Dermatofibrosarcoma protuberans treated at a single institution: a surgical disease with a high cure rate.
OBJECTIVE
Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) beta-chain from gene rearrangement as a key pathogenetic factor, now susceptible of molecular-targeted therapy. The aim of this retrospective analysis was to explore the outcome of patients with primary or recurrent DFSP.


METHODS
Two hundred eighteen patients surgically treated at the Istituto Nazionale per lo studio e la cura dei Tumori (Milan, Italy) over 20 years were reviewed. Local relapse, distant metastasis, and survival were studied.


RESULTS
One hundred thirty-six patients (62.4%) presented with a primary DFSP, while 82 patients (37.6%) had a recurrent disease. In the primary group, margins were microscopically positive in 11.8%, while in the recurrent group they were positive in 14.6% (P =.613). In the primary group, patients undergoing re-excision after inadequate previous surgery had residual disease in 62% of cases. Reconstructive surgery was needed in 30%, significantly more frequently in patients with a recurrence or a head and neck tumor. The crude cumulative incidence of local relapses was 4% at 10 years, and 2% at 10 years for distant metastases. No significant difference was found between primary and recurrent patients, as well as between positive and negative margins.


CONCLUSIONS
This being one of the largest mono-institutional series of DFSP, we confirm that long-term outcome is excellent, in terms of both local and distant control, after a wide excision with negative margins. Reconstructive surgery is often needed. Novel medical therapies will be of use in a limited subgroup of patients.
Fiore
Marco
M
Department of Surgery, Istituto Nazionale per lo studio e la cura dei Tumori, via Venezian 1, 20133 Milano, Italy.
Miceli
Rosalba
R
Mussi
Chiara
C
Lo Vullo
Salvatore
S
Mariani
Luigi
L
Lozza
Laura
L
Collini
Paola
P
Olmi
Patrizia
P
Casali
Paolo G
PG
Gronchi
Alessandro
A
D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D015331:Cohort Studies; D018223:Dermatofibrosarcoma / Q000473:pathology / Q000601:surgery*; D005121:Extremities / Q000473:pathology; D005260:Female; D005500:Follow-Up Studies; D006258:Head and Neck Neoplasms / Q000556:secondary* / Q000601:surgery*; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000473:pathology / Q000601:surgery*; D009367:Neoplasm Staging; D012189:Retrospective Studies; D012307:Risk Factors; D012878:Skin Neoplasms / Q000556:secondary* / Q000601:surgery*