International Journal of Surgery Case Reports 114 (2024) 109050

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International Journal of Surgery Case Reports

journal homepage: www.elsevier.com/locate/ijscr

Case report

Rare case of radiotherapy-induced angiosarcoma (RIAS) after conservative

and radical treatment of breast cancer: About two cases and review of
the literature
Ayoub Amghar *, Imane El Abbassi, Youness Benchrifi, Benhessou Mustapha,
Ennachit Mohammed, Elkerroumi Mohammed
Obstetrics And Gynecology Department, Univesity Hospital Center Ibn, Morocco

A R T I C L E I N F O A B S T R A C T

Keywords: Introduction and importance: Angiosarcoma of the breast is a rare malignant tumour of endothelial origin. It is
Breast angiosarcoma characterised by a high degree of malignancy and a polymorphous clinical and radiological presentation, a
Radiotherapies source of diagnostic error and delay. It has a very poor prognosis. Mammary angiosarcoma is a rare but
Radiotherapy-induced angiosarcoma
formidable complication of radiotherapy. The specificity of this observation is that we are presenting two clinical
RIAS
Breast sarcoma
cases of different surgical management of breast cancer who suffered the same complication from radiotherapy.
Surgery Case presentation: We report two cases of Radiotherapy-induced angiosarcoma (RIAS) in two patients with a
history of breast cancer one treated by conservative surgery and radiotherapy and the other by radical surgery
and radiotherapy both patients were operated.
Clinical discussion: Radiotherapy-induced angiosarcoma (RIAS) is a rare complication of radiotherapy. The
increasing use of conservative treatment of breast cancer, which combines surgery with radiotherapy and
chemotherapy, can rarely be complicated by breast sarcoma.
Conclusion: The natural history of radiation-induced angiosarcoma is more or less rapid, with death occurring in
the setting of metastatic spread after a median survival of 24 months. The quality of the surgical procedure is a
prognostic factor.

1. Introduction sarcomas are haematophilic rather than lymphophilic cancers. A high

Breast sarcomas are very rare tumours that express themselves with
different histological manifestations, they have two aspects either pri­
mary or secondary often to radiotherapy. In recent years, radiotherapy
for breast cancer after surgery is often used as women choose lumpec­
tomy and radiotherapy rather than mastectomy. The increased use of
radiotherapy may lead to an increased incidence of radiation-induced
breast angiosarcoma [1].
These have a poor prognosis due to the high risk of metastasis and the
absence of well-managed treatment. Optimal treatment of sarcomas is a
matter of debate. The therapeutic strategy is discussed by multidisci­
plinary staff at a referral centre for the management of sarcomas. The
current standard of treatment is surgery, with wide resection of healthy
margins, which is usually sufficient, without the need for systematic
axillary lymph node dissection, as axillary extension is rare, and
histological grade, involvement of the surgical margins and tumour size
>5 cm are factors with a poor prognosis. The role of adjuvant chemo­
therapy and radiotherapy remains to be defined in forms at high risk of
relapse [1]. the work has been reported in line with the SCARE criteria.
[2]
2. Clinical observation
2.1. 1st clinical case
patient aged 54, mother of 3 living children, not followed for any
disease and never operated on with no family history of cancer and not
carrying a chronic disease, she has been followed for 5 years for invasive
ductal carcinoma of the left breast for which she has had conservative
treatment: lumpectomy of the left upper external quadrant of a 2 cm

* Corresponding author at: Department of Gynecology and Obstetrics, University Hospital Center Ibn Rochd, Faculty of Medecine and Pharmacy, Hassan II
University, 1 rue des hôpitaux, Casablanca, Morocco.
E-mail address: ayoub.amghar@gmail.com (A. Amghar).

