Angiosarcoma (AS) is a very rare and aggressive tumor; it represents about 1- 2% of all sarcomas, and only 5% of all skin magnlinities [1,2]. Skin is the main site followed by liver and bones [3]. Angiosarcomas are characterized by their aggressively, difficulty of histological diagnosis, and problem to define the adequate treatment [4]. We report a case of a locally advanced facial angiosarcoma successfully treated by only radiotherapy.

secondary to kidney stones, presented with a scalp lesion, localized at the left temporal area, measuring approximately 8 cm, the tumor progressed in one year and was ignored by the patient because of his economic conditions. Dermatological examination revealed 80 x 56 mm violaceous-red plaque, with a large central ulceration, and a bleeding zones, in contact with pinna of the left ear but without invasion (Figure 1). Histopathologic examination demonstrated a spindle-cell proliferation occupying the whole thickness of the dermis and invading subcutaneous tissue, it was associated with an irregular anastomosing vascular channels lined by atypical endothelial cells. Endothelial cells presented an enlarged and hyperchromatic nuclei. Immunohistology analysis was positive for vascular markers CD31 and CD34, and negative for cytokeratin AE1/AE3, EMA and HMB45, concluding to the diagnosis of cutaneous angiosarcoma.

Facial computed tomography showed a large lesion invading the whole skin, without bone extension (figure 2), and with no lymph nodes disease.  Work up including a chest CT, as well as abdominal ultrasounds were normal.

 Because of the large size of the tumor, and its localization, the patient was unable for surgery, he benefited from an exclusive external beam radiotherapy, 70 Gy over 53 days (2 Gy daily), without concurrent chemotherapy. Two years later, results of follow-up physical and radiological examinations did not reveal any signs or symptoms of tumor recurrence (Figure 3).

Cutaneous angiosarcoma is a rare vascular tumor associated with a poor prognosis and limited treatment options [1,2]. It predominantly affects elderly patients with an average age of 70 years [3]. Skin angiosarcoma are usually associated with risk factors, especially chronic lymphedema, and history of trauma or skin irradiation [4,6]. AS secondary to previously radiotherapy affect especially women followed for breast cancer, and appear usually 10 to 20 years after irradiation [7]. Cases of primary cutaneous AS without risk factors, affect mainly the face and the scalp as described in our case.

Diagnosis of angiosarcoma is difficult, and differential diagnosis involves a large range of spindle-cell tumors, including spindle-cell squamous-cell carcinoma, spindle-cell melanoma, leiomyosarcoma, pleomorph sarcoma, fibroxanthoma, and Kaposi’s sarcoma. Consequently, immunohistochemistry is necessary to confirm diagnosis of AS. Endothelial markers as CD31 and CD34 represents the most sensitive and specific markers for demonstrating vascular endothelial differentiation [8].

The optimal treatment of cutaneous angiosarcoma has not been defined; because of its rarity, there are no adequate randomized prospective studies for therapeutic standardization [9]. Management of AS includes a multidisciplinary team and may involve a combination of surgery and radiotherapy .A large excision with sufficient margins is the treatment of choice. Unfortunately, in most cases however, complete excision is not possible due to the extension and multifocal nature of the disease, and surgery alone frequently failed to eradicate scalp angiosarcoma [4,10].

Radiotherapy is usually performed in locally advanced and unrespectable AS. Several authors recommend a combination of surgery and RT as an optimal treatment [11]. In 1 series of 28 angiosarcomas, Mark et al [12] reported better survival after a median 32 months with combined surgery and RT compared with surgery alone. In cases unsuited for surgery, radiotherapy can be considered as only treatment option. In this discussed case, surgery was not possible, and only radiotherapy was performed. Fortunately, we had a complete remission, with a minimum of sequels. Generally, radiotherapy of scalp is well tolerated and we can give high doses (70 Gy). Chemotherapy may be useful as a palliative option in systemic disease. The efficacy of chemotherapy is undefined; some studies reported a clinical response but no survival benefit. Chemotherapeutic agents used have included doxorubicin, cyclophosphamide, dacarbazine, actinomycin D, and gemcitabin [13].

The prognosis of angiosarcoma is poor because of its high potential for metastasis, and overall survival in five years does not exceed 30%. The best chances of survival are observed in patients with localized disease who can benefit from large excision. Prognostic factors include age, clinical staging, primary site, tumor size and histological grade. Because of the difficulty of resection with free margins, tumors located in the head or neck present worse prognosis [14].

Angiosarcoma is a rare and aggressive tumor, its optimum treatment still undefined. A large excision followed by adjuvant radiotherapy may offer maximum chances of cure. Radiotherapy is also being indicated for cases unsuited for surgery. In this work we have reported a case of AS successfully treated by only radiotherapy. More cases and studies are necessary to confirm the efficiency of radiotherapy in management of skin angiosarcoma.

AS: Angiosarcoma; CT: Computed tomography; RT: Radiotherapy

The authors and planners have disclosed no potential conflicts of interest, financial or otherwise.

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Figure 1: Large, painless, and violaceous lesion, with a central ulceration, and bleeding zones.

Figure 2: Facial CT showing a large lesion invading the whole skin, without bone extension.

Figure 3: Clinical appearance after radiotherapy, Patient is in complete remission.