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摘要


淋巴管瘤為一罕見發育性異常,主要原因為先天性淋巴組織與主要靜脈系統分離。盛行率約0.12-0.28%,男性與女性均等,頭頸部佔全身之50-75%。在分子生物學角度上,學者發現Proxl藉由調節VEGF receptor-3及podoplanin,繼而使淋巴組織與靜脈系統分離。其依管腔大小可分為:(一)微管型淋巴管瘤(capillary lymphangioma)、(二)靜脈管型淋巴管瘤(cavernous lymphangioma)及(三)囊狀淋巴管瘤(cystic lymphangioma)。Cystic lymphangioma好發於後頸部;而口內較常見者則是capillary lymphangioma或cavernous lymphangioma。治療方式以手術或注射硬化劑Picibanil (OK-432)為主。發生位置是影響預後相當重要的因素,舌骨上的腫瘤不論復發率或併發症均較舌骨下者為高。至於頸部囊狀淋巴管瘤(cervical cystic lymphangioma)大多建議以手術摘除,預後良好。

並列摘要


Lymphangioma is a rare developmental malformation of lymphantic system. The most likely etiologies include failure of the lymphatic system to connect with or separate from the venous system. The incidence of lymphangiomas has been reported to be as low as 1.2 to 2.8 per 1000 and the male to female ratio appears to be equal. Lymphangioma has a marked predilection for head and neck, which accounts for 50% to 75% of all cases. With the increasing prevalence of routine ultrasonography, around 90% of all lesions are diagnosed before 2 years of age. Hong et αl. showed that Proxl expression up-regulates the lymphatic endothelial cell markers podoplanin and VEGF receptor-3 and is a key player in the molecular pathway leading to separation of lymphatic channels from the cardinal vein. There are three types of lymphangioma: (1) capillary lymphangioma, (2) cavernous lymphangioma, and (3) cystic lymphangioma. Cystic lymphangiomas most often occur in the neck while cavernous or capillary lymphangiomas are more often in the mouth. The treatment of lymphangioma usually consists of surgical excision or injection with Picibanil (OK-432). The site of the lesion is the most important determinate for a successful curative operation. Some studies show an increase recurrent rate, morbidity, and complication rate in those lesions presenting in the suprahyoid region. Surgery is recommended for lesions below the level of the hyoid and mostly in the posterior triangle.

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