Dermatofibrosarcoma protuberans treated at a single institution: A surgical disease with a high cure rate

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Abstract

Purpose: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) β-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. Patients and 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. Conclusion: 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.

Original languageEnglish
Pages (from-to)7669-7675
Number of pages7
JournalJournal of Clinical Oncology
Volume23
Issue number30
DOIs
Publication statusPublished - 2005

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Dermatofibrosarcoma
Reconstructive Surgical Procedures
Recurrence
Molecular Targeted Therapy
Neoplasm Metastasis
Gene Rearrangement
Platelet-Derived Growth Factor
Sarcoma
Italy
Neck
Head

ASJC Scopus subject areas

  • Cancer Research
  • Oncology

Cite this

@article{75262624cf4c4b348ee3f6a7636c4b98,
title = "Dermatofibrosarcoma protuberans treated at a single institution: A surgical disease with a high cure rate",
abstract = "Purpose: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) β-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. Patients and 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. Conclusion: 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.",
author = "Marco Fiore and Rosalba Miceli and Chiara Mussi and {Lo Vullo}, Salvatore and Luigi Mariani and Laura Lozza and Paola Collini and Patrizia Olmi and Casali, {Paolo G.} and Alessandro Gronchi",
year = "2005",
doi = "10.1200/JCO.2005.02.5122",
language = "English",
volume = "23",
pages = "7669--7675",
journal = "Journal of Clinical Oncology",
issn = "0732-183X",
publisher = "American Society of Clinical Oncology",
number = "30",

}

TY - JOUR

T1 - Dermatofibrosarcoma protuberans treated at a single institution

T2 - A surgical disease with a high cure rate

AU - Fiore, Marco

AU - Miceli, Rosalba

AU - Mussi, Chiara

AU - Lo Vullo, Salvatore

AU - Mariani, Luigi

AU - Lozza, Laura

AU - Collini, Paola

AU - Olmi, Patrizia

AU - Casali, Paolo G.

AU - Gronchi, Alessandro

PY - 2005

Y1 - 2005

N2 - Purpose: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) β-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. Patients and 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. Conclusion: 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.

AB - Purpose: Dermatofibrosarcoma protuberans (DFSP) is a rare, low-grade, cutaneous sarcoma with autocrine overproduction of the platelet-derived growth factor (PDGF) β-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. Patients and 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. Conclusion: 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.

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U2 - 10.1200/JCO.2005.02.5122

DO - 10.1200/JCO.2005.02.5122

M3 - Article

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AN - SCOPUS:32944457118

VL - 23

SP - 7669

EP - 7675

JO - Journal of Clinical Oncology

JF - Journal of Clinical Oncology

SN - 0732-183X

IS - 30

ER -