A Recurrent Angiosarcoma Isolated to the Eyelid without the Recurrence on the Primary Lesion of the Forehead

Abstract

Angiosarcoma is a highly malignant vascular tumor of endothelial origin. Initially, a cutaneous manifestation presents as a singular or multifocal bruise-like patches on the skin, most frequently on the face, the scalp or the neck regions. On progression, the lesions become violaceous, and ill-defined spongy nodular tumors appear. Our patient was a 71-year-old man with a previous history of angiosarcoma on the right forehead and was treated with an excision and local radiation 3 years ago. Several months after the treatment, a dark brownish to violaceous patch with edema arose from the right upper eyelid and spread out to the lower eyelid. Clinically, an ecchymosis caused by trauma or other hemangioma was suspected more than a recurrent angiosarcoma. Histopathologic examinations including immunohistochemical studies were consistent with cutaneous angiosarcoma. Herein, we report a rare case of a recurrent isolated angiosarcoma without the recurrence of a primary lesion.

Recurrence of an Angiosarcoma in the Eyelid: A Case Report of a Rare Entity

“…Cutaneous angiosarcoma is a rare vasoformative malignant neoplasm arising from the vascular or lymphatic endothelium [3]. Soft tissue sarcomas account for less than 1 % of all cancers, and angiosarcomas account for 1-2 % of them.…”

Exuberant angiosarcoma

“…Clinical differential diagnoses include seborrheic dermatitis, ecchymosis, hemangioma, cellulitis, erysipela, rosacea, angioedema, melanoma and Kaposi's sarcoma. 2 In histopatholo-gy, pleomorphic malignant endothelial cells may be noticed, forming vascular sinusoids that dissect collagen fibers. Architecture becomes chaotic, when the disease is more aggressive, with undefined vascular spaces.…”

“…When the tumor is in the initial stage, radical surgical excision constitutes the first therapeutic option and may be accompanied by adjuvant radiation therapy, as the tumor may display clinically undetectable local dissemination.² However, when the disease is extensive, radiation therapy or chemotherapy may be considered. 2 , 5 For metastatic disease cases, cytotoxic chemotherapy is the preferred treatment. Standard palliative chemotherapy for soft tissue sarcoma has been performed with doxorubicin.…”

Primary Angiosarcoma of the Skin Presenting as Mild Erythema

“…Initially, primary angiosarcoma of the skin presents as solitary or multiple purpuric patches on the face, neck, or scalp. Early lesions can be confused with other skin lesions, such as bruises 2 , salmon patches, hemangiomas, hematomas 3 , rosacea 4 , rhinophyma 5 , and early Kaposi's sarcoma. The patient in this case exhibited a unique clinical feature, in that he presented with mild erythema rather than with angiosarcoma.…”