ORIGINAL ARTICLE – BONE AND SOFT TISSUE SARCOMAS
Dermatofibrosarcoma Protuberans: How Wide Should
We Resect?
Jeffrey M. Farma, MD
1
, John B. Ammori, MD
2
, Jonathan S. Zager, MD
3
, Suroosh S. Marzban, BS
3
,
Marilyn M. Bui, MD, PhD
4,5
, Christopher K. Bichakjian, MD
6
, Timothy M. Johnson, MD
6
, Lori Lowe, MD
7
,
Michael S. Sabel, MD
8
, Sandra L. Wong, MD
8
, G. Douglas Letson, MD
5
, Jane L. Messina, MD
3,4,9
,
Vincent M. Cimmino, MD
2
, and Vernon K. Sondak, MD
3
1
Department of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, PA;
2
Department of Surgical Oncology,
Memorial Sloan Kettering Cancer Center, New York, NY;
3
Department of Cutaneous Oncology, Moffitt Cancer Center,
Tampa, FL;
4
Department of Anatomic Pathology, Moffitt Cancer Center, Tampa, FL;
5
Department of Sarcoma Oncology,
Moffitt Cancer Center, Tampa, FL;
6
Department of Dermatology, University of Michigan Medical School, Ann Arbor, MI;
7
Department of Pathology, University of Michigan Medical School, Ann Arbor, MI;
8
Department of Surgery, University of
Michigan Medical School, Ann Arbor, MI;
9
Departments of Pathology, Cell Biology and Dermatology, University of
South Florida College of Medicine, Tampa, FL
ABSTRACT
Background. Dermatofibrosarcoma protuberans (DFSP)
is a rare dermal tumor with local recurrence rates ranging
from 0 to 50%. Controversy exists regarding margin width
and excision techniques, with some advocating Mohs sur-
gery and others wide excision (WE). We reviewed the
experience in two tertiary centers using WE with total
peripheral margin pathologic evaluation.
Materials and Methods. Institutional Review Board
approved retrospective review of patients with DFSP from
1991 to 2008. Patients had initial WE using 1–2 cm mar-
gins with primary or delayed closure; further excision was
done whenever feasible for positive margins. Pathologic
analysis included en face sectioning. We evaluated margin
width, number of WE, reconstruction methods, radiation,
and outcomes.
Results. A total of 206 DFSP lesions in 204 patients (76
males, 128 females), median age 41 years (range 1–84)
were treated. Locations were trunk (135), extremities (43),
and head and neck (28). The median number of excisions
to achieve negative margins was 1 (range 1–4) with a
median excision width of 2 cm (range 0.5–3 cm). Closure
techniques included primary closure (142; 69%), skin
grafting (52; 25%), and tissue flaps (9; 4%). There were 9
patients who received postoperative radiation, 6 for posi-
tive margins after maximal surgical excision. At a median
follow-up of 64 months (range 1–210), 2 patients (1%)
with head and neck primaries recurred locally.
Conclusions. Using a standardized surgical approach
including meticulous pathologic evaluation of margins, a
very low recurrence rate (1%) was achieved with relatively
narrow margins (median 2 cm), allowing primary closure
in 69% of patients. This approach spares the additional
morbidity associated with wider resection margins and in
our experience represents the treatment of choice for DFSP
occurring on the trunk and extremities.
Dermatofibrosarcoma protuberans (DFSP) is a rare
dermal malignancy with a propensity to be locally
aggressive but rarely metastatic.
1
Rouhani et al. recently
performed an analysis of the Surveillance, Epidemiology,
and End Results (SEER) program database from 1992 to
2004 and found DFSP to comprise 18.4% of all cutaneous
sarcomas diagnosed during that time period, with an equal
gender distribution and a higher incidence among blacks
than seen for other cutaneous sarcomas.
2
These authors
found DFSP to be very rare in children; incidence rates
climbed exponentially until the age of 20 years, at which
point they plateaued.
DFSP generally presents as an asymptomatic, firm,
raised dermal nodule or plaque, and diagnosis is frequently
delayed because of the large differential diagnosis and the
Ó Society of Surgical Oncology 2010
First Received: 12 January 2010;
Published Online: 31 March 2010
J. M. Farma, MD
e-mail: Jeffrey.Farma@fccc.edu
Ann Surg Oncol (2010) 17:2112–2118
DOI 10.1245/s10434-010-1046-8