risk Very Low (<1%) [5] Significant (up to 15%) [1]
Treatment Focus Local Surgical Control [18] Multi-disciplinary / Imaging [19]

Why Subtype Matters

If your report mentions fibrosarcomatous features, your medical team may recommend a more intensive approach [20]. This might include wider surgical margins, more frequent follow-up exams, or baseline imaging (like a chest CT) to ensure the cancer has not spread [20][21][22]. Regardless of the subtype, the goal remains the same: complete removal of the tumor to give you the best possible outcome [18][23].

Common questions in this guide

What is the difference between classic DFSP and fibrosarcomatous DFSP?
Classic DFSP is a low-grade tumor that rarely spreads to other organs, though it can grow into nearby skin and fat. Fibrosarcomatous DFSP (FS-DFSP) is a more aggressive, high-grade variant that has a higher risk of returning after surgery and a greater chance of spreading to other parts of the body.
What does a 'herringbone' pattern mean on my DFSP pathology report?
A herringbone pattern noted under the microscope indicates fibrosarcomatous DFSP (FS-DFSP). This means a portion of the tumor has become more aggressive, which typically requires a more intensive treatment approach like wider surgical margins and more frequent follow-up exams.
Will I need imaging scans for my DFSP?
If your pathology report shows high-risk fibrosarcomatous features, your doctor may recommend baseline imaging, such as a chest CT scan. This is done to ensure the cancer has not spread to other parts of the body, like the lungs.
What is a Bednar tumor?
A Bednar tumor is a rare, pigmented variant of DFSP that contains melanin-producing cells, giving it a darker color. Despite its unique appearance, a Bednar tumor behaves just like classic DFSP and has the same generally positive prognosis.
Can children get DFSP?
While classic DFSP usually affects adults, a specific variant called Giant Cell Fibroblastoma (GCF) most commonly occurs in children. It is often referred to as the juvenile version of DFSP, shares the same genetic causes, and is treated with similar surgical care.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.What percentage of my tumor is 'classic' vs 'fibrosarcomatous'?
  2. 2.Does the presence of the 'herringbone' pattern mean I need more aggressive surgery or wider margins?
  3. 3.Given my subtype, do I need baseline imaging (like a chest CT) to check for spread?
  4. 4.How does my subtype change the frequency or type of follow-up appointments I will need?
  5. 5.If I have the Giant Cell Fibroblastoma variant, should my child be evaluated by a pediatric specialist?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (23)
  1. 1

    Dermatofibrosarcoma protuberans: A clinical analysis.

    Lyu A, Wang Q

    Oncology letters 2018; (16(2)):1855-1862 doi:10.3892/ol.2018.8802.

    PMID: 30008876
  2. 2

    Dermatofibrosarcoma Protuberans: An Updated Review of the Literature.

    Jozwik M, Bednarczuk K, Osierda Z

    Cancers 2024; (16(18)) doi:10.3390/cancers16183124.

    PMID: 39335097
  3. 3

    Review of dermatofibrosarcoma protuberans.

    Lim SX, Ramaiya A, Levell NJ, Venables ZC

    Clinical and experimental dermatology 2023; (48(4)):297-302 doi:10.1093/ced/llac111.

    PMID: 36630365
  4. 4

    Wide local excision vs. Mohs Tübingen technique in the treatment of dermatofibrosarcoma protuberans: a two-centre retrospective study and literature review.

    Veronese F, Boggio P, Tiberio R, et al.

    Journal of the European Academy of Dermatology and Venereology : JEADV 2017; (31(12)):2069-2076 doi:10.1111/jdv.14378.

    PMID: 28573714
  5. 5

    Dermatofibrosarcoma Protuberans.

    Allen A, Ahn C, Sangüeza OP

    Dermatologic clinics 2019; (37(4)):483-488 doi:10.1016/j.det.2019.05.006.

    PMID: 31466588
  6. 6

    A case of aggressive giant dermatofibrosarcoma protuberance occurring in the parotid gland.

    Adilbay D, Khozhamkul F, Toiynbekova S, Ahmetov D

    International journal of surgery case reports 2019; (55()):58-61 doi:10.1016/j.ijscr.2018.12.006.

    PMID: 30703718
  7. 7

    Dermatofibrosarcoma Protuberans: A Study of 148 Patients.

    Marcoval J, Moreno-Vílchez C, Torrecilla-Vall-Llosera C, et al.

    Dermatology (Basel, Switzerland) 2024; (240(3)):487-493 doi:10.1159/000536172.

    PMID: 38228098
  8. 8

    Fibrosarcomatous dermatofibrosarcoma protuberans: a rapidly growing 30 cm mass on the posterior scalp.

    Lerttiendamrong B, Annoppornchai P, Promniyom P

    Asian biomedicine : research, reviews and news 2023; (17(4)):200-205 doi:10.2478/abm-2023-0060.

    PMID: 37860677
  9. 9

    Clinicopathological features of fibrosarcomatous dermatofibrosarcoma protuberans and the construction of a back-propagation neural network recognition model.

