Choi Won-Tak, Stetsenko Galina Y, Zhang Jiong, Olerud John E, Argenyi Zsolt B, George Evan
University of Washington School of Medicine.
Dermatol Online J. 2013 Nov 15;19(11):20409.
Cutaneous angiosarcoma of the head and neck is a rare, highly malignant neoplasm; prognosis is heavily influenced by tumor size, resectability, and stage at initial diagnosis. Most patients present with one to several erythematous to violaceous patches, plaques, or nodules. However, the clinical presentation is highly variable and leads to delayed diagnosis. We report cutaneous angiosarcoma in a 43-year-old man who presented with an 11-month history of progressive solid (non-pitting) edema involving his entire face, scalp, eyelids, and neck without characteristic clinical features of cutaneous angiosarcoma. A skin biopsy had shown non-specific findings consistent with solid facial edema or rosacea. Various etiologies were considered but there was no significant improvement after directed medical therapy. Repeat skin biopsies revealed angiosarcoma involving the dermis and sub-cutis. Computed tomography (CT) of the chest showed multiple lung nodules bilaterally and a lytic lesion in the T6 vertebra consistent with metastases. He was treated with single agent chemotherapy (paclitaxel), and had a partial response that restored his ability to open both eyes spontaneously; However, his edema has recently progressed 7 months after diagnosis. This is a rare example of cutaneous angiosarcoma presenting as progressive solid facial edema, which underscores the diverse range of clinical manifestations associated with this neoplasm.
头颈部皮肤血管肉瘤是一种罕见的高度恶性肿瘤；预后受到肿瘤大小、可切除性及初始诊断时分期的严重影响。大多数患者表现为一至数个从红斑到紫蓝色的斑片、斑块或结节。然而，其临床表现高度多变，导致诊断延迟。我们报告了一名43岁男性的皮肤血管肉瘤病例，该患者有11个月的进行性实性（非凹陷性）水肿病史，累及整个面部、头皮、眼睑和颈部，并无皮肤血管肉瘤的典型临床特征。一次皮肤活检显示非特异性结果，符合实性面部水肿或酒渣鼻。考虑了多种病因，但针对性药物治疗后并无显著改善。重复皮肤活检显示血管肉瘤累及真皮和皮下组织。胸部计算机断层扫描（CT）显示双侧多个肺结节，T6椎体有一个溶骨性病变，符合转移表现。他接受了单药化疗（紫杉醇），有部分反应，恢复了双眼自主睁开的能力；然而，在诊断7个月后，他的水肿最近又进展了。这是皮肤血管肉瘤表现为进行性实性面部水肿的一个罕见病例，突出了与该肿瘤相关的临床表现的多样性。
