1. AbstractCutaneous angiosarcoma is a soft tissue sarcoma that presents as a violaceous plaque, resembling a persistent bruise on the head and neck. The hematoma-like lesions are pitfalls that could be the first spy of malignant vascular tumors. The low survival rates of cutaneous angiosarcoma require a recognition as early as possible. We report a case of misdiagnosed cutaneous angiosarcoma of the head, which developed after a car crash and that, was firstly diagnosed as bruise.
3. IntroductionBruises could be pitfalls especially when they are long-lasting and unresponsive to traditional therapy. They could be the first spy of malignant vascular tumors like cutaneous angiosarcoma and Kaposi sarcoma. A detailed anamnesis and an evaluation of risk factors are important to direct the diagnosis. We report a case of misdiagnosed cutaneous angiosarcoma of the head which developed after a car crash and was firstly diagnosed as bruises.
4. Case presentationA 86-year-old man presented to the Dermatology Department with a 2-month history of new-onset violaceous erythematous-edematous lesion on the top of the head. This appeared concurrently with multiple break cracked ribs during a car crash and it was diagnosed, in the first instance, as an ecchymotic bruising. He had a history of melanoma pT1a on the back 15 years ago and a history of melanoma pT2b overlying left shoulder blade 5 years ago with negative sentinel lymph node. On examination, the patient showed an erythematous-violaceous indurated ecchymotic plaque with violaceous nodules and multiple ulcerations on his head (Figure 1A). A skin incisional biopsy was executed and the histopathological examination showed irregular dissecting vascular proliferation through the dermis. Atypical endothelial cells surrounded vascular areas and they sometimes aggregated themselves (Figure 1B). Since patient’s history, we suspected a Kaposi sarcoma and a metastatic melanoma. Immunohistochemistry stain were performed resulting negative for Human herpesvirus 8, pS100, HMB45 and melan-A ruling out this diagnoses. Positive CD31+ with high percentage of Ki67 were diagnostic of cutaneous angiosarcoma (Figure 1C)
5. Discussion We describe a case of a patient with persistent bruises on the head following a car crash. Long-term bruises evolving in nodular lesions could be suspect for malignant vascular tumors, such as cutaneous angiosarcoma and Kaposi sarcoma. Cutaneous angiosarcoma accounting for 1% of soft tissue sarcomas, fourth for frequency among cutaneous sarcomas [1]. Average age of diagnosis is 73 years, with a slight male white patient predominance [2]. The most important risk factor of cutaneous angiosarcoma is ultraviolet light with higher risk in patients with genetic susceptibility. Cutaneous angiosarcoma is usually an idiopathic primitive form that occurs more frequently on the face and scalp (angiosarcoma of Wilson Jones). Other two forms of angiosarcoma are known: chronic lymphedema-associated cutaneous angiosarcoma (Stewart-Treves syndrome) arising secondary to lymphedema following axillary lymphnode dissection1 and the post irradiation cutaneous angiosarcoma arising in areas previously treated for solid tumor malignancies like breast cancer. A violaceous plaque, resembling a persistent bruise on the head and neck is the most typical clinical presentation. Usually it can lead to rapid growth, ulceration, localized hemorrhage or invasion into adjacent structures due to its aggressive nature. Nodular lesion is significantly more common in cutaneous angiosarcoma on the scalp [3]. Histopathology of cutaneous angiosarcoma includes weird endothelial cells arranged in irregular, disarrayed vascular lines or sinusoids shifting normal, regularly oriented collagen within the dermis. Immunohistochemical expression of CD31, CD34, D2-40, and VEGFR3 can help to differentiate cutaneous angiosarcoma from nonendothelial neoplasms. Secondary forms of cutaneous angiosarcoma can present MYC expression [2]. The differential diagnosis of hematoma-like lesions include vascular tumor such as Kaposi sarcoma. It could be differentiated by cutaneous angiosarcoma by histology and a positive immunohistochemistry for Human herpesvirus 8. In addition, since our patient have removed two melanomas, the diagnosis of metastasis should be excluded by histology and immunohistochemistry. Five-year survival of cutaneous angiosarcoma is approximately 50%, with lowest survival rates among individuals with head and neck lesions [1]. Treatments of cutaneous angiosarcoma include surgery, radiation therapy, and systemic chemotherapy. Wide local excision with negative margins is suggested, because is associated with improved survival. The management of large cutaneous angiosarcoma required multimodal strategies (eg, excision plus radiation treatment). Tumors larger 5 cm are associated with worse outcomes and they may benefit from multimodal treatment with taxanes, first-line treatment, plus radiation. In addition, Eribulin mesylate showed a promising response rate in patients previously treated with taxane [4]. Emerged immunotherapies (eg pembrolizumab) and electrochemotherapy [5] are also in assessment. We describe a case of cutaneous angiosarcoma, a tumor with low survival rate recognized rarely in early stage. We suggest being careful with long-lasting ecchymotic lesions, they require a biopsy to speed up the diagnosis of cutaneous neoplasms as angiosarcoma.
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4. Fujisawa Y, Fujimura T, Matsushita S, et al. The efficacy of eribulin mesylate for patients with cutaneous angiosarcoma previously treated with taxane: a multi-center, prospective, observational study [published online ahead of print, 2020 Mar 20]. Br J Dermatol. 2020; 10.1111/bjd.19042.
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Ilaria T .Bruises as First Manifestation of Cutaneous Angiosarcoma . Annals of Clinical and Medical Case Reports 2020