（2026.V2）NCCN临床实践指南：隆突性皮肤纤维肉瘤

指南肿瘤内科· 其他肿瘤 · 2026

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

*Jeremy Bordeaux, MD, MPH/Chair Case Comprehensive Cancer Center/ University Hospitals Seldman Cancer Center and Cleveland Clinic Taussig Cancer Institute

*Paul Nghiem, MD, PhD Vice Chair Fred Hutchinson Cancer Center

Sumaira Z. Aasi, MD $\omega$ Stanford Cancer Institute

Murad Alam,MD,MBA,MSCI $\pi$ Robert H. Lurie Comprehensive Cancer Center of Northwestern University

Arya Amini, MD §City of Hope National Medical Center

Diana Bolotin, MD, PhD The UChicago Medicine Comprehensive Cancer Center

Jennifer Chang, MD φUC San Diego Moores Cancer Center

Pei-Ling Chen,MD,PhD，默认ift Cancer Center

Carlo M. Contreras, MD 1 The Ohio State University Comprehensive Cancer Center - James Cancer Hospital and Solve Research Institute

Jessica M. Donigan,MD $\varpi$ Huntsman Cancer Institute at the University of Utah

Daniel Eisen,MD $\varpi$ UC Davis Comprehensive Cancer Center

Jeffrey M. Farma, MD 11 Fox Chase Cancer Center

*Kelly Harms, MD, PhD Wd University of Michigan Rogel Cancer Center

Nicole LeBoeuf, MD, MPH NOM 9 Dana-Farber/Brigham and Women's Cancer Center | Mass General Cancer Center

John Nicholas Lukens, MD §Abramson Cancer Center at the University of Pennsylvania

Susan Manber￥￥Publicis Health

Lawrence Mark,MD,PhD![](752,443,766,462) Indiana University Melvin and Bren Simon Comprehensive Cancer Center

Stacy McMurray, MD 口 Vanderbilt-Ingram Cancer Center

Theresa Medina, MD †University of Colorado Cancer Center

Kishwer S. Nehal, MD 4 Memorial Sloan Kettering Cancer Center

Kelly Olino,MD 11 Yale Cancer Center/Smilow Cancer Hospital

Gyorgy Paragh, MD, PhD, FAAD 0

Soo Park,MD

UC San Diego Moores Cancer Center

Tejesh Patel, MD $\varphi$

The University of Tennessee Health Science Center

Jason Rich,MD

Siteman Cancer Center at Barnes-Jewish Hospital and Washington University School of Medicine

Ashok R. Shaha, MD 1

Memorial Sloan Kettering Cancer Center

Bhavina Sharma,MD,MPH

Fred & Pamela Buffett Cancer Center

Olayemi Sokumbi, MD $\varphi$ \#

Mayo Clinic Comprehensive Cancer Center

Divya Srivastava, MD $\varphi$

UT Southwestern Simmons Comprehensive Cancer Center

Joel Sunshine, MD, PhD≠

Johns Hopkins Kimmel Cancer Center

Valencia Thomas, MD $\varphi$ \#

The University of Texas MD Anderson Cancer Center

Puja Venkat, MD §

UCLA Jonsson Comprehensive Cancer Center

Melodi Whitley, MD, PhD

Duke Cancer Institute

Yaohui Gloria Xu,MD,PhD $\mathcal{W}$

University of Wisconsin

Carbone Cancer Center

Siegrid Yu,MD $\varphi$

UCSF Helen Diller Family Comprehensive Cancer Center

Mehran Yusuf,MD

O'Neal Comprehensive Cancer Center at UAB

NCCN

Sara Espinosa, PhD Beth McCullough,RN,BS

Dermatology

Φ Diagnostic/Helointerventional radiology

Hematology/Hematology oncology

P Internal medicine

Medical oncology

Otolaryngyology

≠ Pathology/Dermatopathology

￥Patient advocacy

§ Radiotherapy/Radiation oncology

Y Reconstructive surgery

Surgery/Surgical oncology *Dtolaryngology *Discussion Section Writing

NCCN Guidelines Panel Disclosures

NCCN Guidelines Version 2.2026

Dermatofibrosarcoma Protuberans

Version 2.2026, 10/24/2025 © 2025 National Comprehensive Cancer Network® (NCCN®), All rights reserved. NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN.

