Prognosis of angiosarcoma at different anatomic sites Biing-Luen Lee, MD. Cheng-Feng Chen Division of Plastic and Reconstructive Surgery, Department of Surgery, Taipei Veterans General Hospital. Koo Foundation Sun Yat-Sen Cancer Center Taipei, Taiwan

Introduction Angiosarcomas are rare soft-tissue sarcomas of endothelial cell origin that have a poor prognosis Angiosarcomas are subdivided into cutaneous angiosarcoma, lymphoedema-associated angiosarcoma, radiation-induced angiosarcoma, primary-breast angiosarcoma, and soft-tissue angiosarcoma -Robin J Young et al. Review: Angiosarcoma. Lancet Oncol 2010; 11: 983–91

Treatment Surgery with wide excision as the main curative treatment option. Local control is improved with adjuvant radiotherapy. There is no compelling evidence for adjuvant chemotherapy -Robin J Young et al. Lancet Oncol 2010; 11: 983–91

Anatomic sites distribution - Bo-Wen Li et al, J Taiwan Soc of Plast Surg 2010;19:287∼300) -Robin J Young et al. Lancet Oncol 2010; 11: 983–91

-Matthew G. Fury et al, Cancer J 2005:11:241-247

Primary site in the liver, presence of metastases and performance status were correlated to poor overall survival, whereas soft tissue primary tumors independently correlated to better survival - J. Fayette et al, Annals of Oncology 18: 2030–2036, 2007

Material and Method Scalp and face Trunk Liver Retrospective chart review From 1996 to 2011, pathology reported angiosarcoma from Koo Foundation Sun Yat- Sen Cancer Center and Taipei veteran general hospital Angiosarcoma at: Scalp and face Trunk Liver

Patients with incomplete data, concurrent cancer were excluded Start time: since symptoms appeared Operation: tumor wide excision/ metastasectomy, excisional biopsies were not included

Results Total 44 patients 24 patients had angiosarcoma at scalp and face 9 at trunk (7 at breast, 1 at lower abdomen and 1 at left supra-scapula region) 11 at liver

Patient and tumor characteristics Scalp and face Trunk liver P value Sex <0.0001 Male 20 (83.3%) 0 (0%) 4 (36.4%) Female 4 (16.7%) 9 (100%) 7 (63.6%) Age (mean±SD) 76.58±9.353 42.33±7.794 68.36±12.948 Alcohol 5 (20.8%) 1 (11.1%) 2 (18.2%) 0.799 Smoking 8 (33.3%) 1 (9.1%) 0.062 HTN 12 (50%) 5 (45.5%) 0.024 DM 0.246 CAD 2 (8.3%) 1.000 Hepatitis 0.001 Hyperlipidemia 6 (25%) 2 (22.2%) Hyperuricemia 3 (12.5%) 0.643 TB 0.404

Scalp and face Trunk liver P value Focality 0.002 Solitary 16 (66.7%) 7 (77.8%) 1 (9.1%) Multifocal 8 (33.3%) 2 (22.2%) 10 (90.9) Size (mm) 59.25±77.790 82.44±57.267 59.32±60.063 0.278 Operation 19 (79.2%) 8 (88.9%) <0.0001 Stage operation 9 (37.5%) 6 (66.6%) 0 (0%) 0.003 Radiation Chemotherapy 12 (50%) 6 (66.7%) 5 (45.5%) 0.665 Distant mets 5 (55.6%) 4 (36.4%) 0.259 Lung mets 4 (44.4%) 2 (18.2%) 0.425 Liver mets 2 (8.3%) 3 (33.3%) 0.185 Bone mets 3 (12.5%) 11 (25%) 0.399 LN mets 7 (29.9%) 2 (18%) 0.212 5-year survival 8.3% 64.8% 0%

Overall survival Liver Scalp and face Trunk Kaplan–Meier method, Log rank P<0.0001

Cox regression forward stepwise Variables HR 95% CI of HR P value Lower Upper Location (Trunk vs. head) 0.040 0.009 0.190 <0.0001 Location (Liver vs. head) 6.890 2.261 20.998 0.001 Focality (Solitary vs. multifocal) 0.378 0.147 0.970 0.043 Operation 0.060 0.017 0.207 Chemotherapy 0.090 0.030 0.272 Lung mets 4.213 1.650 10.753 0.003 Liver mets 7.271 2.121 24.919 0.002 Variables: age, sex, location, focality, size, op, RT, chemo, dis met, lung met, liver met, bone met, LN mets

Cases received operation (Head vs. Trunk) Scalp and face (n=19) Trunk (n=8) P value Sex <0.0001 Male 16 (84.2%) 0 (0%) Female 3 (15.8%) 8 (100%) Age 76.21±9.537 41.25±7.573 Alcohol 4 (21.1%) 1 (12.5%) 1.000 Smoking 8 (33.3%) 0.055 HTN 11 (57.9%) 0.008 DM 2 (10.5%) CAD Hepatitis Hyperlipidemia 2(25.0%) Hyperuricemia 2 (25.0%) 0.558 TB

Scalp and face (n=19) Trunk (n=8) P value Focality 0.633 Solitary 13 (68.4%) 7 (85.7%) multifocal 6 (31.6%) 1 (12.5%) Size (mm) 39.58±36.34 90.25±55.869 0.014 Stage operation 0.529 Once 10 (52.6%) 2 (25%) More then twice 9 (48.4%) 6 (75%) Radiation 16 (84.2%) 7 (87.5%) 1.000 Chemotherapy 9 (47.4%) 6 (75.0%) 0.236 Margin status 0.419 R0 13 (72.2%) 8 (100%) R1 2 (11.1%) 0 (0%) R2 3 (16.7%) Local recurrence 15 (78.9%) 3 (37.5%) 0.398 Distant mets 16 (66.7%) 5 (62.5%) Lung mets 8 (42.1%) 4 (50.0%) Liver mets 2 (10.5%) 0.136 Bone mets 3 (15.8%) 0.227 LN mets Reconstruction 0.002 No 5 (71.4%) Skin graft 7 (38.9%) Flap 2 (28.4%) Flap and graft

Overall survival Scalp and face Trunk P=0.014

Recurrence-free and metastasis-free survival P=0.010 P=0.130

Results Scalp and face angiosarcomas are more common in old male patients, more difficult to reach free margin status, more likely to recur locally and metastasize distantly with poor prognosis Usually needs flaps or grafts for reconstruction

Trunk (breast) angiosarcomas are more common in younger women, usually solitary but bigger in size and easier to reach free margin status, with better prognosis and survival Patients with liver angiosarcomas often had multifocal lesions, less possible to receive operation with the worst prognosis

Discussion Represents clinical presentation and treatment of angiosarcoma cases in Taiwan All 7 cases of breast angiosarcomas in our series didn’t have radiation exposure history, and are primary-breast angiosarcomas More primary-breast angiosarcoma cases in Taiwan

Breast angiosarcoma cases at Mayo Clinic from 1960 to 2008 were identified - JEFFREY S. SCOW et al, Journal of Surgical Oncology 2010;101:401–407

Limitations Small sample size Incomplete patient information Lack of pathological data

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