Morphology

Angiosarcoma of the scalp

Marıa del Mar Saez de Ocariz, MD, Fernando de la Barreda, MD, and Leticia BoetaAngeles, MD

From the Dermatology Department, An 82-year-old woman was seen at our Dermatology Department for a plaque on the rightHospital General ‘‘Dr Manuel Gea parietal scalp that had recently increased in size, and bled. The lesion had been presentGonzalez,’’ and Dermatology Service, for 3 months. The patient had a previous diagnosis of chronic bronchitis, noninsulin-Instituto Nacional de Cancerologıa,

dependent diabetes mellitus, and hypertension, but no previous history of cancer.MexicoPhysical examination revealed a 7 3 10 cm plaque, composed of a central necrotic and

Correspondence bleeding surface, surrounded by small purple–red satellite nodules (Fig. 1). A biopsyMarıa del Mar Saez de Ocariz, MD showed an ill-defined infiltrative intradermal mass with a pattern of hypercellular sheets ofDermatology Department large cells alternating with areas of dilated, irregular, blood-filled channels, dissecting theHospital General ‘‘Dr Manuel Gea

collagen bundles. The endothelial cells lining these channels were plump andGonzalez’’pleomorphic, surrounded by other spindle-shaped cells with pleomorphic and atypicalCalzada de Tlalpan 4800

Mexico 14000 nuclei (Fig. 2). The diagnosis of angiosarcoma was made, and the patient was sent to an

oncology center for further evaluation and treatment, where a computed tomography head

scan was taken revealing no erosion of the skull. The patient refused surgery, so

radiotherapy was proposed. One month later, she developed lymph node enlargement of

the left anterior cervical nodes. A needle aspiration biopsy was consistent with sarcoma.

Two weeks later, she was started on palliative radiotherapy: a programmed dose of

4500 cGy was proposed of which she only received 3000 cGy because of treatment

withdrawal and loss to follow-up. During this time, she showed partial initial response, but

despite treatment the disease relentlessly progressed, with hemorrhage and severe pain

being the most striking features (Fig. 3).

Discussion

Angiosarcomas are highly aggressive mesenchymal tumors

with elements of vascular differentiation. They may occur

in any region of the body, but most frequently on the head

and neck.1,2 Angiosarcomas are usually found on the

scalp in the elderly population, and they behave as an

aggressive tumor.

Angiosarcoma generally presents as an erythematous

macule or plaque with irregular borders that may be

associated with small adjacent nodules. The lesion may be

very large and varies from red to violet. The clinical

recognition, as in our patient, is easy with this classical

presentation. In the initial phases, however, the appearance

may be quite benign and confused with bruises or hemangi-

omas.1–4

Angiosarcoma of the scalp has a progressive malignant

behavior. This tumor expands with gradual centrifugal

infiltration, grows rapidly, and has the highest rate of

lymph node metastases of all soft tissue sarcomas of the

head and neck.5 It may metastasize to cervical nodes and

lungs. Extensive local spread is the rule, and the prognosis

is dismal, with few patients surviving longer than

3 years.1,4,6 A better prognosis is indicated if the tumor is

© 1999 Blackwell Science Ltd International Journal of Dermatology 1999, 38, 697–699

697

smaller, and there is a more intense lymphocytic infiltrate

around the tumor site.7,8

Angiosarcomas of the scalp are often multifocal lesions,

whether or not this is clinically evident. Therefore, because

of the multifocality and inapparent spread, the optimal

surgical management and establishment of surgical margins

have not been defined.4 Results with surgery alone have

been disappointing, with high rates of recurrence.4,9 Extens-

ive involvement makes surgical removal impractical, and

other options must be kept in mind. Mohs’ surgery has

been tried in some instances, but the overall results are no

better than those of the other reported modalities of

treatment, and it should be used only as a tissue sparing

method.10

Radiotherapy has been considered as a rational treatment

modality for angiosarcoma, as a wide region of the dermis

can be treated and the underlying tissues can be spared.9

Furthermore, there have been some reports of survival for

longer than 2 years after radiotherapy alone.11 Even though

there is still not enough experience with radiotherapy, it

has been offered both as a curative and as a palliative

measure, as in our case. Chemotherapy alone has not

proven to be useful in the treatment of angiosarcomas.

698 Morphology Angiosarcoma of the scalp del Mar Saez de Ocariz, de la Barreda, and Angeles

Figure 1 Right parietal plaque, with a necrotic center and a

bleeding surface

Figure 2 Histology shows an ill-

defined intradermal mass, with

pleomorphic and atypical cells

dissecting collagen bundles, as well as

some irregular blood channels

Nowadays, surgery combined with postoperative radio-

therapy offers the best chance of long-term survival.11,12

Conventional surgery alone has been abandoned as the

treatment for cutaneous angiosarcomas, and the trend is

to the administration of wide field radiation after limited

surgical resections.11–13 Overall results have not been

encouraging, but some authors are convinced that radio-

therapy offers the only real chance of obtaining adequate

local control even if surgical excision is carried out.9 The

addition of chemotherapy might be of value for treatment

in some cases.4

Due to its aggressive behavior, the early recognition of

angiosarcoma of the scalp, and complete surgical excision,

is the best approach to increase survival in these patients.

International Journal of Dermatology 1999, 38, 697–699 © 1999 Blackwell Science Ltd

Figure 3 Spread of the neoplasm: a large black plaque with a

central ulcer involving three-quarters of the scalp with

extension to the face, particularly the right eye

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From the collection of Lawrence Charles Parish MD, Philadelphia, Pennsylvania.

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