NCCN Guidelines Index Table of Contents Discussion

NCCN Dermatofibrosarcoma Protuberans Panel Members

Summary of the Guidelines Updates

Clinical Presentation and Workup (DFSP-1)

Treatment (DFSP-2)

Follow-up and Therapy for Recurrence/Metastasis (DFSP-3)

Principles of Pathology (DFSP-A)

Principles of Surgery (DFSP-B)

Principles of Radiation Therapy (DFSP-C)

Abbreviations (ABBR-1)

The NCCN Guidelines® are a statement of evidence and consensus of the authors regarding their views of currently accepted approaches to

treatment. Any clinician seeking to apply or consult the NCCN Guidelines is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patient’s care or treatment. The National Comprehensive Cancer Network® (NCCN®) makes no representations or warranties of any kind regarding their content, use or application and disclaims any responsibility for their application or use in any way. The NCCN Guidelines are copyrighted by National Comprehensive Cancer Network®. All rights reserved. The NCCN Guidelines and the illustrations herein may not be reproduced in any form without the express written permission of NCCN. ©2025.

Find an NCCN Member Institution: https://www.nccn.org/home/memberinstitutions.

NCCN Categories of Evidence and Consensus: All recommendations are category 2A unless otherwise

indicated.

See NCCN Categories of Evidence and Consensus.

NCCN Categories of Preference: All recommendations are considered appropriate.

See NCCN Categories of Preference.

NCCN Guidelines Version 2.2026

Dermatofibrosarcoma Protuberans

NCCN Guidelines Index Table of Contents Discussion

UPDATES

Updates in Version 1.2026 of the NCCN Guidelines for Dermatofibrosarcoma Protuberans from Version 1.2025 include:

DFSP-1

• Preliminary Workup, second bullet, sub-bullet removed: Debulking specimens from all excisions should be examined to identify fibrosarcomatous

transformation of dermatofibrosarcoma protuberans (FS-DFSP).

• Footnote removed: If areas of transformation to fibrosarcoma or other sarcoma subtypes are identified, multidisciplinary consultation for consideration of further treatment and surveillance is recommended. FS-DFSP is associated with a higher metastasis risk. The patient should be referred to a

center with expertise in management of soft tissue sarcomas. See NCCN Guidelines for Soft Tissue Sarcoma for multimodal therapy and surveillance considerations including CT of draining nodal basin and chest.

DFSP-2

• Treatment, new bullet added: Debulking specimens from all excisions should be examined to identify fibrosarcomatous transformation of

dermatofibrosarcoma protuberans (FS-DFSP).

• Adjuvant Treatment bottom pathway revised: Multidisciplinary consultation for consideration of Consider radiation therapy (RT) or other therapy if

margins are deemed narrow by treating physician and further resection would not be feasible upon recurrence.

• Follow-Up header and bullets in that column removed:

Physical exam with focus on primary site every 6–12 months.

Patient education about regular self-exam.

Consider MRI surveillance for deeply invasive disease or other concerns for recurrence.

• Last column revised: Therapy for Recurrence/ Metastasis Follow-up (DFSP-3).

• Footnotes revised:

Footnote c: . . .The patient should be referred to a center with expertise in management of soft tissue sarcomas. See NCCN Guidelines for Soft Tissue

Sarcoma for multimodal therapy and surveillance considerations including CT of draining nodal basin and chest.

Footnote e: . . .Wide undermining is discouraged prior to confirmation of clear margins due to the difficulty of interpreting subsequent re-excised

margins, and the risk of concealing residual tumor below mobilized tissue. See Principles of Surgery (DFSP-B). Curtis KK, et al. J Natl Compr Canc

Netw 2024;22:e247036. (Also page DFSP-3)

• Footnote removed: When Mohs or other forms of PDEMA are not utilized and margins are negative, RT is not recommended. When Mohs or other

forms of PDEMA are not utilized, consider RT if margins are considered narrow by the treating physicians. RT if not given previously and further

resection is not feasible.

Continued

Updates in Version 2.2026 of the NCCN Guidelines for Dermatofibrosarcoma Protuberans from Version 1.2026 include:

MS-1

• The discussion section has been updated to reflect changes in the algorithm.

NCCN Guidelines Version 2.2026

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NCCN Guidelines Index Table of Contents Discussion

DFSP-3

• New header added: Follow-Up.

New bullets added:

◊ Physical exam with focus on primary site every 6–12 months.

◊ Patient education about regular self-exam.

◊ Consider MRI surveillance when clinical exam is insufficient to assess local recurrence.

• Footnotes revised:

Footnote h: When Mohs or other forms of PDEMA are utilized and margins are negative, RT is not recommended. When Mohs or other forms of

PDEMA are not utilized, consider RT if margins are considered narrow by the treating physicians and further resection would not be feasible upon

recurrence. RT if not given previously and further resection is not feasible.

Footnote i: Confirm tumor mutation with COL1A1::PDGFB translocation by fluorescence in situ hybridization (FISH) for the translocation of PDGRF.

Navarrete-Dechent C, et al. JAMA Dermatol 2019;155:361-369.