https://doi.org/10.1016/j.ijscr.2023.109050
Received 4 October 2023; Received in revised form 8 November 2023; Accepted 9 November 2023
Available online 21 November 2023
2210-2612/© 2023 Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd. This is an open access article under the CC BY-NC-ND license
(http://creativecommons.org/licenses/by-nc-nd/4.0/).
A. Amghar et al.
tumour with homolateral axillary dissection. Anatomopathological
study showed an invasive breast carcinoma, SBRIII (Modified Scarff,
Bloom and Richardson histo-pronostic grade) measuring 3.5x5x4cm,
which came into contact with the edges of the resection in situ, and for
which immunohistochemistry showed luminal B, Her2 negative, no
positive nodules were found in any of the 14 lymph nodes sampled
during axillary excision. The patient received 6 courses of chemotherapy
(3 EC 100 (anthracycline-taxane-capecitabine) and 3 docetaxel), com­
bined with radiotherapy (50 Gy over the mammary gland with 10 Gy
over the tumour bed). Anti-oestrogen hormone therapy was prescribed
for five years.
Ten years later, the patient presented with nodules on the lumpec­
tomy scar. Clinical examination (Fig. 1) revealed an indurated breast
with ulceration in the superolateral quadrant. A mammogram was
performed (Fig. 2) and biopsies were taken. Histological analysis
revealed a high-grade sarcoma, suggesting a high-grade leiomyo­
sarcoma at first. The extension work-up did not reveal any secondary
tumour locations. The patient received 5 courses of chemotherapy, then
her case was reviewed at the multidisciplinary consultation meeting,
where the decision was taken to perform a mastectomy. The mastectomy
was performed with healthy margins and the anatomopathological
analysis confirmed the diagnosis of breast angiosarcoma. The post-
operative course was straightforward.
2.2. 2nd clinical case
patient aged 68, married with 6 living children, type 2 diabetic on
insulin, never operated on, with no family history of cancer and no
chronic disease, She had been followed for 7 years for invasive breast
carcinoma of the left breast having undergone radical treatment for a 6
cm cancerous mass which consisted of mastectomy and axillary curage
with anatomopathological examination of the surgical specimen
showing invasive ductal carcinoma SBR II (Modified Scarff, Bloom and
Richardson histo-pronostic grade) Luminal A with two positive lymph
nodes on axillary curage out of 18 lymph nodes removed, The patient
received hormone therapy with anti-aromatase agents over 5 years and
55 Gy of radiotherapy to the mammary gland with 10 Gy of
overimpression.
6 years after the initial treatment, the patient was consulted about
the appearance of a skin tumour at the mastectomy scar. Clinical ex­
amination revealed indurated and pulsatile skin macules (Fig. 3). Breast
ultrasound revealed 3 limited, hypoechoic, heterogeneous poorly
limited opposite the mastectomy scar, with no detectable circumscribed
lesion, measuring 36 mm, 21 mm and 11 mm. The extension work-up
was negative, and the case was referred to the multidisciplinary
consultation meeting, where the decision was taken to perform a sur­
gical resection of the tumour bed with wide excision. Surgery was per­
formed with healthy margins and anatomopathological analysis
confirmed the diagnosis of breast angiosarcoma with healthy margins.
The post-operative course was straightforward (Fig. 4).
Fig. 1. Indurated breast with ulceration in the superolateral quadrant.
2
International Journal of Surgery Case Reports 114 (2024) 109050
3. Discussion
Mammary angiosarcoma is a rare malignant mesenchymal tumour
that develops in mammary vascular tissue. It accounts for 0.004 to 1 %
of all malignant tumours of the breast, and 8 to 10 % of breast sarcomas.
[3]
It may be primary in a young patient aged 40 or radiation-induced in
an elderly patient who has undergone conservative surgery and adjuvant
radiotherapy.
Secondary angiosarcoma mainly affects cutaneous tissue and occurs
in two distinct aetiopathogenic contexts:
• Chronic lymphoedema following radical surgical treatment of breast
cancer (mammectomy and axillary curage), first described in 1948
by Stewart and Treves.
• A history of radiotherapy after conservative treatment of breast
cancer, without associated lymphoedema. [4,5]
CAHAN's work led to the establishment of the following diagnostic
criteria [6].
a. history of breast irradiation.
b. A latency period of several years (average of 12 years)
c. Occurrence of sarcoma in the irradiated field
d. Histological confirmation of sarcomatous origin.
3.1. Clinical signs [7]
– Painless, vascular, pulsatile breast nodule or mass.
– Black, purplish colour.
– Between 2 and 11 cm long.
– Rapidly growing.
– Adenopathies are exceptional and only occur in very advanced
forms.
3.2. Imaging [8]
– On ultrasound, ASM appears as a hypoechoic, hypoechoic or mixed
image simulating a tissue mass, with a Doppler study confirming the
vascular origin.
– The contribution of CT is crucial; it shows a hypervascular mass
which is untimely enhanced at arterial time with partial homogeni­
sation at portal time. CT is particularly useful for searching for
distant metastases.
– MRI reveals a heterogeneous mass with haemorrhagic areas or
venous lakes in T1 hypersignal; the intensity of enhancement de­
pends on the histological grade. The interest of MRI can be summed
up in the evaluation of the local extension of the tumour, in partic­
ular the extension towards the underlying muscular plane.
A. Amghar et al. International Journal of Surgery Case Reports 114 (2024) 109050

Fig. 2. Asymmetrical breasts with a large, poorly defined mass behind the nipple, associated with macrocalcifications, architectural distortion and thickening of the
envelope without any lesion on the right breast.

Fig. 3. Mammary angiosarcoma on a mastectomy scar.

