Breast
.jpg "Joel Shapiro, MD, PhD photo")
Joel Shapiro, MD, PhD
Surgical Oncology Fellow
Department of Surgery, University of Toronto, Toronto, ON, Canada
Toronto, Ontario, Canada
Joel Shapiro, MD, PhD
Surgical Oncology Fellow
Department of Surgery, University of Toronto, Toronto, ON, Canada
Toronto, Ontario, Canada
Joel Shapiro, MD, PhD
Surgical Oncology Fellow
Department of Surgery, University of Toronto, Toronto, ON, Canada
Toronto, Ontario, Canada
Harini Suraweera, MS
Data manager
Department of Surgery, University of Toronto, Toronto, ON, Canada, United States
David McCready, MSc, MD
Surgeon
Department of Surgery, University of Toronto, Toronto, ON, Canada, United States
Geoffrey Watson, BSc, MSc, MuDr
Medical Oncologist
Department of Medical Oncology, University of Toronto, Toronto, ON, Canada, United States
David Shultz, MD, PhD
Radiation Oncologist
Department of Radiation Oncology, University of Toronto, Toronto, ON, Canada, United States
Anne O'Neill, MBBCh, PhD
Plastic Surgeon
Department of Plastic Surgery, University of Toronto, Toronto, ON, Canada, United States
Miralem Mrkonjic, MD, PhD
Pathologist
Department of Pathology, University of Toronto, Toronto, ON, Canada, United States
Rebecca Gladdy, MD, PhD
Surgeon
Department of Surgery, University of Toronto, Toronto, ON, Canada, United States
Andrea Covelli, MD, PhD
Surgeon
Department of Surgery, University of Toronto, Toronto, ON, Canada, United States
Sarcomas of the breast (SB) are a relatively rare and heterogeneous group of tumors arising from the non-glandular connective tissues of the breast. SB accounts for < 1% of all breast malignancies and < 5% of all soft tissue sarcomas. Historically, SB has been treated by both breast cancer and sarcoma teams. However, as their biology may not be fully appreciated, SB management has been controversial. Here we present our institutional experience with a histology-based multidisciplinary approach of primary SB.
Methods:
All patients with primary SB treated at our institution between 2003 and 2022 were identified from a prospectively collected database and were included in the present study. Phyllodes tumors and metastatic sarcomas to the breast were excluded. Overall survival (OS) was estimated by Kaplan-Meier.
Results:
A total of 93 patients were identified, 91 (98%) were female, median age at diagnosis was 63.7 years (range 15.0 – 90.1), common types were angiosarcoma, 68 (73%), desmoid, 9 (10%), dermatofibrosarcoma protuberans (DFSP), 5 (5%) and other histologies, 11 (12%). Median tumor size at diagnosis was 60 mm for the whole group. Tumor multifocality was found in 56 patients (60%), with a clear predominance in the angiosarcoma group (53 patients; 95%). 62 patients (67%) received prior radiotherapy (RT) to the chest of which 59 (95%) had an angiosarcoma. Other RT-associated types were epithelioid hemangioendothelioma, MPNST and myxofibrosarcoma. Median time from RT-to-sarcoma diagnosis was 8.8 years (range: 3.2 to 20.4).
Initial sarcoma treatment was resection in 65 patients (70%), multimodality treatment in 24 (26%), systemic treatment only in 2 (2%) and watchful waiting in 2 (2%). In total, 90 patients (97%) underwent surgery during treatment, 62 (68%) at our institute and 28 (32%) at outside hospitals, prior to presenting to us. Margins were clear in 84 patients (94%). Further details are summarized in Table 1.
Median length of follow-up was 50.0 months. Overall, 43 patients (46%) developed disease recurrence, 32 (35%) locoregional only and 11 (12%) distant. Median time to recurrence was 14.1 months (range 2.3 to 96.0). In total, 30 patients (32%) died of their disease, while 4 (4%) died of other causes. In this series the 5-year OS was 67%.
Conclusions:
Primary SB is relatively rare, even at high-volume referral sarcoma centers. The incidence of SB is higher in patients post-RT. Treatment for SB requires a histology-based approach and multi-institutional collaborative efforts are needed to further improve treatment outcomes in these rare tumor types.