    Li Y, Liang J, Xu X, et al.

    Orphanet journal of rare diseases 2021; (16(1)):48 doi:10.1186/s13023-021-01698-4.

    PMID: 33499900
  10. 10

    Dermatofibrosarcoma protuberans with platelet-derived growth factor-D rearrangement; two cases with morphologically distinct presentations.

    Campbell K, Bridge JA, DiMaio D, et al.

    Journal of cutaneous pathology 2022; (49(3)):274-277 doi:10.1111/cup.14148.

    PMID: 34628665
  11. 11

    A Bednar tumor in a healthy woman.

    Yamada T, Ohwada S

    Clinical case reports 2020; (8(10)):2076-2077 doi:10.1002/ccr3.3029.

    PMID: 33088558
  12. 12

    Bednar Tumor: An Uncommon Entity.

    Amonkar GP, Rupani A, Shah A, Deshpande R

    Dermatopathology (Basel, Switzerland) 2016; (3(2)):36-8 doi:10.1159/000445714.

    PMID: 27504443
  13. 13

    Pigmented Dermatofibrosarcoma Protuberans: Description of a pediatric case.

    Nieto-Benito LM, Berenguer-Fröhner B, Parra-Blanco V, Campos-Domínguez M

    Revista chilena de pediatria 2020; (91(1)):99-104 doi:10.32641/rchped.v91i1.1303.

    PMID: 32730419
  14. 14

    Bednar's tumor at right shoulder in an adult male: a case report of a rare entity.

    Acharya B, Yadav DK, Chetry A, et al.

    Annals of medicine and surgery (2012) 2024; (86(2)):1196-1199 doi:10.1097/MS9.0000000000001672.

    PMID: 38333248
  15. 15

    Recent advances in the diagnosis, classification and molecular pathogenesis of cutaneous mesenchymal neoplasms.

    Papke DJ, Hornick JL

    Histopathology 2022; (80(1)):216-232 doi:10.1111/his.14450.

    PMID: 34958499
  16. 16

    COL1A1-PDGFB Fusion Gene Detection Through Bulk RNA-Seq and Transcriptomic Features of Dermatofibrosarcoma Protuberans.

    Peng R, Zhang G, Li H

    Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.] 2023; (49(5S)):S27-S33 doi:10.1097/DSS.0000000000003771.

    PMID: 37115997
  17. 17

    Alternative PDGFD rearrangements in dermatofibrosarcomas protuberans without PDGFB fusions.

    Dadone-Montaudié B, Alberti L, Duc A, et al.

    Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc 2018; (31(11)):1683-1693 doi:10.1038/s41379-018-0089-4.

    PMID: 29955147
  18. 18

    Dermatofibrosarcoma Protuberans Re-excision and Recurrence Rates in the Netherlands Between 1989 and 2016.

    van Lee CB, Kan WC, Gran S, et al.

    Acta dermato-venereologica 2019; (99(12)):1160-1165 doi:10.2340/00015555-3287.

    PMID: 31410492
  19. 19

    Diagnosis and treatment of dermatofibrosarcoma protuberans. European consensus-based interdisciplinary guideline.

    Saiag P, Grob JJ, Lebbe C, et al.

    European journal of cancer (Oxford, England : 1990) 2015; (51(17)):2604-8.

    PMID: 26189684
  20. 20

    Preoperative Risk Factors for Fibrosarcomatous Transformation in Dermatofibrosarcoma Protuberans.

    Mallett KE, Almubarak S, Claxton RM, et al.

    Anticancer research 2022; (42(1)):105-108 doi:10.21873/anticanres.15463.

    PMID: 34969715
  21. 21

    Fifth-Time Recurrence of Dermatofibrosarcoma Protuberans at Distinct Sites: A Rare Case Report.

    Tariq H, Javed H, Amin MHJ

    Clinical case reports 2025; (13(12)):e71636 doi:10.1002/ccr3.71636.

    PMID: 41438601
  22. 22

    Outcome After Surgical Treatment of Dermatofibrosarcoma Protuberans (DFSP): Does it Require Extensive Follow-up and What is an Adequate Resection Margin?

    Alshaygy I, Mattei JC, Basile G, et al.

    Annals of surgical oncology 2023; (30(5)):3106-3113 doi:10.1245/s10434-022-12953-8.

    PMID: 36658251
  23. 23

    Treatment of dermatofibrosarcoma of the head and neck with Mohs surgery with paraffin sections.

    González A, Etchichury D, Rivero JM, Adamo L

    Journal of plastic, reconstructive & aesthetic surgery : JPRAS 2021; (74(5)):1061-1070 doi:10.1016/j.bjps.2020.10.062.

    PMID: 33317985

This page explains DFSP subtypes and pathology terminology for educational purposes only. Always consult your pathologist or oncologist to interpret your specific report and discuss your recurrence risk.

Get notified when new evidence is published on Dermatofibrosarcoma protuberans.

We monitor PubMed for new peer-reviewed studies on this topic and email a short summary when something meaningful changes.