77: Current Patterns of Care and Outcomes for Dermatofibrosarcoma Protuberans: An International Multi-institutional Collaborative

Thursday, March 23, 2023

11:26 AM – 11:36 AM

Location: Ballroom A

Abstract Presenter(s)

Leah K. Winer, MD

Complex General Surgical Oncology Fellow
Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, USA
Philadelphia, Pennsylvania, United States

Disclosure(s): No financial relationships to disclose

First Author(s)

Leah K. Winer, MD

Complex General Surgical Oncology Fellow
Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, USA
Philadelphia, Pennsylvania, United States

Disclosure(s): No financial relationships to disclose

Author(s)

RA

Rita Akumuo, MD

General Surgery Resident
Department of General Surgery, Temple University Hospital, Philadelphia, USA, United States

Disclosure information not submitted.
EH

Elizabeth Handorf, PhD

Associate Professor
Division of Biostatistics and Bioinformatics, Fox Chase Cancer Center, Philadelphia, USA, United States

Disclosure information not submitted.
Stephanie Greco, MD, FACS

Assistant Professor
Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, USA, Pennsylvania, United States

Disclosure information not submitted.
Alex Bartholomew, MD (he/him/his)

General Surgery Resident
Duke University Medical Center, North Carolina, United States

Disclosure information not submitted.
Dan Blazer, III, MD

Associate Professor of Surgery
Duke University Medical Center
Durham, North Carolina, United States

Disclosure(s): No financial relationships to disclose
LG

Lucie Gabrielova, MD

Assistant Professor
Department of Surgery, Masaryk Memorial Cancer Institute, Brno, Zluty kopec, Czech Republic, United States

Disclosure information not submitted.
DK

Dagmar A. Krakorova, MD, PhD

Assistant Professor
Department of Surgery, Masaryk Memorial Cancer Institute, Brno, Zluty kopec, Czech Republic, United States

Disclosure information not submitted.
EB

Ericka Bernard-Bedard, n/a

Assistant Professor
Division of Surgical Oncology, Ottawa Hospital, Ottawa, Canada, United States

Disclosure information not submitted.
Carolyn Nessim, MD, MSc, FRCSC, FACS (she/her/hers)

Surgical Oncologist, Associate Professor, Clinician Investigator, CGSO Program Director
The Ottawa Hospital, University of Ottawa, Ottawa Hospital Research Institute
Ottawa, Ontario, Canada

Disclosure(s): No financial relationships to disclose
AK

Attila Kollár, MD

Consultant
Department of Medical Oncology, Inselspital University Hospital Bern, Freiburgstrasse, Switzerland, United States

Disclosure information not submitted.
RO

Radu Olariu, MD PhD

Deputy Head of Department
Department of Plastic and Hand Surgery, Inselspital University Hospital Bern, Freiburgstrasse, Switzerland, United States

Disclosure information not submitted.
RC

Romi Cencelj-Arnez, MD

Surgical Oncologist
Institute of Oncology, United States

Disclosure information not submitted.
DH

Daphne Hompes, MD, PhD

Professor
Department of Surgical Oncology, University Hospitals Leuven, Leuven, Belgium, United States

Disclosure information not submitted.
SF

Sam Ford, n/a

Surgical Oncologist
University Hospital Birmingham NHS Foundation Trust, United States

Disclosure information not submitted.
Kenneth Cardona, MD

Patricia R. Reynolds Professor of Sarcoma, Associate Chief of Surgery EUH Midtown; Lead, Sarcoma Working Group, Winship Cancer Institute
Winship Cancer Institute, Emory University
Atlanta, GA, United States

Disclosure information not submitted.
KS

Kenji Sato, MD

Assistant Professor
Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan, United States

Disclosure information not submitted.
SI

Shintaro Iwata, MD

Assistant Professor
Department of Musculoskeletal Oncology, National Cancer Center Hospital, Tokyo, Japan, United States

Disclosure information not submitted.
Jeffrey M. Farma, MD

Interim Chair of Surgery, Professor of Surgical Oncology
Fox Chase Cancer Center
Philadelphia, Pennsylvania, United States

Disclosure information not submitted.
AV

Anthony M. VIllano, MD

Clinical Instructor
Division of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, USA, United States

Disclosure information not submitted.

Disclosure(s):

Leah K. Winer, MD: No financial relationships to disclose

Dan Blazer, MD, III: No financial relationships to disclose

Carolyn Nessim, MD, MSc, FRCSC, FACS: No financial relationships to disclose

Introduction: Dermatofibrosarcoma protuberans (DFSP) is a cutaneous sarcoma with an infiltrative growth pattern that renders resection challenging. Data regarding DFSP natural history, management, and outcomes are limited because of its rarity.

Methods: Data were retrospectively collected for adult patients with DFSP who underwent resection at 10 institutions in 8 countries. Patient demographics, tumor characteristics, treatment strategies, and outcomes were analyzed using descriptive statistics.

Results: Analysis included 281 patients consisting of mostly young (median 42 years), white (79.6%), males (55.4%) with truncal lesions (51.8%). The majority (70.3%) were symptomatic at presentation. Preoperative imaging was utilized in 55.8% of cases. Diagnosis was established via excisional biopsy in 53% of cases versus incisional biopsy in 28.4%. The median time to resection from diagnosis was 52 days. Despite planned margins of >1.0 cm in 65% of cases, the median first pathologic margin was 2.0mm (IQR 0.18-7.0mm), and 68.5% were R0 resections. Fibrosarcomatous transformation (FST) was observed on final pathology in 10.9% of specimens. Clavien-Dindo grade III or higher complications were reported in 18.9% of patients.

Nearly one-third of patients underwent >1 additional re-excision, most commonly to planned margins of 0.6-1cm (47.1%) or 1.1-2cm (38.8%). R0 resection was achieved at a second procedure in 77.5% and a third procedure in 84.8%, leaving 1.8% (n=5) with a persistent R1 margin (Table). Multimodal therapy was used sparingly: 10.2% of patients underwent radiation, and 1.5% underwent systemic therapy. In total, 6.8% (n=19) recurred (14 local, 5 distant) at a median of 13.6 months after index resection; the majority (89.5%) underwent additional resection. Of the five distant recurrences, 80% had FST, whereas only 21.4% of local recurrences were associated with FST. At a median follow-up of 55.5 months, disease-specific survival was 93.5%.

Conclusions: In this international, multicenter collaborative, surgical management of DFSP achieved favorable recurrence rates and survival. Multiple excisions to obtain clear margins remain commonplace and can inform future research efforts to optimize margin selection.

Learning Objectives:

Characterize the typical presentation of Dermatofibrosarcoma Protuberans.
Understand the heterogeneity of approaches to diagnosing and treating Dermatofibrosarcoma Protuberans.
Appreciate the complexity of surgical management and the challenges of achieving an R0 resection.