DFSP-A

• Fifth bullet revised: For equivocal lesions, consider fluorescence in situ hybridization (FISH), polymerase chain reaction (PCR), or conventional

cytogenetics to detect t(17;22)(q22;q13), which is a hallmark of DFSP. Fusion of the collagen type I alpha 1 gene (COL1A1) at 17q22, with the plateletderived growth factor Beta gene (PDGFBβ) at 22q13, form the oncogenic chimeric fusion gene COL1A1::PDGFBβ.

DFSP-C

• General Treatment Information, Adjuvant RT, Negative Margins, second sub-bullet revised: When Mohs or other forms of PDEMA are not utilized,

consider RT if margins are considered narrow by the treating physician and further resection would not be feasible upon recurrence, RT not given

previously, and further resection is not feasible.

Updates in Version 1.2026 of the NCCN Guidelines for Dermatofibrosarcoma Protuberans from Version 1.2025 include:

UPDATES

National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Index Table of Contents Discussion

CLINICAL PRESENTATION

PRELIMINARY WORKUP

DIAGNOSIS

ADDITIONAL WORKUP

![\nLesion suspicious\nfor skin cancer\nHistory and physical (H&P)\n·Biopsya,b\nHematoxylin and eosin (H&E)\nImmunopanel(eg, CD34, factor XIIIa)\nDFSP confirmed\n·Complete skin exam\n·Multidisciplinary consultation at a center with specialized expertise should be strongly considered, especially for large or recurrentderumed, especially for large or recurrentderomated layer, is strongly recommended for sufficient tissue sampling and accurate pathologic assessment. If biopsy is indeterminate or clinical suspicion remains, biopsy is recommended. Wide undermining is discouraged due to the difficulty of interpreting subsequent re-excisions pathologically.\n](2/aj0YEe4oUhYkBdfpda0fYP/figure479854c132a64c5f9a0f64a763da090c_0_8.png) a This tumor is frequently misdiagnosed due to inadequate tissue sampling/superficial biopsy. Punch, incisional, or core biopsy, preferably including the deeper subcutaneous layer, is strongly recommended for sufficient tissue sampling and accurate pathologic assessment. If biopsy is indeterminate or clinical suspicion remains, biopsy is recommended. Wide undermining is discouraged due to the difficulty of interpreting subsequent re-excisions pathologically.

Note: All recommendations are category 2A unless otherwise indicated.

National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Index Table of Contents Discussion

![TREATMENT\nExcision with Mohs or other forms of peripheral and deep en face margin assessment(PDEMA)c,d,e\n●Debulking specimens from all excisions should be examined to identify fibrosarcomatous transformation of dermatofibrosarcoma protuberans (FS-DFSP)c\n●For unresectable/borderline resectable disease, consider tumor mutation analysis and neoadjuvant Imatinib\nNegativemorphins\n●Re-resectiond,e\n●until margins until margins\norpossible or surgerly clear or surgery\n●Positive margins\n●For unresectable/borderline resectable disease, consider tumor mutation analysis and neoadjuvant Imatinib\nPositive margins\n●For resectible-d,euntil margins\n●For unresectable/borderline resectable disease, consider tumor mutation analysis and neoadjuvant Imatinib\n●For resectible margins\n●For residue-in the limit may be discussed 2023\nPositive margins\n●For resectible radiation disease, consider tumor mutation analysis and neoadjuvant lmatinib\n●In reconsiderad unvasilant radiation surface as feasible.\) Re reduction therapy (RT)g if margins are deemed narrow by treating physician and further resection would not be feasible upon recurrence\n\n](2/aj0YEe4oUhYkBdfpda0fYP/figure06a0c99c80d44f3b9d10dfb2cd28e3f4_0_4.png) c If areas of transformation to fibrosarcoma or other sarcoma subtypes are identified, multidisciplinary consultation for consideration of further treatment and surveillance is recommended. FS-DFSP is associated with a higher metastasis risk. The patient should be referred to a center with expertise in management of soft tissue sarcomas. See NCCN Guidelines for Soft Tissue Sarcoma for multimodal therapy and surveillance considerations including CT of chest.

d The most commonly used form of PDEMA is Mohs. See NCCN Guidelines for Squamous Cell Skin Cancer- Principles of PDEMA Technique. When anatomic structures at the deep margin(eg, major vessels, nerves, bone) preclude complete histologic evaluation of the marginal surface, Mohs or other forms of PDEMA should be used to evaluate as much of the marginal surface as feasible. Treatment considerations for non-visualized areas may be the subject of multidisciplinary discussion.

e If PDEMA is unavailable, consider wide excision. Wide undermining is discouraged prior to confirmation of clear margins due to the difficulty of interpreting subsequent re-excised margins, and the risk of concealing residual tumor below mobilized tissue. See Principles of Surgery(DFSP-B). Curtis KK, et al. J Natl Compr Canc Netw 2024;22:e247036.

f Consider neoadjuvant Imatinib for patients in whom resection with negative margins may result in unacceptable functional or cosmetic outcomes. Ugurel S, et al. Clin Cancer Res 2014;20:499-510.