Fig. 4. ultrasound image of breast angiosarcoma on a mastectomy scar.

3.3. Histology [9]
The diagnosis of certainty is purely histological. Macroscopically,
angiosarcomas of breast behave like an aggressive locally infiltrating
tumour containing necrotic-haemorrhagic zones; microscopically, they
present structural anomalies depending on their stage of differentiation;
a well-differentiated angiosarcoma infiltrates the dermis, hypodermis or
parenchyma with neoformed vascular channels, which destroy the fibres
of the connective tissue without destroying the galactophoric channels
or the mammary lobes. Breast sarcomas in irradiated areas are most
often high-grade, with tumour necrosis and numerous mitoses. Other
histological factors include size, cellular pleiomorphism and stromal
atypia. Hormone receptor analysis by immunohistochemistry (IHC),
routinely performed in the management of breast cancer, can charac­
terise a phyllodes tumour, but without any real therapeutic impact, since
the prognosis depends on the stromal, and not the epithelial, compo­
nent. The identification of genetic differences between sporadic and
post-irradiation forms of angiosarcoma could make it possible to
distinguish these entities in terms of therapeutic targets; recent studies
have shown that amplification of the c-MYC oncogene is more frequent
in radiation-induced cases, characterised by a transcriptomic signature
of 135 genes [10,11].
3.4. Surgery: gold standard treatment for radiation-induced
angiosarcoma [12]
R0 resection should always be considered. Radical mastectomy is the
treatment of choice for this type of tumour, and axillary dissection is
only justified if there is lymph node involvement. Wide resection with
healthy margins of 2 to 3 cm confirmed by pathology is recommended.
This type of surgery, known as wide resection, raises the issue of skin
coverage (flaps/grafts, etc.).
3.5. Chemotherapy: neoadjuvant, initial or induction treatment
Objectives: to reduce the initial tumour volume in order to facilitate

3
A. Amghar et al.
operating time, to act early on subclinical metastases and, above all, to
test tumour chemosensitivity in vivo in order to better select patients
who may benefit from possible adjuvant chemotherapy [13].
3.6. Indications
– Size >8 or 10 cm.
– The tumour is smaller but has invaded vascular, nerve, joint or bone
structures.
– There is skin inflammation.
– In the event of local relapse.
– initial incomplete surgery with macroscopic tumour residue.
– initial multi-drug therapy (combination of doxorubicin +
ifosfamide).
3.7. Chemotherapy: adjuvant treatment
The aim of chemotherapy is to reduce the incidence of local recur­
rence and metastases, thereby improving recurrence-free survival and/
or overall survival.
It is not a standard treatment.
It is usually proposed for high-risk tumours (grade 2–3, >5 cm). It is
usually a combination of doxorubicin and ifosfamide (IA) at optimal
doses for 4 to 6 cycles. [14].
Adjuvant radiotherapy Its place is controversial, in the absence of
randomised prospective studies [15].
3.8. Prognosis
Staging of breast sarcomas is based on the AJCC (American Joint
Committee on Cancer) classification of MTS: (method which uses pri­
mary Tumour Size); the grade was included in the seventh edition
published in 2010 [16] Angiosarcoma has a poor prognosis, with a
median survival of 15 years for grade I, 12 years for grade II and only 15
months for grade III. [16] The natural history of radiation-induced
angiosarcoma is a more or less rapid progression to death, which oc­
curs in the setting of metastatic spread after a median survival of 24
months. The quality of the surgical procedure is a prognostic factor [16].
4. Conclusion
Angiosarcoma of the breast is a rare and heterogeneous malignancy.
It behaves aggressively and has a poor prognosis, with a 5-year overall
survival rate of 27–48 %.
Because of its rarity, most information about the diagnosis and
management of this disease comes from case reports and analyses of
retrospective series involving a relatively small number of patients [17].
Ethical approval
Ayoub Amghar declares on my honour that ethical approval has been
waived for my establishment by the competent ethics committee of the
Ibn Rochd University Hospital Centre competent in its field under
reference Doc.1389
Consent
Written informed consent was obtained from the patient for publi­
cation of this case report and accompanying images. A copy of the
written consent is available for review by the Editor-in-Chief of this
journal on request.
CRediT authorship contribution statement
Ayoub Amghar: Corresponding author writing the paper
International Journal of Surgery Case Reports 114 (2024) 109050
El Abbassi Imane : writing the paper
Youness Benchrifi: study concept
Ennachit Mohammed: study concept
Benhessou Mustapha: study concept
Elkerroumi Mohammed: correction of the paper
Registration of research studies
Research registry 2464.
Sources of funding
None.
Guarantor
Dr Amghar Ayoub.
Declaration of competing interest
The authors declare having no conflicts of interest for this article.
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