Angiosarcoma of the face and scalp

ARTICLE

An 88-year-old man presented with a two year history of a progressively enlarging, asymptomatic red patch over the left face. He was referred for dermatological consultation after failed systemic antibiotic treatment for "cellulitis" by the general physicians. He enjoyed good general health except for mild diabetes mellitus which was well controlled with glipizide 80 mg bd. Physical examination revealed an infiltrative, oedematous, dusky erythematous plaque involving a large area of the left face (Fig. 1). Cervical lymphadenopathy was not detectable. Skin biopsy of the lesion was performed which revealed a malignant skin condition. He received radiotherapy but died two months after diagnosis. Another male patient, 81-years-old, presented with a progressively enlarging growth over the scalp for two months (Fig. 2). He had been suffering from hypertension, ischaemic heart disease, gout and hyperlipidaemia. Examination revealed a 3.5 x 1.8 cm eroded fleshy tumour over the left side of the vertex. There was no cervical lymphadenopathy. Diagnostic skin biopsy was taken and revealed the same condition as in patient 1. He was treated with surgical excision and post-operative radiotherapy. He eventually succumbed two years after diagnosis due to brain metastasis. What is your diagnosis?

Angiosarcoma of the face and scalp

Histology examination of patient 1 revealed loosely adhesive aggregates of markedly atypical cells within irregular dilated vascular channels disposed haphazardly in the entire dermis, with fibrin thrombi (Fig. 3A, B). The neoplastic cells within the vessels stained positively with antibodies directed against von Willebrand factor (vWf), CD31 and CD34 at varying intensity and extent (Fig. 4A, B, C) indicative of the endothelial nature of this proliferation. The histological diagnosis is angiosarcoma.

Comments

Angiosarcoma is a malignant vascular tumour arising from both vascular and lymphatic endothelium. It comprises only approximately 1-2% of all soft tissue tumours. They can occur in any region of the body, but 60% arise in skin or superficial soft tissue. Fifty percent of cutaneous angiosarcoma occur in the head and neck [1]. Cutaneous angiosarcoma usually occurs in three settings:

1. Face and scalp of elderly people, with male outnumbering female by two to one. They can present as a bruise, cellulitis-like plaque (as in patient 1), nodules, plaque or erosion. They are highly aggressive and characteristically spread widely through the skin and soft tissue of the head and neck region before metastases. Metastases occur both via lymphatic or haematogenous spread. Most common sites for metastases are lymph nodes, lungs and liver.

2. In chronic lymphedematous areas, such as occurs in the upper arm after mastectomy with lymph node dissection or radiotherapy (Stewart Treves Syndrome).

3. In previously irradiated sites.

Cutaneous angiosarcoma varies in its histological appearance from well to poor differentiation. The well-differentiated lesions, consisting of irregular anastomosing vascular channels slated wide-apart in the dermis and subcutis, may be difficult to distinguish from an ill-defined haemangioma, as in the biopsy of patient 1. The atypical lining cells of the vascular channels and the cellular piling are clues to the correct diagnosis. In the first case, the very scattered disposition of the vessels and the prominence of intraluminal malignant cells also raise the differential diagnostic consideration of malignant angioendotheliomatosis. In these atypical cases, immunohistochemical markers are very helpful in making the diagnosis [2]. Neoplastic cells of angiosarcoma stain positively for von Willebrand factor, CD31 and CD34, while malignant angioendotheliomatosis is an intravascular lymphoma and stains immunologically with lymphoid markers LCA and L26. Making the correct diagnosis is absolutely critical for choosing the proper therapeutic modality and prognostication.

Due to the late presentation, difficulty in diagnosis and the highly aggressive nature of the tumour, the overall prognosis is poor. Mark, et al. showed the actuarial 2- and 5-year disease free survival were 44% and 24%, respectively. There is a propensity for both local recurrence and distant metastases [3]. The prognosis of radiation-associated angiosarcoma is dismal, due mostly to its poor differentiation and frequent diagnostic delay [4].

Radiotherapy is the current mainstay of treatment for angiosarcoma which was given to our two patients. Small localised diseases are amenable to surgical excision but the tendency for diffuse and clinically undetectable local spread hampers complete surgical excision. At present, wide surgical excision plus radiotherapy offer the best chance of survival. Bullen, et al. suggested local control of angiosarcoma of the scalp by assessment of the tumour margin by peripheral biopsies or Mohs technique followed by electron beam radiation [5]. Recently, a new chemotherapeutic agent, paclitaxel has been shown to be effective in treating angiosarcoma. Fata, et al. treated nine patients with angiosarcoma with the drug and obtained major response in eight (four partial response and four complete clinical response) and minor response in one [6]

References

1. Yang JC, Rosenberg SA, Glatstein EJ, Antman KH. Sarcomas of soft tissue. In: DeVita VT, Hellman S, Rosenberg SA, editors. Cancer: principles and practice of oncology, 4th edition. Philadelphia: J.B. Lippincott, 1993: 1436-55.

2. Hitchcock MG, Hurt MA, Santa Cruz DJ. Cutaneous granular cell angiosarcoma. J Cutan Pathol 1994; 21: 256-62.

3. Mark RJ, Poen JC, Tran LM, Fu YS, Juillard GF. Angiosarcoma, A report of 67 patients and a review of the literature. Cancer 1996; 77: 2400-6.

4. Cafiero F, Gipponi M, Peressini A, Queirolo P, Bertoglio S, Comandini D. Radiation-associated angiosarcoma: diagnostic and therapeutic implications-two case reports and a review of the literature. Cancer 1996; 77: 2496-502.

5. Bullen R, Larson PO, Landeck AE, et al. Angiosarcoma of the head and neck managed by a combination of multiple biopsies to determine tumor margin and radiation therapy. Report of three cases and review of the literature. Dermatologic Surgery 1998; 24: 1105-10.

6. Fata F, O'Reilly E, Ilson D, et al. Paclitaxel in the treatment of patients with angiosarcoma of the scalp or face. Cancer 1999; 86: 2034-7.