9Principles of Radiation Therapy(DFSP-C).

NCCN

National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Index Table of Contents Discussion

FOLLOW-UP

THERAPY FOR RECURRENCE/METASTASIS

![● Physical exam with focus on primary site every 6-12 months\n● Patient education about regular self-exam\n●Physical exam with focus on primary site every 6-12 months\n●Patient education about regular self-exam\n●\n](2/aj0YEe4oUhYkBdfpda0fYP/figureba709bf1d87b40f38620c830f387393d_0_7.png) d The most commonly used form of PDEMA is Mohs. See NCCN Guidelines for Squamous Cell Skin Cancer- Principles of PDEMA Technique. When anatomic structures at the deep margin(eg, major vessels, nerves, bone) preclude complete histologic evaluation of the marginal surface, Mohs or other forms of PDEMA should be used to evaluate as much of the marginal surface as feasible. Treatment considerations for non-visualized areas may be the subject of multidisciplinary discussion.

eIf PDEMA is unavailable, consider wide excision. Wide undermining is discouraged prior to confirmation of clear margins due to the difficulty of interpreting subsequent re-excised margins, and the risk of concealing residual tumor below mobilized tissue. See Principles of Surgery(DFSP-B). Curtis KK, et al.J Natl Compr Canc Netw 2024;22:e247036.

g Principles of Radiation Therapy(DFSP-C).

h When Mohs or other forms of PDEMA are utilized and margins are negative, RT is not recommended. When Mohs or other forms of PDEMA are not utilized, consider RT if margins are considered narrow by the treating physicians and further resection would not be feasible upon recurrence.

Confirm COL1A1::PDGFB translocation by fluorescence in situ hybridization(FISH). Navarrete-Dechent C,et al. JAMA Dermatol 2019;155:361-369.

j NCCN Guidelines for Soft Tissue Sarcoma(Synchronous STAGE IV[EXTSARC-5]).

NCCN Guidelines Version 2.2026

Dermatofibrosarcoma Protuberans

NCCN Guidelines Index Table of Contents Discussion

• Evaluation by a qualified physician with specific expertise in sarcoma/soft tissue pathology or dermatopathology is preferred (if available).

• The spindled cells arranged in a storiform or fascicular pattern are typically bland with minimal cytologic atypia.

• Immunohistochemistry (IHC) for CD34 is mostly positive, and factor XIIIa negative.

• FS-DFSPb is characterized by transition from storiform to a herringbone pattern, with a higher degree of cellularity, cytologic atypia, mitotic activity (>5/10 high-power fields [HPFs]), and frequent loss of CD34 immunostaining. When CD34 is negative, other markers such as S100

should also be negative to rule out other spindle cell tumors.

• For equivocal lesions, consider fluorescence in situ hybridization (FISH), polymerase chain reaction (PCR), or conventional cytogenetics to detect t(17;22)(q22;q13), which is a hallmark of DFSP. Fusion of COL1A1 at 17q22, with PDGFB at 22q13, form the oncogenic chimeric fusion gene COL1A1::PDGFB.

• Margin control during excision (see Principles of Surgery [DFSP-B]) may occasionally be aided by H&E sections supplemented by CD34 IHC.

a Currently, no American Joint Committee on Cancer (AJCC) or College of American Pathologists (CAP) synoptic reporting is defined.b If areas of transformation to fibrosarcoma or other sarcoma subtypes are identified, multidisciplinary consultation for consideration of further treatment and surveillance is recommended. FS-DFSP is associated with a higher metastasis risk. The patient should be referred to a center with expertise in management of soft tissue

sarcomas. See NCCN Guidelines for Soft Tissue Sarcoma for multimodal therapy and surveillance considerations including CT of draining nodal basin and chest.

Note: All recommendations are category 2A unless otherwise indicated.

DFSP-A

PRINCIPLES OF PATHOLOGYa

NCCN Guidelines Version 2.2026

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NCCN Guidelines Index Table of Contents Discussion

DFSP-B

PRINCIPLES OF SURGERY

Goal:

• Every effort should be made to achieve clear surgical margins. Complete histologic surgical margin examination to include the entire

excised peripheral and deep margin is recommended, whenever possible. Tumor characteristics include long, irregular, subclinical

extensions. Debulking specimens from all excisions should be examined to identify FS-DFSP since this is associated with metastatic

potential.

Surgical Approach: Mohs or Other Forms of PDEMA

• NCCN Guidelines for Squamous Cell Skin Cancer - Principles of PDEMA Technique

• If Mohs or other forms of PDEMA are unavailable, consider wide excision.1

Reconstruction should be delayed until clear margins have been verified to avoid the risk of translocating the tumor within the resection

bed, thus making further margin assessments inaccurate.

