ANGIOSARCOMA OF THE HEAD AND NECK

By Gary M. White, MD

Angiosarcoma of the head and neck This older woman's upper face and scalp was infiltrated with angiosarcoma. Note the contrast between the normal-colored skin on the right cheek and nose with the left cheek and forehead.


Angiosarcoma of the head and neck is an often fatal tumor primarily of adult men. Prior radiation may predispose.

Clinical

A bruise-like lesion which may develop vascular papules, nodules, or plaques in an elderly patient is characteristic. Ulceration and bleeding are common. This highly aggressive vascular tumor may eventuate in death from local invasion or distant metastases. Other less common presentations include a rosacea-like eruption, angioedema, a scarring alopecia, and skin-colored papules.

Treatment

Treatment is often difficult. Mortality is high. Wide surgical excision at an early stage is the best hope for cure. Mohs surgery has been used. Chemotherapy combined with radiotherapy has also been used.

Propranolol

In an intriguing report [JAMA Dermatol 2015;151;1226 and Sep 16 ahead of print], a patient with a β-adrenergic-positive multifocal stage T2 cutaneous angiosarcoma (≥20 cm) involving 80% of the scalp, left forehead, and left cheek, with no evidence of metastasis responded to propranolol 40 mg twice a day alone initially. Subsequently, propranolol 40 mg 3 times a day was combined with paclitaxel poliglumex, 2 mg/m2 infused weekly, and radiotherapy. During the subsequent 8 months, the tumor regressed with no detectable metastases. The authors suggest that β-blockade alone substantially reduced angiosarcoma proliferation and, in combination with standard therapy, is effective for reducing the size of the tumor and preventing metastases.

Miscellaneous

Pazopanib treatment was helpful in one patient [JAAD 2014 Jan]. Protracted intralesional interleukin-2 therapy induced complete remission in another [BJD 2015;172;156].

Prognosis

Prognosis is often poor. Some have suggested the following as poor prognostic signs:

Additional Pictures

Angiosarcoma of the head and neck

References

Scalp Tumor. Indian Dermatol Online J 2016;7:64-5

An 80-year-old man. Indian J Dermatol 2014;59:632

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