An 87-year-old male patient was admitted to our Clinic for diagnostic evaluation and treatment of extensive, confluent erythematous-livid areas that affected almost the entire skin of his face, scalp, and neck Figure (1). The patient also showed left eyelid ectropion and a palpable subcutaneous, painless nodule in the left mandibular region the size of an apple that bled easily. The patient had been treated for undiagnosed erythematous papules on his left cheek 2 years before and reported that his condition had dramatically worsened over the last few weeks. The patient was otherwise healthy and was only being treated for hypertension and hyperlipemia. Multiple computed tomography (CT) scans were performed which showed an extensive disease affecting the skin and subcutaneous tissue of almost the entire face, scalp and neck, with occasional involvement of muscle tissue as well. Numerous secondarily altered lymph nodes of the neck and multiple tumour infiltrates in the liver were also seen. A skin biopsy from the scalp was performed Figure (2). Histopathology showed a tumoral lesion composed of irregular, anastomosing vascular spaces and an increased number of surrounding endothelial cells with hyperchromatic, atypical nuclei. The clinical and pathological features corresponded to a typical presentation of angiosarcoma (AS). The patient had an inoperable disseminated disease and palliative chemotherapy with paclitaxel was initiated. After three cycles of therapy, the patient's general condition worsened, and was admitted to a palliative care facility. cAS is a rare vascular malignant tumour that has a high risk of both local and distant metastatic dissemination despite aggressive therapy. It is classified into primary AS that occurs sporadically, usually in the scalp or face, and secondary AS that is most often related to a history of radiation treatment and chronic lymphedema.1 This malignant tumour resembles a “spreading bruise” that varies from blue to red in colour. The diagnosis of cAS is often delayed due to its subtle initial clinical appearance as it is often mistaken for an ecchymosis or haematoma, especially in elderly patients on anticoagulants. Most patients are asymptomatic although some may present with bleeding, oedema, and/or ulceration. Diagnosis is based on clinical examination and histopathological and immunohistochemical analysis. The extent of the disease may be assessed with magnetic resonance imaging or CT.1, 2 AS represents less than 0.1% of all head and neck malignancies but is associated with the highest rate of lymphatic dissemination among all head and neck soft tissue sarcomas.3, 4 AS shows aggressive behaviour and carries a poor prognosis, thus early diagnosis is crucial. The optimal treatment is surgery followed by wide-field radiotherapy. Clear surgical margins are of paramount importance and correlate with better outcome, but the disease is often so extensive at the time of diagnosis that it is not possible to excise.5, 6 Despite inconsistent data regarding chemotherapy, this is usually employed for patients with metastatic or inoperable disease. Doxorubicin-based regimens are most recommended, but recent use of paclitaxel has shown better efficacy compared with doxorubicin.6, 7 Though involvement of the face and scalp is common in cAS, the extent of disease in our case was striking. AS should be considered in the differential diagnosis of violaceous or ecchymotic lesions on the head or scalp in the elderly. All authors contributed to the manuscript conception, design, and writing. All authors read and approved the final manuscript. The authors have not declared a specific grant from any funding agency in the public, commercial or not-for-profit sectors. The authors declare no conflict of interest. This article did not require the approval of the Ethics committee. The patient in this manuscript has given written informed consent to the publication of his case details. Due to privacy and ethical concerns, neither the data nor the source of the data can be made available.
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Mar 19, 2024
L Spelman + 9
Open Access