1 Farma JM, Ammori JB, Zager JS, et al. Dermatofibrosarcoma protuberans: how wide should we resect? Ann Surg Oncol 2010;17:2112-2118.

Note: All recommendations are category 2A unless otherwise indicated.

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NCCN Guidelines Index Table of Contents Discussion

Note: All recommendations are category 2A unless otherwise indicated.

DFSP-C

PRINCIPLES OF RADIATION THERAPY

General Treatment Information

• Adjuvant RT:

Positive Margins/Gross Disease

◊ 50–60 Gy for indeterminate or positive margins, and up to 66 Gy for positive margins or gross tumor (2-Gy fractions per day).

◊ Fields to extend widely beyond surgical margins (eg, 3–5 cm) when clinically feasible.

Negative Margins

◊ When Mohs or other forms of PDEMA are utilized and margins are negative, RT is not recommended.

◊ When Mohs or other forms of PDEMA are not utilized, consider RT if margins are considered narrow by the treating physician and further resection would not be feasible upon recurrence.

• Recurrence/Metastasis:

RT if not given previously and further resection is not feasible; 50–60 Gy for indeterminate or positive margins, and up to 66 Gy for positive

margins or gross tumor (2-Gy fractions per day).

Fields to extend widely beyond surgical margins (eg, 3–5 cm) when clinically feasible.

NCCN

National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

ABBREVIATIONS

|CAP|College of American Pathologists| |---|---| |DFSP|dermatofibrosarcoma protuberans| |FISH|fluorescence in situ hybridization| |FS-DFSP|fibrosarcomatous transformation of DFSP| |H&E|hematoxylin and eosin| |H&P|history and physical| |HPF|high-power field|

FISH fluorescence in situ hybridization FS-DFSP fibrosarcomatous transformation of DFSP

H&E hematoxylin and eosin

HPF high-power field

IHC immunohistochemistry

PCR polymerase chain reaction PDEMA peripheral and deep en face margin assessment

NCCN Guidelines Index Table of Contents Discussion

National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Index Table of Contents Discussion

||NCCN Categories of Evidence and Consensus| |---|---| |Category 1|Based upon high-level evidence (≥1 randomized phase 3 trials or high-quality, robust meta-analyses), there is uniform NCCN consensus (≥85% support of the Panel) that the intervention is appropriate.| |Category 2A|Based upon lower-level evidence, there is uniform NCCN consensus (≥85% support of the Panel) that the intervention is appropriate.| |Category 2B|Based upon lower-level evidence, there is NCCN consensus (≥50%, but <85% support of the Panel) that the intervention is appropriate.| |Category 3|Based upon any level of evidence, there is major NCCN disagreement that the intervention is appropriate.|

All recommendations are category 2A unless otherwise indicated.

|NCCN Categories of Preference|| |---|---| |Preferred|Interventions that are based on superior efficacy, safety, and evidence; and, when appropriate, affordability.| |Other recommended|Other interventions that may be somewhat less efficacious, more toxic, or based on less mature data; or significantly less affordable for similar outcomes.| |Useful in certain circumstances|Other interventions that may be used for selected patient populations (defined with recommendation).|

All recommendations are considered appropriate.

Discussion

Table of Contents

Overview ...................................................................................................................................................................................................... MS-2

Guidelines Update Methodology .................................................................................................................................................................... MS-2

Literature Search Criteria and Guidelines Update Methodology ...................................................................................................................... MS-2

Sensitive/Inclusive Language ......................................................................................................................................................................... MS-2

Evaluation .................................................................................................................................................................................................... MS-3

Treatment ..................................................................................................................................................................................................... MS-3

Follow-up ...................................................................................................................................................................................................... MS-5

References .................................................................................................................................................................................................... MS-6

This discussion corresponds to the NCCN Guidelines for Dermatofibrosarcoma Protuberans. Last updated: October 24, 2025

NCCN

National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

Overview

Dermatofibrosarcoma protuberans (DFSP) is an uncommon, low-grade sarcoma of fibroblast origin with an incidence rate of 4.1 to 4.5 cases per million persons per year in the United States.1-4 A predilection for occurring in African Americans has been reported in one study.3 They are often clinically misdiagnosed, leading to prolonged time to diagnosis. However,DFSP rarely metastasizes.5 When metastasis occurs, it is typically in the lung, bone, or regional lymph nodes.Three-dimensional reconstruction of DFSP6 has revealed tumors with highly irregular shapes and frequent finger-like extensions.7 As a result,incomplete removal and subsequent recurrence are common without attention to full assessment of the peripheral and deep margin. The local recurrence rate for wide local excision (WLE) of DFSP in studies ranges from 10% to 60%, whereas the rate of development of regional or distant metastatic disease is only 1% and 4-7.4%, respectively.8,9

Guidelines Update Methodology

The complete details of the Development and Update of the NCCN Guidelines are available at www.NCCN.org.

