Angiosarcoma of the head and face skin C44.-

Klemperer and Livingston, 1926

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).

Capillitium, forehead area, face.

• Initially, clinically inconspicuous, persistent, blurred, 1.0-3.0 cm large, painless, smooth, teleangiectatic red spots appear, which are often misunderstood as "banal telangiectases in the context of rosacea or light-damaged skin" in an early phase of tumor development.
• Over the course of months, the area of the skin expands; increasingly intensive red colouring of the skin continues; these tend to form flat haematomas. These flat skin bleedings (which occur with banal traumas) should, if other explanations do not make one think of the diagnosis "angiosarcoma of the scalp". Later formation of flat, blue-red papules, plaques and nodules, which tend to disintegrate into ulcers with the formation of extensive erosions and ulcers. Followed by lymphorrhoea.
• Note on the clinical differential diagnosis (head-tilt manoeuvre: The filling and the appearance of the filling after a short lowering of the head (head-tilt manoeuvre = 20 sec. lowering of the head) is an important diagnostic sign.
• Metastasis: Haematogen, especially in the lungs and skeletal system. Lymphogen, in regional lymph nodes.

• Notice! In case of unexplained extensive bleeding in the scalp area, always remember the angiosarcoma!

• Initial: Bizarre capillary-like structures lined by atypical button-like enlarged endothelials protruding into the lumina, irregularly expanded, often anastomosing 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 surrounded or only slightly surrounded by actin-positive cells.

Clinic, histology.

• Erythematous rosacea: Mostly bilateral; characteristic side effects of rosacea (variable redness with flush symptoms with temperature changes, alcohol consumption etc.; never suggestive).
• Nevus flammeus: History rules out angiosarcoma.
• Haematoma (initial): medical history rules out angiosarcoma.
• Systemic lupus erythematosus: symmetry of erythema; disturbances of the AZ; serological autoimmune phenomena.
• Pellagroid: Occurs only in exposed areas, after UV-exposure itching or burning, colour rather red-brown.
• Kaposi's sarcoma: exclusion of HIV infection (laboratory).
• Sarcoidosis: Chronic persistent red spots or plaques, livid discoloration possible in cold weather, may be associated with lupus pernio. Diascopic: self-infiltrate. Histological: detection 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.

The best results are achieved with surgical measures ( excision). The aim is the complete removal of the affected skin areas with plastic covering. The timely and generous excision requires a histologically secured tumor margin determination. The clinically visible "margins" are not reliable, as there is usually incongruent growth. The surgically set defect should remain open and only be covered by split skin after the edges have been safely cut free of tumor. Only in exceptional cases should other plastic surgery procedures ( flap plastics) be chosen.

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.

Therapeutic outlook: Data with classical chemotherapy suggest that new concepts are needed to improve the situation. First experiences have been made with the anti-VEGF Bevacizumab (Avastin).

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