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JNMA I VOL 52 I NO. 8 I ISSUE 192 I OCT-DEC, 2013 634
Primary Cutaneous Angiosarcoma on the Nose in a Patient with Multiple
Nonmelanoma Skin Cancers
Kayhan Basak,1 Pinar Yuksel Basak,2 Hakan Demirel,3 Nimet Karadayi1
1Department of Pathology, Dr. Lutfi Kirdar Kartal Educational and Research Hospital, 2Department of Dermatology, Suleyman
Demirel University, Faculty of Medicine, 3Department of Plastic and Reconstructif Surgery, Dr. Lutfi Kirdar Kartal Educational
and Research Hospital, Turkey.
ABSTRACT
Cutaneous angiosarcoma is an uncommon, potentially metastatic and highly aggressive vascular
tumor that may arise as de novo or be associated with previous radiotherapy. A 70-year-old female
with a solitary lesion on the nose was initially diagnosed as actinic keratosis. However, when the
recurring lesion at the same region within 6 months was reexcised, the histological diagnosis was
defi nitively established as well-differentiated angiosarcoma. This case was presented in order to
increase awareness of this rare malignancy in an uncommon localisation, especially in the light
of pathological fi ndings. Moreover, occurrence of cutaneous angiosarcoma within a short period
following a previous excision in a patient with a history of multiple nonmelanoma skin cancers was
interestingly pointed out.
_______________________________________________________________________________________
Keywords: angiosarcoma; face; nonmelanoma skin cancers.
_______________________________________________________________________________________
______________________________________
Correspondence: Dr. Pinar Yuksel Basak, Suleyman Demi-
rel Universitesi Tip Fakultesi Dermatoloji AD, Isparta, Turkey. E-mail:
pinarbasak@hotmail.com, Phone: +90 246 211 9313.
INTRODUCTION
Cutaneous angiosarcoma of the head and neck is
mostly localised at the scalp of elderly men with poor
prognosis.1,2 Although a defi nite diagnosis can be
delayed because of the rarity doubled with complex
and diffi cult pathological identifi cation of this highly
malignant soft-tissue cancer, early detection and
immediate treatment are essential.2
CASE REPORT
A 70-year-old Caucasian female was presented with
a plaque on the nose. She was learnt to be operated
numerous times for different individual lesions on the
face in the last 30 years. Pathological diagnoses of
right infraorbital epidermoid carcinoma 10 years ago,
left infraorbital basal cell carcinoma nine years ago, and
left infraorbital keratoacantoma and basosquamous cell
carcinoma on the forehead eight years ago were noted.
She gave no history of previous radiotherapy.
Dermatological examination revealed an ill-defi ned,
erythematous plaque on the right nasal dorsum. Focal
thinning of the rete ridges in hyperkeratotic epidermis,
enlargement, hypercromasia, atypia and loss of polarity
of the keratinocytes neighboring basal layer focally
were detected histopathologically. Solar elastosis
and superfi cial perivascular mononuclear lymphocytic
infi ltration in dermis were additional fi ndings consistent
CASE REPORT J Nepal Med Assoc 2013;52(192):634-6
JNMA I VOL 52 I NO. 8 I ISSUE 192 I OCT-DEC, 2013 635
with actinic keratosis.
The patient was represented with an enlarging purple
nodule at the same region six months later. On physical
examination, a 1.5 cm painless, violaceous nodule was
scattered (Figure 1). She had Fitzpatrick type-1 skin with
multiple lentigines on the face. Histological examination
of the totally excised lesion revealed anastomosis of
bizarre vascular spaces lined with atypical pleomorphic
endothel-like cells forming a tumoral structure in an
irregularly bordered area in dermis (Figure 2a,b). Low
mitotic counts (0-5 mitoses per 10 high-power fi elds)
and intense lymphocytic infi ltrate were observed.
Immunohistochemically, tumor cells stained strongly by
CD31, CD34 and factor VIII, and high index of Ki67
were detected. The fi nal diagnosis was considered to
be well-differentiated primary cutaneous angiosarcoma.
The initial pathologic specimens were reexamined
which lacked nuclear pleomorphism and thus, was not
found to be related to angiosarcoma.
Figure 1. Close up view of the violaceous fl eshy no-
dular lesion on the nose.
Results of the basic metabolic profi le, chest X-ray,
computed tomography of the neck and chest
revealed no abnormal fi ndings. Following re-excision
and reconstruction, the patient was given adjuvant
radiotherapy, and she has been surviving for 15 months
without any signs of metastasis.
Figure 2. a) Bilobulated nodular tumour mass in der-
mis signed between black arrowhead, invaded to
subcutaneous adipous tissue (whole section, original
magnifi cation H&E, X 40).
b) Irregular vascular channels lined by atypical endot-
helial cells (H&E, X 400).