Literature Search Criteria and Guidelines Update Methodology

Prior to the update of this version of the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Dermatofibrosarcoma Protuberans, an electronic search of the PubMed database was performed to obtain key literature using the following search term:dermatofibrosarcoma protuberans. The PubMed database was chosen as it remains the most widely used resource for medical literature and indexes peer-reviewed biomedical literature.10

The search results were narrowed by selecting studies in humans published in English. Results were confined to the following article

types:Clinical Trial; Guideline; Meta-Analysis; Practice Guideline;Randomized Controlled Trial; Systematic Reviews; and Validation Studies.

The data from key PubMed articles as well as articles from additional sources deemed as relevant to these guidelines as discussed by the Panel during the Guidelines update have been included in this version of the Discussion section. Recommendations for which high-level evidence is lacking are based on the Panel's review of lower-level evidence and expert opinion.

Sensitive/Inclusive Language

NCCN Guidelines strive to use language that advances the goals of equity,inclusion, and representation.11 NCCN Guidelines endeavor to use language that is person-first; not stigmatizing; anti-racist,anti-classist,anti-misognist,anti-ageist,anti-ableist,and anti-weight biased;and inclusive of individuals of all sexual orientations and gender identities. NCCN Guidelines incorporate non-gendered language,instead focusing on organ-specific recommendations. This language is both more accurate and more inclusive and can help fully address the needs of individuals of all sexual orientations and gender identities.NCCN Guidelines will continue to use the terms men, women, female,and male when citing statistics, recommendations, or data from organizations or sources that do not use inclusive terms. Most studies do not report how sex and gender data are collected and use these terms interchangeably or inconsistently. If sources do not differentiate gender from sex assigned at birth or organs present, the information is presumed to predominantly represent cisgender individuals. NCCN encourages researchers to collect more specific data in future studies and organizations to use more inclusive and accurate language in their future analyses.

NCCN

National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

Evaluation

Preliminary workup for DFSP consists of history and physical (H&P)examination, and biopsy. It should be noted that this tumor is frequently misdiagnosed due to inadequate tissue sampling resulting from shallow biopsy. As the superficial aspect of a DFSP may not be distinct from benign lesions, a punch or incisional biopsy that samples the subcutaneous layer is strongly recommended. If a biopsy is indeterminate or clinical suspicion remains, rebiopsy is recommended.Histologically, DFSP typically presents as a storiform or fascicular proliferation of bland spindled cells that extends from the dermis into the subcutis.12,13 Virtually all cases are immunohistologically CD34-positive and factor XIIa-negative with rare exceptions.14,15 Currently, no synoptic reporting is recommended.

In most cases, examination of hematoxylin and eosin-stained specimens by light microscopy results in an unequivocal diagnosis.However, differentiation of DFSP from dermatofibroma can be difficult at times. Staining with CD34,15,16 factor Xlla,14,17 and other immunomarkers such as stromelysins 3, nestin, apolipoprotein D, and cathepsin K,18-21 might be useful in such instances. The NCCN Panel recommends that appropriate and confirmatory immunostaining be performed in all cases of suspected DFSP.

After DFSP confirmation, additional workup may include a complete skin examination as well as consideration of preoperative MRI with contrast for treatment planning if there is suspicion of extensive subcutaneous extension. As decisions about diagnosis and resection may be complex, multidisciplinary consultation at a center with specialized expertise should be strongly considered, especially for large or recurrent DFSP as it may optimize clinical and reconstructive outcomes.22,23

Treatment

Initial treatment of DFSP is surgical. Because of its proclivity for irregular and frequently deep subclinical extensions, every effort should be made to remove this tumor in its entirety at the time of initial therapy.Excision with Mohs micrographic surgery(Mohs) or other forms of peripheral and deep en face margin assessment(PDEMA) is recommended over WLE. En face sectioning is preferred to prevent missing small foci of tumor. The most commonly used form of PDEMA is Mohs(See NCCN Guidelines for Squamous Cell Skin Cancer-Principles of PDEMA Technique, available at www.NCCN.org).24 When anatomic structures at the deep margin(eg, major vessels, nerves,bone) preclude complete histologic evaluation of the marginal surface via Mohs or other forms of PDEMA, these surgical techniques should be used to evaluate as much of the marginal surface as feasible. A combination of PDEMA and WLE for the deep margin has been reported in the literature.23 Treatment considerations for non-visualized areas may be the subject of multidisciplinary discussion. If PDEMA is unavailable, WLE can be considered. Wide undermining is discouraged prior to confirmation of clear margins due to the difficulty of interpreting subsequent re-excised margins, and the risk of concealing residual tumor below mobilized tissue.

