Maseda Eduardo, Blanco Rafael, Ablanedo Armando, Iglesias Eduardo
Servicio de Otorrinolaringología, Hospital San Agustín, SESPA, Avilés, España.
Acta Otorrinolaringol Esp. 2008 Apr;59(4):198-9.
The kaposiform hemangioendothelioma is a very infrequent tumor proceeding from the endothelial-derived spindle cells, more often found on the limbs, although peritoneal, retroperitoneal and sacrum locations are also prevailing. Head and neck are exceptional locations. The kaposiform hemangioendothelioma is almost exclusively found in children and early adolescents, and it is highly associated with the Kasabach-Merritt syndrome and lymphangiomatosis. The main treatment is the tumor surgical removal, including wide margins, plus supporting therapy when Kasabach-Merritt syndrome is linked. We report an isolated oropharyngeal kaposiform hemangioendothelioma 9-10-year old male case.
卡波西样血管内皮瘤是一种非常罕见的肿瘤,起源于内皮来源的梭形细胞,更常见于四肢,尽管腹膜、腹膜后和骶骨部位也较为常见。头颈部是例外部位。卡波西样血管内皮瘤几乎仅见于儿童和青少年早期,且与卡萨巴赫-梅里特综合征和淋巴管瘤病高度相关。主要治疗方法是手术切除肿瘤,包括广泛切缘,当伴有卡萨巴赫-梅里特综合征时加用支持治疗。我们报告一例9至10岁男性孤立性口咽卡波西样血管内皮瘤病例。