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FERRAND
DERMATOFIBROSARCOMA PROTUBERANS
Source: Orphanet
Darier J, Ferrand M. Dermatofibromes progressifs et rcidivants ou fibrosarcomes de la peau. Ann Dermatol Syph. 1924;
5:545 - 62.
Hoffmann E. Uber das Knollentreinbende Fibrosarkom der Haut (Dermatofibrosarcoma protuberans). Dermatol Z. 1925;
43:1.
Taylor RW. Sarcomatous Tumors ressembling in some respects Kelod. J Cutan Genitourin Dis. 1890; 8:384 - 387
Stout, A. P. and Lattes, R. (1967) Atlas of Tumor Pathology, Second Series, Fascicle I, Tumors of the Soft Tissues. Washington,
United States Armed Forces Institute (of Pathology), 35-37.
EPIDEMIOLOGY
Prevalence is estimated to be 1 / 10 000.
Annual incidence increases by 43% (3.1 to 4.4 per million per year)
only in whites.
Criscione VD, Weinstock MA. Descriptive epidemiology of dermatofibrosarcoma protuberans in the United States,
1973 to 2002. J Am Acad Dermatol. 2007 Jun;56(6):968-73.
VARIATIONS & RACE
Simon MP, Pedeutour F, Sirvent N, et al. Deregulation of the platelet-derived growth factor B-chain gene via fusion with
collagen gene COL1A1 in dermatofibrosarcoma protuberans and giant-cell fibroblastoma. Nat Genet. 1997 Jan. 15(1):
95-8.
CAUSES
Immunohistochemistry studies
Source, emedicine
have shown moderate-to-strong
staining of human progenitor cell
antigen CD34 in tumor cells. In
dermatofibroma, tumor cells are
positive for factor XIIIa and are
rarely positive for CD34.
Additionally, immunostaining
using CD34 as a marker is helpful
in identifying tumor cells at the
surgical margins, particularly when
treating recurrent DFSP in which
tumor cell fascicles are often
interspersed with the scar tissue.
[
HISTOLOGIC FINDINGS
DuBay D, Cimmino V, Lowe L, Johnson TM, Sondak VK. Low recurrence rate after surgery for dermatofibrosarcoma
protuberans: a multidisciplinary approach from a single institution. Cancer. 2004 Mar 1. 100(5):1008-16.
Farma JM, Ammori JB, Zager JS, et al. Dermatofibrosarcoma protuberans: how wide should we resect?. Ann Surg Oncol.
2010 Aug. 17(8):2112-8
SURGICAL CARE
DFSP is characterized by its aggressive local invasion. The tumor invades local
tissue by extending tentaclelike projections underneath healthy skin,
rendering complete removal of the tumor very difficult. this accounts in part
for the high recurrence rate after standard surgical excision.
A superior cure rate (an overall average recurrence rate of 1.3% among 463
cases reported) and tissue conservation are seen when Mohs micrographic
surgery is used; thus, Mohs micrographic surgery is now considered the
treatment of choice, particularly when a lesion is located in the head and neck
region.
Clinical practice guidelines in Oncology: DFSP v. 1.2012
Reconstruction using Integra
SURGERY AND ADJUVANT TREATMENTS
Fields RC, Hameed M, Qin LX, et al. Dermatofbrosarcoma protuberans (DFSP): predictors of recurrence and the use of
systemic therapy. Ann Surg Oncol 2011;18:328336.
MEDICAL CARE: CHEMOTHERAPY
Imatinib mesylate has been approved by the FDA (19/10/2006) for the
treatment of unresectable, recurrent, and/or metastatic DFSP in adult patients.
MEDICAL CARE: RADIOTHERAPY
High local recurrence rate (up to 49% reported). Most recurrences occur within 3
years of the primary excision (mean 32 months)
Patients should be seen every 6 months during this period and annually thereafter.
The extent of surgical excision determines the prognosis for the patient.
Age > 50 years is a risk factor associated with a poor clinical outcome.
OTHER PROGNOSTIC FACTORS
A total of 5249 cases were identified. Of these, 3.1% of patients died during an
average of 51.4 months of follow up.
Higher second surgery rates were associated with Hispanic ethnicity, while lower
rates were associated with female gender.
Trofymenko O, Bordeaux JS, Zeitouni NC. Survival in patients with primary Dermatofibrosarcoma Protuberans:
National Cancer Data Base analysis. J Am Dermatol 2017 (accepted)