It is unclear whether the histologic features of a high mitotic rate or evidence of fibrosarcomatous(FS)-DFSP change have prognostic significance in DFSP.25,26 Studies in the biomedical literature both support27-35 the connection between FS-DFSP and an increased risk of local recurrence, lower time to recurrence, and increased risk of metastasis, and refute36,37 this notion. A systematic review of 225 patients with FS-DFSP and 1422 with DFSP reported risks of local recurrence(29.8% vs.13.7%), metastasis(14.4% vs.1.1%), and death(14.7% vs.0.8%) from the disease to be significantly higher in FS-DFSP.38 Overall, FS-DFSP is associated with a metastatic risk range of

NCCN

National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

10% to 23.5%.26,29,39 The NCCN Panel recommends that the debulking specimens from all excisions should be examined to identify FS transformation of DFSP. If FS transformation or other sarcoma subtypes are found, multidisciplinary consultation for consideration of further treatment and surveillance is recommended. Clinicians should consult the NCCN Guidelines for Soft Tissue Sarcoma(available at www.NCCN.org) for multimodal therapy and surveillance considerations including chest CT.

Additionally, tumor mutation analysis and neoadjuvant imatinib can be considered options for unresectable or borderline resectable disease.Consider neoadjuvant imatinib for patients in whom DFSP resection with negative margins may result in unacceptable functional or cosmetic outcomes.40 The NCCN Panel recommends that if a negative margin is achieved, no adjuvant treatment is necessary. When Mohs or other forms of PDEMA are used, radiation therapy (RT) is not recommended.

If initial surgery yields positive margins, re-resection is recommended whenever possible, with the goal of achieving clear margins. Mohs or modified Mohs surgery, and traditional WLE with wider margins, which has been associated with higher tumor clearance and lower rates of recurrence,41-43 are all methods to achieve complete histologic assessment. Studies examining outcomes of both Mohs and WLE have consistently reported lower recurrence rates for the former(0%-6.6%vs.1.7%-30.8%).44-53 A large retrospective series of 204 patients with DFSP showed a very low local recurrence rate(1%) using WLE with total peripheral margin pathologic evaluation, underscoring the importance of meticulous pathologic margin evaluation with any surgical technique.54 This notion was also supported by more studies.55,56 It is recommended that any reconstruction involving extensive undermining be avoided. Tissue rearrangement, if necessary, should be delayed until negative histologic margins are verified to prevent displacing a

potentially positive margin or hampering interpretation of re-excisions. If there is concern that the surgical margins are not clear when Mohs or PDEMA is not available, split-thickness skin grafting should be considered to monitor for recurrence.

While radiation has occasionally been used as a primary therapeutic modality for DFSP,"57-59 it is most beneficial as adjuvant therapy after surgery.57-64 In a single-institution retrospective review of 53 patients with DFSP treated with surgery and preoperative or postoperative RT,local control was 93% and actuarial overall survival was 98% at 10 years.37 Another small patient series reported that 86% of patients with DFSP who were treated with postoperative RT remained disease-free at a median follow-up of 10.5 years.63 In a systematic review and meta-analysis of adjuvant RT for DFSP after WLE, the overall recurrence rate was reported to be 11.74%. Patients with positive/close margins had a recurrence rate of 14.23% whereas those with negative margins had no recurrence.65 The NCCN Panel recommends that if margins are positive after re-serection,RT can be considered as an adjuvant treatment if the treating physician deems the margins to be narrow and further resection would not be feasible upon recurrence.

DFSP can be treated by targeted platelet-derived growth factor receptor(PDGFRs). DFSP is characterized by a translocation between chromosomes 17 and 22 [t(17;22)(q22;q13)] resulting in the overexpression of PDGFB. ${ }^{66-68}$ These findings suggest that targeting PDGFRs may be an effective treatment for DFSP. In published results,imatinib mesylate, a protein tyrosine kinase inhibitor, has shown clinical activity against DFSP, ${ }^{40,69-73}$ which has led to its approval by the U.S.Food and Drug Administration (FDA) for the treatment of unresectable,recurrent, and/or metastatic DFSP in adult patients. It is still unclear whether or not and the extent to which the COL1A1::PDGFB fusion gene dictates imatinib response. ${ }^{70}$ In a systematic review that included

NCCN

National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

patients receiving imatinib as monotherapy, adjuvant, or neoadjuvant therapy, complete response, partial response, stable disease, and progressive disease were reported in 5.2%, 55.2%, 27.6%, and 9.2% of patients,respectively.70 In the neoadjuvant setting, complete response,partial response, stable disease, and progressive disease rates were reported to be 7.1%, 50%, 35.7%, and 7.1%, respectively.40

Follow-up

Given the historically high local recurrence rates for DFSP, ongoing clinical follow-up with focus on the primary site every 6 to 12 months is indicated, with re-biopsy of any suspicious regions. Although metastatic disease is rare, a guided H&P and patient education about regular self-examination are recommended. Consider MRI surveillance for deeply invasive disease or other concerns related to recurrence.

