Angiosarcoma Following Radiation Therapy for Breast Cancer: Case Presentation and Clinical Management Considerations
184
with localized disease and 23% in patients with metas-
tatic disease (p < 0.001) [6]. Univariate analysis in a
separate series revealed a statistically significant differ-
ence (p< 0.001) in disease-specific survival (DSS) be-
tween patients with advanced, unresectable AS at cuta-
neous (median DSS = 10.3 mo) an d deep soft tissue sites
(median DSS = 2.8 mo) [7]. For patients with localized
or advanced disease, another series demonstrated im-
proved overall survival for patients with superficial (3.6
± 1.0 yrs) versus deep (2.3 ± 0.5 yrs) tumors [2]. In pa-
tients with localized cutaneous disease, primary tumor
origin in the head/n eck, extremities or breast was associ-
ated with significantly longer survival than tumors aris-
ing in the trunk (median survival: 3.6 ± 0.5 yrs vs. 2.1 ±
0.2 yrs; p = 0.01) [2]. Among patients with primary
breast AS, the presence of preexisting lymphedema and
radiation fields were negative prognostic indicators for
local and distal disease recurrence and overall survival
[2,7]. Several studies have failed to find a significant as-
sociation between microscopically positive surgical mar-
gins and poor disease outcomes [2,13]. Furthermore, the
role of adjuvant and neoadjuvant chemoradiation for pa-
tients with resectable AS is unclear. In our patient, we
observed favorable histologic results using neoadjuvant
irradiation. In two case series, adjuvant radiotherapy pro-
vided no survival benefit in AS patients irrespective of
tumor margin status [2,6]. Trials of adjuvant chemother-
apy using paclitaxel-based regimens have not shown a
survival advantage over more widely used doxorubicin-
based therapies [2,6]. Paclitaxel may, however, may be
of greater benefit than doxorubicin in patients with ad-
vanced, unresectable AS [13].
5. Recommendations
Angiosarcoma is incompletely understood and is gener-
ally very aggressive. Unfortunately, AS is likely to in-
crease in incidence. With the appropriate pathological
analyses it is possible 1) to make an early diagnosis, 2) to
monitor the tumor for treatment efficacy, and 3) to sur-
vey for recurrence. Interestingly our patient’s malign-
nancy was positive for p63, which alters VEGF expres-
sion [17] and is identified in approximately 22% of an-
giosarcomas. However, this marker is somewhat non-
specific and its presence should be carefully interpreted.
In contrast, the identification of establish ed markers such
as CD34 may yield more helpful prognostic and surveil-
lance information [18]. Based on clinical and pathologic
characteristics, we used a staged, multidisciplinary ap-
proach to post-mastectomy reconstruction, which favors
definitive reconstruction only after appropriate time for
adjuvant treatment and follow-up to rule-out disease re-
currence has occurred. Unfortunately, our patient did
have local recurrence following the first-stage of treat-
ment.
REFERENCES
[1] R. J. Young, N. J. Brown, M. W. Reed, D. Hughes and P.
J. Woll, “Angiosa rcoma,” Lancet Oncology, Vol. 11, No.
10, 2010, pp. 983-991.
http://dx.doi.org/10.1016/S1470-2045(10)70023-1
[2] M. G. Fury, C. R. Antonescu, K. J. Van Zee, M. F.
Brennan and R. G. Maki, “A 14-Year Retrospective Re-
view of Angiosarcoma: Clinical Characteristics, Prognos-
tic Factors, and Treatment Outcomes with Surgery and
Chemotherapy,” Cancer Journal, Vol. 11, No. 3, 2005,
pp. 241-247.
http://dx.doi.org/10.1097/00130404-200505000-00011
[3] C. Leowardi, U. Hinz, Y. Hormann, M. N. Wente, G.
Mechtersheimer, F. Willeke, et al., “Malignant Vascular
Tumors: Clinical Presentati on, Surgical Therapy, and Long-
Term Prognosis,” Annals of Surgical Oncology, Vol. 12,
No. 12, 2005, pp. 1090-1101.
http://dx.doi.org/10.1245/ASO.2005.09.002
[4] J. R. Toro, L. B. Travis, H. J. Wu, K. Zhu, C. D. M.
Fletcher and S. S. Devesa, “Incidence Patterns of Soft
Tissue Sarcomas, Regardless of Primary Site, in the Sur-
veillance, Epidemiology, and End Results Program, 1978-
2001: An Analysis of 26,758 Cases,” International Jour-
nal of Cancer, Vol. 119, No. 12, 2006, pp. 2922-2930.
http://dx.doi.org/10.1002/ijc.22239
[5] P. Rouhani, D. M. C. Fletcher, S. S. Devesa and J. R.
Toro, “Cutaneous Soft Tissue Sarcoma Incidence Patterns
in the U.S.: An Analysis of 12,144 Cases,” Cancer, Vol.
113, No. 3, 2008, pp. 616-627.
http://dx.doi.org/10.1002/cncr.23571
[6] J. Fayette, E. Martin, S. Piperno-Neumann, A. Le Cesne,
C. Robert, S. Bonvalot, et al., “Angiosarcomas, a Het-
erogenous Group of Sarcomas with Specific Behaviors
Depending on Primary Site: A Retrospective Study of
161 Cases,” Annals of Oncology, Vol. 18, No. 12, 2007,
pp. 2030-2036.
http://dx.doi.org/10.1093/annonc/mdm381
[7] J. A. Abraham, F. J. Hornicek, A. M. Kaufman, D. C.
Harmon, D. S. Springfield, K. A. Raskin, et al., “Treat-
ment and Outcome of 82 Patients with Angiosarcoma,”
Annals of Surgical Oncology, Vol. 14, No. 6, 2007, pp.
1953-1967. http://dx.doi.org/10.1245/s10434-006-9335-y
[8] S. T. Schindera, M. Streit, U. Kaelin, E. Stauffer, L.
Steinbach and S. E. Anderson, “Stewart-Treves Syn-
drome: MR Imaging of a Postmastectomy Upper-Limb
Chronic Lymphedema with Angiosarcoma,” Skeletal Ra-
diology, Vol. 34, No. 3, 2005, pp. 156-160.
http://dx.doi.org/10.1007/s00256-004-0807-5
[9] A. Abu-Zaid and S. Mohammed, “Primary Pleural An-
giosarcoma in a 63-Year-Old Gentleman,” Case Reports
in Pulmonology, Vol. 2013, 2013, Article ID: 974567.
[10] I. W. Mattoch, J. B. Robbins, R. L. Kempson and S.
Kohler, “Post-Radiotherapy Vascular Proliferations in
Mammary Skin : A Clinicopathologic Study of 11 Cases,”
Journal of the American Academy of Dermatology, Vol.
57, No. 1, 2007, pp. 126-133.
http://dx.doi.org/10.1016/j.jaad.2006.10.025
[11] C. Gengler, J. M. Coindre, A. Leroux, M. Trassard, D.
Ranchère-Vince, I. Valo, J. J. Michels and L. Guillou,
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