Angiosarcoma of the head and face skinC44.-

Rare, highly malignant and aggressively growing vascular tumour in the sun-damaged scalp of elderly people. It is characterized by rapid and often uncontrollable growth with a very poor prognosis.

m>w; usually 60th to 80th year of life. Angiosarcomas account for about 0.01% of all malignant tumors of the adult and 5% of cutaneous sarcomas.

Mostly occurring between 60 and 80 years of age (average age 65 years).

Initially, clinically inconspicuous, locally persistent, blurred, 1.0-3.0 cm large, painless, smooth, telangiectatic red spots appear, which in an early phase of tumor development are often misjudged as "banal telangiectasias in the context of rosacea or light-damaged skin".

Over the course of months, the area expands; the red coloration of the skin continues to increase in intensity; these tend to form extensive hematomas. These extensive skin hemorrhages (occurring with banal trauma) should lead to the diagnosis of "angiosarcoma of the scalp" if other explanations are not conclusive. Later formation of flat, blue-red papules, plaques and nodules, which tend to ulcerate with the formation of extensive erosions and ulcers. Subsequent lymphorrhea.

Note on clinical differential diagnosis (head-tilt maneuver: filling and becoming visible after a short head-down position (head-tilt maneuver = 20 sec. head-down position) is an important diagnostic sign.

Metastasis: Hematogenous, especially in the lungs and skeletal system. Lymphogenous, in regional lymph nodes.

Note! Always think of angiosarcoma in the case of unexplained extensive hemorrhages in the area of the scalp!

Initial: Enlarged endothelia lined by atypical, irregularly dilated, bizarre capillary-like structures that protrude into the lumina like buttons and often anastomose with each other. The endothelia show enlarged chromatin-rich nuclei and increased Ki-67 positivity.

Later: Solid masses of polymorphic spindle cells, blood-filled clefts and erythrocyte extravasations.

Immunohistology: Tumor cells express CD31, CD34 and vimentin. The neoplastic vascular spaces are either not or only slightly surrounded by actin-positive cells.

• Erythematous rosacea: Mostly bilateral; characteristic concomitant symptoms of rosacea (variable redness with flushing symptoms due to temperature changes, alcohol consumption, etc.; never smudging).
• Morbihan's disease: Mostly bilateral; characteristic accompanying symptoms of rosacea possible. No smugillations.
• Nevus flammeus: History excludes angiosarcoma.
• Hematoma (initial): History excludes angiosarcoma.
• Systemic lupus erythematosus: Symmetry of erythema; disorders of AZ; serologic autoimmune phenomena.
• Pellagroid: Only occurring in exposed areas, itching or burning after UV exposure, color rather red-brown.
• Kaposi's sarcoma: Exclusion of HIV infection (laboratory).
• Sarcoidosis: Chronic persistent red patches or plaques, livid discoloration possible in cold weather, may be associated with lupus pernio. Diascopic: autoinfiltrate. Histologically: Evidence of sarcoid granulomas!

Larger studies could prove the value of a postoperative adjuvant radiation therapy (linear accelerators are recommended) (significant reduction of mortality). Dose: 55-60 Gy, up to 75 Gy for suspected residual tumour. A large-scale safety level should be maintained. Radiation therapy alone is not recommended as the clinical results are inferior to combination therapy.

Caution! Recurrences are increasingly less radiation-sensitive!

In the case of non-resectable findings, there is good experience with pegylated liposomal doxorubicin and paclitaxel (Taxol). As a rule of thumb, about 25% of patients respond to doxorubicin-based regimens (50 mg/m2 every 28 days), with the additional administration of ifosfamide achieving a trend improvement, although with a considerable increase in toxicity.

In a large retrospective analysis (125 patients), the combination of liposomal doxorubicin and paclitaxel was found to be relatively effective, as was the combination of doxorubicin and ifosfamide.

Other possible chemotherapeutic agents for angiosarcoma are docetaxel, vinorelbine, gemcitabine and epirubicin.

Combined chemo/radiation therapy (taxanes) does not seem to achieve any improvement over pure chemotherapy.

Very poor prognosis for tumours > 4 cm in diameter, as early haematogenic metastasis (lung) occurs. 5-year survival rate is <10%. A better prognosis can be expected for angiosarcomas <4 cm.

1. Bong AB et al (2004) Treatment of scalp angiosarcoma by controlled perfusion of a carotis externa with pegylated liposomal doxorubicin and intralesional application of pegylated interferon alpha. J Am Acad 52: S20-S23
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