KEGG   DISEASE: LymphangiomaHelp
Entry
H01471                      Disease                                

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
Vascular disease
Brite
Human diseases [BR:br08402]
 Cardiovascular diseases
  Vascular diseases
   H01471  Lymphangioma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Benign neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Benign mesenchymal neoplasms
    2E81  Benign vascular neoplasms
     H01471  Lymphangioma
BRITE hierarchy
Gene
VEGFR-3 (overexpression) [HSA:2324] [KO:K05097]
Comment
ICD-O-3.1: 9170/0 Lymphangioma, NOS
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
Reference
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
Reference
  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
Reference
  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
Reference
PMID:12107533 (drug)
  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
Reference
PMID:20170968 (drug)
  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

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