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Dermatofibrosarcoma Protuberans, Version 1.2025

delete2025-01-01
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PRE
AI
J
Jeremy S. Bordeaux
R
Rachel Blitzblau
S
Sumaira Z. Aasi
M
Murad Alam
A
Arya Amini
K
Kristin Bibee
D
Diana Bolotin
P
Pei-Ling Chen
C
Carlo M. Contreras
D
Dominick J. DiMaio
J
Jessica M. Donigan
J
Jeffrey M. Farma
K
Karthik Ghosh
K
Kelly L. Harms
N
Nicole R. LeBoeuf
J
John N. Lukens
S
Susan Manber
L
Lawrence A. Mark
T
Theresa Medina
K
Kishwer S. Nehal
P
Paul Nghiem
K
Kelly Olino
G
György Paragh
P
Patel, Tejesh
R
Rich, Jason
S
Shaha, Ashok R.
S
Sharma, Bhavina
S
Sokumbi, Yemi
S
Srivastava, Divya
T
Tomblinson, Courtney
P
Park, Soo
T
Thomas, Valencia
P
Puja Venkat
Y
Yaohui Xu
S
Siegrid S. Yu
M
Mehran Yusuf
B
Beth McCullough
S
Sara Espinosa
DOI:10.6004/jnccn.2025.0001delete
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Abstract

Abstract

En 中文
Dermatofibrosarcoma protuberans (DFSP) is a rare cutaneous soft tissue sarcoma and affects an estimated 1,500 people annually in the United States. DFSP frequently exhibits extensive local infiltration. Initial treatment is through surgical excision, and care should be taken to ensure that negative margins are achieved to minimize recurrence. Although DFSP has a reported high rate of recurrence, metastasis is more uncommon. Fibrosarcomatous DFSP is an aggressive variant with an increased risk for local recurrence and metastasis. If achieving negative margins or resection is not feasible, radiation therapy or systemic treatment are options that may be considered by a multidisciplinary team. The NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) outline recommended treatment options available for DFSP.
Keywords:
MOHS MICROGRAPHIC SURGERY
WIDE LOCAL EXCISION
MULTICENTER PHASE-II
DIFFERENTIAL-DIAGNOSIS
IMATINIB MESYLATE
USEFUL MARKER
FIBROSARCOMATOUS TRANSFORMATION
MULTIDISCIPLINARY APPROACH
PROGNOSTIC-SIGNIFICANCE
COL1A1-PDGFB FUSION

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