Lymphangioma

Definition:

Lymphangiomas are congenital malformations of the lymphatic system, which is located mostly on the head and neck region. The lesions consist of dilated endothelium-lined spaces that vary in size from microscopic channels (cavernous lymphangioma) to large cysts (cystic hygroma). Although histologically benign, these lesions may expand into surrounding tissues and/or infiltrate vital structures similar to malignancies, sometimes causing life-threatening complications. The etiology of lymphangioma is not certain. The presence of lymphatic endothelial cell (LEC)-specific markers on the endothelial lining of lymphangiomas (e.g. the transcription factor Prox1) indicates that the neoplasm may result from transformed LECs and/or stromal cells. This transformation is modulated by VEGF-C, a specific lymphatic endothelial growth factor, and its receptor VEGFR-3.

References:

[1]. J C Oosthuizen, et al. Lymphatic malformations: a proposed management algorithm. Int J Pediatr Otorhinolaryngol. 2010 Apr;74(4):398-403. [Content Brief]

[2]. J Laranne, et al. OK-432 (Picibanil) therapy for lymphangiomas in children. Eur Arch Otorhinolaryngol. 2002 May;259(5):274-8. [Content Brief]

[3]. Rui-Cheng Ji, et al. Multiple expressions of lymphatic markers and morphological evolution of newly formed lymphatics in lymphangioma and lymph node lymphangiogenesis. Microvasc Res. 2010 Sep;80(2):195-201. [Content Brief]

[4]. S Ogita, et al. OK-432 therapy for lymphangioma in children: why and how does it work?. J Pediatr Surg. 1996 Apr;31(4):477-80. [Content Brief]

[5]. S Ogita, et al. OK-432 therapy in 64 patients with lymphangioma. J Pediatr Surg. 1994 Jun;29(6):784-5. [Content Brief]

[6]. Susanne Norgall, et al. Elevated expression of VEGFR-3 in lymphatic endothelial cells from lymphangiomas. BMC Cancer. 2007 Jun 21;7:105. [Content Brief]

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