Split Facies with Destructive and Leonine Appearance: An Atypical Presentation of Angiosarcoma

Dear Editor, Angiosarcoma is a rare, aggressive cutaneous vascular tumor that typically affects the elderly population and involves the head and neck region. Clinical manifestations vary widely, and diagnosis is confirmed through histopathology, which shows irregular vascular channels lined by plump endothelial cells exhibiting atypia and mitosis, and by immunochemistry. We present a case of an elderly patient with eyelid destruction and an infiltrative plaque with scaling, crusting, and ulceration, giving a split-facies appearance. The left side of the face showed a leonine facies with marked nodularity. A 63-year-old woman presented with progressive papulo-nodular and ulcerative lesions on her face for the past year. It started with multiple reddish to skin-colored papules and nodules on her left cheek, gradually spreading over her entire face, scalp, and neck. In the past three months, there was rapid progression, associated with weight loss, loss of appetite, and difficulty in opening the eyes due to eyelid involvement. Examination revealed leonine facies with diffuse infiltration and nodules, more prominent on the left side of her face, with a red-to-purple hue extending to the scalp, neck, and pinna [Figure 1]. Areas of ulceration, telangiectasias, and hemorrhagic crusting were present. Eyelid infiltration resulted in the inability to open her eyes, along with ulceration in the infraorbital region. Complete blood count, liver and kidney function tests were normal. Magnetic resonance imaging of the head and neck showed thickening of the dermis with increased vascularity and preserved subcutaneous fat. There was retinal detachment in the right eye with hemorrhagic content in the posterior chamber along with clear facial sinuses.Figure 1: (a) The right side face shows infiltrative plaque with overlying scaling while the left side face shows more nodularity (split facies). (b) Papulo-nodules and plaques on the left side of the face (leonine facies) with overlying ulceration, hemorrhagic crusting, and eyelid destructionHistopathological analysis revealed numerous irregularly-shaped, branching, and anastomosing vascular channels in the dermis, lined by markedly plump endothelial cells, infiltrating into the adjacent tissue [Figure 2]. The endothelial cells were polygonal in shape, hobnailing into the vascular lamina, displayed atypia, brisk mitosis (4–5/10 high power field), and were positive for CD34 in immunohistochemistry [Supplementary Figure 1].Figure 2: (a) Dermis shows numerous irregularly-shaped branching and anastomosing vascular channels lined by endothelial cells (H and E, 4x). (b) Numerous irregularly-shaped branching and anastomosing vascular channels lined by endothelial cells with marked atypia and brisk mitosis (H and E, 10x). (c) Atypia with brisk mitosis along with hobnailing of endothelial cells (H and E, 40x)Supplementary Figure 1: IHC marker for CD34 positive (40x)Based on the clinical features and histopathology, a diagnosis of cutaneous angiosarcoma was made, and the patient was treated with intravenous paclitaxel (initially 80 mg/cm2, weekly, then 160 mg/cm2 thrice weekly for a total of nine cycles) supportive therapy, which showed significant improvement [Supplementary Figure 2].Supplementary Figure 2: Significant resolution in papulo-nodules and plaque with overlying ulcer on the left side of the face after nine cycles of chemotherapyAngiosarcoma is a highly aggressive malignant tumor with a predilection for cutaneous involvement in elderly patients. It accounts for 2–3% of all soft tissue sarcomas.[1] This tumor arises from the endothelial cells of either lymphatic or blood vessels. Various risk factors associated with angiosarcoma include high-dose radiation, chronic lymphedema (e.g., Stewart–Treves syndrome, filariasis, Milroy’s disease, idiopathic, traumatic, and congenital), environmental factors (e.g., arsenic ingestion, thorium dioxide, vinyl chloride), and genetic syndromes (xeroderma pigmentosum, neurofibromatosis, retinoblastoma, Ollier disease, Maffucci disease, and Klippel–Trenaunay syndrome). The clinical manifestations vary, making the diagnosis challenging. It can involve the skin (with the head and neck being the most common sites), soft tissues, visceral organs, bones, and retroperitoneum.[2] It typically presents as reddish-purple papules, nodules, or flat infiltrations that ulcerate, bleed, and develop hemorrhagic crusting. Other manifestations include rosacea-like, bruise-like, eczema-like, hematoma-like, cellulitis-like, xanthelasma-like, and facial angioedema.[3,4] In our case, a split facies presentation was observed: the right side showed a diffuse erythematous to purplish infiltrative plaque with a smooth surface, crusting and scaling, while the left side showed erythematous to purplish papulo-nodular and plaque lesions with attenuation of facial lines, coarsening, and a waxy appearance (leonine facies) with an irregular surface, ulceration, and crusting. Angiosarcoma commonly metastasizes to the lungs, lymph nodes, and other viscera. Eyelid destruction has been reported in earlier studies.[4] Histopathology of angiosarcoma can be well-differentiated (numerous irregular vascular spaces lined by atypical endothelial cells) or poorly differentiated (including spindle, epithelioid, polygonal, and primitive round-shaped cells with increased mitosis and atypia with poorly formed vascular spaces).[1] Treatment includes surgery, radiotherapy (>50 Gy over a wide field or postoperative low dose within three weeks of tumor resection), and a combination of chemotherapy (doxorubicin, ifosfamide, olaratumab, paclitaxel, propranolol, anti-VEGF, anti-tyrosine kinase inhibitors, and immunotherapy), depending on the site, size, resectability, type of tumor, and metastasis.[1,5] In conclusion, we report a rare case of aggressive cutaneous angiosarcoma with an atypical presentation, i.e., split facies with leonine appearance, confirmed by histopathology, which had significant improvement with chemotherapy. Despite various treatments, the five-year survival rate ranges from 12 to 40%, with 20 to 40% of patients experiencing local recurrence or metastasis.[1] Hence, early diagnosis can improve survival, patients’ quality of life, and prevent disfigurement. Declaration of patient consent The authors certify that they have obtained all appropriate patient consent forms. In the form the patient has given her consent for her images and other clinical information to be reported in the journal. The patient understands that her name and initials will not be published and due efforts will be made to conceal her identity, but anonymity cannot be guaranteed. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest. Use of artificial intelligence (AI) The preparation of this manuscript was carried out entirely by the author without the use of artificial intelligence technologies.