Recurrent tumors should be resected whenever possible. Adjuvant RT may be considered after surgery. For patients who are not surgical candidates, RT alone is an option if not given previously. Imatinib mesylate should be considered in cases where the disease is unresectable following multiple resections, or if unacceptable functional or cosmetic outcomes will occur with further resection. The NCCN Panel recommends that the COL1A1::PDGFB translocation be confirmed by fluorescence in-situ hybridization(FISH).

In the rare event of metastatic disease, multidisciplinary consultation is recommended to coordinate treatment(See NCCN Guidelines for Soft Tissue Sarcoma, available at www.NCCN.org).

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National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

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National Comprehensive Cancer Network®

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

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表 1
CAP	College of American Pathologists
DFSP	dermatofibrosarcoma protuberans
FISH	fluorescence in situ hybridization
FS-DFSP	fibrosarcomatous transformation of DFSP
H&E	hematoxylin and eosin
H&P	history and physical
HPF	high-power field

表 2
	NCCN Categories of Evidence and Consensus
Category 1	Based upon high-level evidence (≥1 randomized phase 3 trials or high-quality, robust meta-analyses), there is uniform NCCN consensus (≥85% support of the Panel) that the intervention is appropriate.
Category 2A	Based upon lower-level evidence, there is uniform NCCN consensus (≥85% support of the Panel) that the intervention is appropriate.
Category 2B	Based upon lower-level evidence, there is NCCN consensus (≥50%, but <85% support of the Panel) that the intervention is appropriate.
Category 3	Based upon any level of evidence, there is major NCCN disagreement that the intervention is appropriate.

表 3
NCCN Categories of Preference
Preferred	Interventions that are based on superior efficacy, safety, and evidence; and, when appropriate, affordability.
Other recommended	Other interventions that may be somewhat less efficacious, more toxic, or based on less mature data; or significantly less affordable for similar outcomes.
Useful in certain circumstances	Other interventions that may be used for selected patient populations (defined with recommendation).

图示：原图识别中

图示：Lesion suspicious for skin cancer History and physical (H&P) ·Biopsya,b Hematoxylin and eosin (H&E) Immunopanel(eg, CD34, factor XIIIa) DFSP confirmed ·Complete skin exam ·Multidisciplinary consultation at a center with specialized expertise should be strongly considered, especially for large or recurrentderumed, especially for large or recurrentderomated layer, is strongly recommended for sufficient tissue sampling and accurate pathologic assessment. If biopsy is indeterminate or clinical suspicion remains, biopsy is recommended. Wide undermining is discouraged due to the difficulty of interpreting subsequent re-excisions pathologically.

图示：TREATMENT Excision with Mohs or other forms of peripheral and deep en face margin assessment(PDEMA)c,d,e ●Debulking specimens from all excisions should be examined to identify fibrosarcomatous transformation of dermatofibrosarcoma protuberans (FS-DFSP)c ●For unresectable/borderline resectable disease, consider tumor mutation analysis and neoadjuvant Imatinib Negativemorphins ●Re-resectiond,e ●until margins until margins orpossible or surgerly clear or surgery ●Positive margins ●For unresectable/borderline resectable disease, consider tumor mutation analysis and neoadjuvant Imatinib Positive margins ●For resectible-d,euntil margins ●For unresectable/borderline resectable disease, consider tumor mutation analysis and neoadjuvant Imatinib ●For resectible margins ●For residue-in the limit may be discussed 2023 Positive margins ●For resectible radiation disease, consider tumor mutation analysis and neoadjuvant lmatinib ●In reconsiderad unvasilant radiation surface as feasible.\) Re reduction therapy (RT)g if margins are deemed narrow by treating physician and further resection would not be feasible upon recurrence

图示：● Physical exam with focus on primary site every 6-12 months ● Patient education about regular self-exam ●Physical exam with focus on primary site every 6-12 months ●Patient education about regular self-exam ●

引用 / 出处

（2026.V2）NCCN临床实践指南：隆突性皮肤纤维肉瘤. 2026.

（2026.V2）NCCN临床实践指南：隆突性皮肤纤维肉瘤

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

Soo Park,MD

Tejesh Patel, MD $\varphi$

Jason Rich,MD

NCCN

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

ABBREVIATIONS

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

Overview

Guidelines Update Methodology

Literature Search Criteria and Guidelines Update Methodology

Sensitive/Inclusive Language

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

Evaluation

Treatment

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

Follow-up

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

References

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

NCCN Guidelines Version 2.2026 Dermatofibrosarcoma Protuberans

相关指南

引用 / 出处