| H01471 | |
| H number | H01471 |
| Name | Lymphangioma |
| Description | 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. |
| Category | Neoplasm |
| Network | - |
| Gene | VEGFR-3 (overexpression) [HSA:2324] [KO:K05097] |
| Pathogen | - |
| Env factor | - |
| Carcinogen | - |
| Drug | - |
| Comment | - |
| Other DBs | ICD-11: 2E81.1 ICD-10: D18.1 MeSH: D008202 |
| Reference | PMID:8801295 AUTHORS Ogita S, Tsuto T, Nakamura K, Deguchi E, Tokiwa K, Iwai N TITLE OK-432 therapy for lymphangioma in children: why and how does it work? JOURNAL J Pediatr Surg 31:477-80 (1996) DOI:10.1016/S0022-3468(96)90478-9 PMID:8078021 AUTHORS Ogita S, Tsuto T, Nakamura K, Deguchi E, Iwai N TITLE OK-432 therapy in 64 patients with lymphangioma. JOURNAL J Pediatr Surg 29:784-5 (1994) DOI:10.1016/0022-3468(94)90370-0 PMID:20382171 AUTHORS Ji RC, Eshita Y, Xing L, Miura M TITLE Multiple expressions of lymphatic markers and morphological evolution of newly formed lymphatics in lymphangioma and lymph node lymphangiogenesis. JOURNAL Microvasc Res 80:195-201 (2010) DOI:10.1016/j.mvr.2010.04.002 PMID:17584927 AUTHORS Norgall S, Papoutsi M, Rossler J, Schweigerer L, Wilting J, Weich HA TITLE Elevated expression of VEGFR-3 in lymphatic endothelial cells from lymphangiomas. JOURNAL BMC Cancer 7:105 (2007) DOI:10.1186/1471-2407-7-105 PMID:12107533 AUTHORS Laranne J, Keski-Nisula L, Rautio R, Rautiainen M, Airaksinen M TITLE OK-432 (Picibanil) therapy for lymphangiomas in children. JOURNAL Eur Arch Otorhinolaryngol 259:274-8 (2002) DOI:10.1007/s00405-001-0438-6 PMID:20170968 AUTHORS Oosthuizen JC, Burns P, Russell JD TITLE Lymphatic malformations: a proposed management algorithm. JOURNAL Int J Pediatr Otorhinolaryngol 74:398-403 (2010) DOI:10.1016/j.ijporl.2010.01.013 |