DISCUSSION
Angiosarcoma of the head and neck is a rare tumor of
vascular origin originating from endothelial cells primarly
localised to scalp, face and neck1,3,4 and uncommonly
reported at periorbital region and sino-nasal cavities.5,6
In women, angiosarcoma was mostly located to the
scalp as ulcerated lesions.4 The nose is a rare site of
presentation and a few cases of angiosarcoma reported
on the nose were male patients.4,7,8 In a series of 14
patients reported by Morrison1 and of three cases by
Hanke et al8 only one female in each was presented
with angiosarcoma on the nose. Therefore, the nodular
angiosarcoma in a female on the nose was a seldom
site reported to date.
Possible relevance of predisposing factors including
history of irradiation, local trauma, insecticides, thorium
dioxide contrast and vascular malformations were
reported in etiology of angiosarcoma.1,4,9 Because these
tumors are more frequent in Caucasians, sun exposure
has also been implicated in its etiology.1,3,4 In contrast,
some authors reported low rate of skin carcinomas in
patients with angiosarcoma.9 However, to the best of
our knowledge, any case of cutaneous angiosarcoma
on the face with multiple nonmelanoma skin cancers
and actinic keratosis was not reported. The short period
between the two excised lesions alerted us about the
possible coincidental association of angiosarcoma and
actinic keratosis in the presented case. Nevertheless,
lack of histopathological fi ndings of angiosarcoma in
the initial specimen and subsequent occurrence of the
nodular lesion diagnosed as angiosarcoma after the
Basak et al. Primary cutaneous angiosarcoma on the nose in a patient with multiple nonmelanoma skin cancers
JNMA I VOL 52 I NO. 8 I ISSUE 192 I OCT-DEC, 2013 636
previous excision made us consider the effect of local
trauma due to the operation.
Variable clinical manifestations of cutaneous
angiosarcoma include asymptomatic bruise-like
lesions, purple nodules, chronic edema or ulcers.4
Enlarging nodular lesion of 5 cm was mostly reported3
while ulceration was found to be the most common
presentation in another series.9 Several benign
conditions such as rosacea, pyogenic granuloma,
erysipelas, contact dermatitis and angioneurotic oedema
might be mistaken.4,5,7 Arteriovenous malformations,
Kaposi’s sarcoma and nodular melanoma should also
be considered in differential diagnosis.7 Therefore,
histological examination is of crucial importance and
diffi culty in pathological assessment may lead to
delay in diagnosis.4 Angiosarcoma contrasts pyogenic
granuloma with nuclear pleomorphism by dissecting
through connective tissue to creat irregular vascular
spaces. Kaposi’s sarcoma contains cellular zones of
spindle cells forming slit-like spaces while anastomosis
of vascular spaces lined with atypical cells was
diagnostic of angiosarcoma as was the case.2 Sharing
certain histological components, angiosarcoma may be
differentiated with positivity of CD31 and factor VIII
from nodular melanoma and hemangiopericytoma.
Five year survival was reported to be 10-30%
in patients with angiosarcoma.3,4,10 Although the
only signifi cant prognostic factor was supposed as
histological grade of tumor in a series,3 the results
of most series have indicated the only expressive
correlation for tumor size with the survival. Mostly
worse prognosis was detected for bleeding and painful
lesions larger than 5 cm.4,9 In contrast to the fi nding of
predominance of poor differentiation in nodular lesions,4
our case presented with a nodular lesion was a well-
differentiated angiosarcoma. Prominent lymphocytic
infi ltrate was suggested to have a more encouraging
outcome7 while only mitotic counts were found to be
signifi cant for prognosis by Naka et al.10 Mild degree
of mitotic counts, intense lymphocytic infi ltrate in this
asymptomatic, small tumor were the factors promising
a better survival of the presented patient. Moreover,
improved survival in patients with central facial
localisation was suggested. Three patients presenting
with nasal angiosarcoma survived 4 to 7 years.8 The
female case of angiosarcoma on the nose reported in
the series of Morrison et al1 was alive 10 years after
diagnosis.
In conclusion, early suspicion and prompt histological
study of an enlarging nodular lesion on the nose in elderly
should be crucial keeping the diagnosis of angiosarcoma
in mind. The presented case of angiosarcoma in a
patient with multiple nonmelanoma skin cancers and
actinic keratosis raised the possibility of actinic damage
to fair skin might be suggested in etiology.
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Peters LJ. Cutaneous angiosarcoma of the head and neck. A
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Ikeda MK, Kowalski LP. Cutaneous angiosarcoma of the
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Basak et al. Primary cutaneous angiosarcoma on the nose in a patient with multiple nonmelanoma skin cancers
