Cutaneous angiosarcoma of scalp.

Sir,

Angiosarcoma (AS) is a rare, malignant tumor of vascular endothelial origin.[1] It can affect any part of the body, but the scalp and face are the favored sites. Patients usually are elderly and present with single or multiple nodules or plaques, which can ulcerate or bleed.[2]

A 58-year-old female presented with complaints of persistent itching, scalp swelling, and occasional bleeding, discovered during combing of her hair since 4–5 months. On clinical examination, there were multiple, well-defined, yellow-to-dark brown plaques of varying sizes (1 × 1 to 3 × 2 cm) involving the right parieto-occipital and the left occipital regions [Figure 1a and b]. Plaques were dry, covered by necrotic slough, and firm in consistency. Surrounding skin showed hypopigmentation. There were no palpable lymph nodes in the neck. Provisional diagnosis of dissecting cellulitis of the scalp was made and skin biopsy was performed. Histological examination showed a tumor composed of anastomosing vascular channels lined by pleomorphic cells [Figure 2a and b]. Few bizarre bi and multinucleated cells were also seen. On immunohistochemistry, tumor cells were positive for vimentin, CD31, and CD34, and negative for PanCK, HMB45, and desmin [Figure 2c and d]. Based on the above findings, diagnosis of angiosarcoma was rendered. Because of the multifocality of the lesion, subsequently chemotherapy comprising docetaxel at a dose of 25–30 mg/m2 weekly for 3 weeks was given. The patient showed good response with regression of the lesion.

Figure 1

(a) Shows yellow crusted plaques with indurated surface and surrounding erythema with loss of hairs; (b) Single 1.5 × 1 cm brownish black plaque with irregular margins and crusted surface

Figure 2

(a) Skin biopsy shows numerous, irregular, and anastomosing vascular channels (H and E, ×100), (b) Shows vascular spaces lined by pleomorphic, hyperchromatic cells (H and E, ×400), Immunohistochemistry revealed positivity for (c) CD31 and (d) CD34 seen on ×400

AS was first described by Livingston and Klemperer in 1926.[3] It accounts for less than 1% of head and neck malignancies.[1] Several predisposing factors for AS have been suggested, including chronic lymphedema, irradiation, exposure to chemical carcinogens, immunosupression in transplant patients, and increasing age. Clinical presentation of AS is variable.[2] Because of its rarity and relative benign appearance clinically, this entity is diagnosed late and patients usually have advanced disease at the time of presentation.

On histopathology, the characteristic feature of AS are variable; low grade AS is a well-differentiated tumor that retains some of the functional and morphological features of normal vascular endothelium, whereas poorly differentiated AS shows sheets of pleomorphic cells with distorted architecture, markedly pleomorphic nuclei, and brisk mitosis, making it difficult to distinguish from melanoma or carcinoma.[2] On immunohistochemistry, AS are positive for CD31, CD34, and factor VIII antigen, and lack expression of pancytokeratin and HMB45. Both low grade and poorly-differentiated AS show rapid and invasive growth involving the dermis and deep structures.[2] AS has a tendency to metastasize by lymphatics or hematogenous route. Distant metastases may occur in up to 50% of the cases, the lung being the most common site followed by liver.[34] Optimal treatment for AS is still not defined owing to limited cases being reported. Wide local excision of the lesion with histologically negative margins is the goal of the surgery. Radiotherapy is generally indicated postsurgery to improve survival rates.[4] However, multifocal and unresectable lesions require combined chemo-radiotherapy. Tumor size (>10 cm), histologic grade, and multifocality are some of the prognostic factors related to relapse. Depending on the modality of treatment, local recurrence have been observed in 35–86% of patients, therefore, lifelong follow up is advised.[2]

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Conflicts of interest

There are no conflicts of interest.