Cutaneous angiosarcoma of head and neck.

Cutaneous angiosarcoma is a rare aggressive tumor of capillary and lymphatic endothelial cell origin. Cutaneous angiosarcoma of the head and neck regions seems to be a distinctive neoplasm with characteristic clinicopathologic features that differ from angiosarcoma in other anatomic locations. Angiosarcoma, regardless of their setting, has a bad prognosis. We presented here a case of 80 years old male, with multiple nontender grouped purple to red hemorrhagic vesicular and bullous lesions over left lower cheek and upper neck area, with bilateral cervical lymph nodes since 1 month. Computed tomography thorax showed nodular opacities in the right upper and midzones. Excisional biopsy showed characterstic "dissection of collagen" with mild nuclear atypia. Immunohistochemistry showed tumor cell positive for CD-31 and Fli-1. Patient died within 1 month of presentation.

What was known?

Angiosarcoma is a malignant tumor derived from the endothelium that occurs in a variety of anatomic sites including the skin. Angiosarcoma, regardless of their setting, has a bad prognosis.

Introduction

Cutaneous angiosarcoma is a rare aggressive tumor of capillary and lymphatic endothelial cell origin. It presents as multiple purple and red papules, and nodules on the head and neck or the extremities.[1] Angiosarcomas of the skin are uncommon and constitute less than 1% of all sarcomas.[2] It mainly occurs in males (sex ratio of 3:1), at an average age ranging from 60 to 80 years.[3] Cutaneous angiosarcoma occurs exclusively in three clinical settings namely idiopathic angiosarcoma of face, scalp and neck; angiosarcoma associated with chronic lymphedema (Stewart–Treves syndrome); and postradiation angiosarcoma.[34] Differential diagnosis of cutaneous angiosarcoma includes intravascular papillary endothelial hyperplasia, Kaposi sarcoma (multiple hemorrhagic sarcoma), Kaposi-like hemangioendothelioma, angiolymphoid hyperplasia, Kimura's disease, and amelanotic melanoma.[5] Lack of awareness of this entity and its variable presentation can result in diagnostic delay.[6]

Case Report

An 80-year-old male presented with multiple nontender grouped, purple red hemorrhagic vesicular and bullous lesions over left lower cheek and upper neck area with blood-stained serous discharge since 1 month. It started as small vesicular lesions over cheek which gradually increased in size and spread to upper neck and behind ear, and clear fluid was replaced by blood-stained fluid. Patient had history of occasional throbbing headache. No history of radiation was present. Boggy infiltration of scalp with pitting edema along with diffuse, erythematous painless swelling below the left eye was present [Figures 1 and 2]. Oral cavity showed cherry red-colored single cystic lesion around 0.5 × 1 cm sized over left buccal mucosa. Bilateral cervical lymph nodes were enlarged. All routine investigations were normal. Computed tomography (CT) of neck showed cervical lymphadenopathy. CT thorax showed fibrotic bands in both upper lobes with thin-walled cavity at cardiac apex and little nodular opacities in the right upper and midzones. The TNM staging system based on the extent of the tumor (T), the extent of spread to the lymph nodes (N), and the presence of distant metastasis (M) for cutaneous angiosarcoma was T2bN1M1. Excisional biopsy showed irregular anastomosing vascular channels lined by single layer of enlarged endothelial cells, which permeate between collagen bundles causing “dissection of collagen”. The cells showed mild pleomorphism with vesicular nuclei and prominent nucleoli. Mild nuclear atypia with mitosis was evident [Figures 3 and 4]. On immunohistochemistry, tumor cell expresssed CD-31 and Fli-1 which was conclusive of angiosarcoma. We referred the patient to cancer center for further management, but he died at home within 4 days after discharge from our hospital.

Figure 1

Purple red hemorrhagic swelling below the left eye with diffuse, erythematous vesicular lesions over ipsilateral cheek

Figure 2

Erythematous vesicular lesions in retroauricular region with diffuse infiltration of scalp

Figure 3

Irregular anastomosing vascular channels lined by single layer of enlarged endothelial cells which permeate between collagen bundles causing “dissection of collagen” (H and E, ×100)

Figure 4

Mild pleomorphism showing vesicular nuclei and prominent nucleoli along with nuclear atypia (H and E, ×400)

Discussion

Angiosarcoma is a rare, malignant neoplasm, composed of endothelial cells comprising less than 1% of all sarcomas, which can occur at any site; skin and superficial soft tissue being the most common locations. Cutaneous angiosarcomas primarily affect elderly persons predominantly males, with more than 50% occurring in the scalp or on face.[7] Cutaneous angiosarcoma of the head and neck regions seems to be a distinctive neoplasm with characteristic clinicopathologic features that differ from angiosarcoma in other anatomic locations. Diagnosis of cutaneous angiosarcoma is often delayed, due to its putatively innocous clinical appearance. There is general agreement that the poor prognosis of cutaneous angiosarcoma of the head and neck regions is not influenced by the histologic grade and mitotic activity, as it is the case with angiosarcoma in other anatomic regions.[89] However, tumor size, depth of tumor invasion, and completeness of surgical resection are more reliable prognostic indicators.[9]

Cutaneous angiosarcoma can metastasize to the lungs, liver, cervical lymph nodes, spleen and rarely, heart and brain. The mean time of survival after metastases in a case series was 4 months.[9] They are aggressive tumors that tend to recur locally and to metastasize despite aggressive multimodal therapy. Because of predilection of cutaneous angiosarcoma for multifocality and inapparent spread, complete surgical resection is often unattainable. Negative microscopic section margins are hardly achieved during surgery, resulting in multiple operations with large postoperative defects. Radiation is an effective modality for treating local disease, especially when used after surgical resection of macroscopic tumor.[1011] Overall prognosis is poor with reported 5-year survival of 10-35%.[9] About half of the patients are dying within 15 to 18 months of presentation.[12] Therefore, the outcome of cutaneous angiosarcoma is disappointing with rapidly fatal course as seen in the present case. Awareness of various presentations of this entity and biopsy will result in the initiation of early treatment.