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Research article | DOI: https://doi.org/10.31579/2578-8868/227
1Head of Department of Neurosurgery Bejaia Hospital, Bejaia, Algeria
2Neurosurgery Department, Mustapha Pacha University Hospital, Algiers, Algeria
*Corresponding Author: Bekralas H., Head of Department of Neurosurgery Bejaia Hospital,Bejaia, Algeria
Citation: Bekralas H., Habchi N. (2022). Angiosarcoma of The Scalp: A Case Report. J. Neuroscience and Neurological Surgery. 11(2); DOI:10.31579/2578-8868/227
Copyright: © 2022 Bekralas H, This is an open-access article distributed under the terms of The Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited
Received: 10 December 2021 | Accepted: 30 December 2021 | Published: 03 January 2022
Keywords: angiosarcomas ; scalp
Angiosarcomas also called hemangioendothelia, are rare and highly malignant vascular tumors of mesodermal origin, they represent 08-10% of cancers and can affect any part of the body.Angiosarcoma mainly affects the skin, the face is the preferred topography in adults, it remains exceptional in children, with a predilection for the mediastinum and pericardium,The angiogenesis of angiosarcomas is still poorly understood; However, chronic lymphedema and exposure to prolonged radiotherapy are blamed. Total surgical excision is the treatment of choice in localized forms, followed by chemotherapy. pre- and post-operative radiotherapy may be necessary.New studies have shown the effectiveness of beta blockers (propranolol) in the management of angiosarcomas.The prognosis depends on the age of the patient, the size of the tumor, the histological grade and the extent of tumor progression.
Cutaneous angiosarcomas are rare, aggressive vascular tumors with a poor prognosis; they represent approximately 1 to 5% of soft tissue sarcomas in adults and 0.2 to 0.3 in children.WILSON – JONES angiosarcoma of the head and neck is a peculiar and exceptional topographic form in children. Congenital or intrauterine forms have been described in the literature
We report a rare case of congenital angiosarcoma of the scalp in a newborn 7 days old, referred to our level by his pediatrician following the observation of an occipital lesion which rapidly increases in size.The clinical examination found a conscious newborn, general condition preserved with skin-mucous paleness, in relation to anemia at 7g / dl. Clinical examination finds a large, coarsely rounded mass, well skinned, hard, purplish, hyper vascularized and extended on the occipital surface, of progressive appearance. (Figure 01)
Cerebral computed tomography and brain angiography revealed a subcutaneous occipital lesion measuring 55 * 63mm richly vascularized supplied by the branches of the occipital artery with infiltration of the galea and the external table. (Figure 02)
The pathological examination concludes with angiosarcoma, an orientation in oncology for an adapted chemotherapy made. (Figure 03)
The course at 1 year postoperatively is favorable, no signs of local or metastatic recurrence.
Angiosarcomas are highly malignant tumors, the cutaneous form mainly affects the elderly, it is exceptional in children.The antenatal diagnosis confirms the congenital nature of our elucidated case.The rarity of congenital forms makes it difficult to institute an adequate therapeutic protocol.The definitive diagnosis of angiosarcoma is histological.The management is multidisciplinary: surgical treatment is the treatment of choice, it is generally followed by adjuvant treatment based on chemotherapy and radiotherapy.Despite advances in neuro-oncology, the prognosis remains bleak.
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