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Cavernous lymphangioma

MedGen UID:
104729
Concept ID:
C0205828
Neoplastic Process
Synonym: Macrocystic lymphatic malformation
SNOMED CT: Cavernous lymphangioma (89056007); Cavernous lymphangioma (1157156002); Macrocystic lymphatic malformation (1157156002); Cavernous lymphatic malformation (1157156002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019328
Orphanet: ORPHA79489

Definition

A rare common cystic lymphatic malformation characterised by a benign cystic lesion composed of dilated lymphatic channels. Macrocystic lesions consist of cysts larger than 1 cm in diameter. They usually present at birth or during the first years of life and most often occur in the head and neck region but may affect any site. Symptoms depend on the location and extent of the lesion. Infection, trauma, or intracystic haemorrhage can lead to lesional expansion. Malignant transformation does not occur. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Cavernous lymphangioma

Professional guidelines

PubMed

Usachev DY, Lukshin VA, Akhmedov AD, Shulgina AA, Ogurtsova AA, Pronin IN, Yakovlev SB
Zh Vopr Neirokhir Im N N Burdenko 2023;87(5):8-20. doi: 10.17116/neiro2023870518. PMID: 37830464
Zhao ZF, Kuang L, Zhang N, Ma SR, Yang Z, Han X, Zhao YF, Gao F, Gong ZJ, Yang L
Surg Laparosc Endosc Percutan Tech 2013 Jun;23(3):299-302. doi: 10.1097/SLE.0b013e31828b8810. PMID: 23751996

Recent clinical studies

Etiology

Assaad M, Acharya R, Shamsi W, Springsted E, Foroozesh M, Del Mar Cirino-Marcano M
BMC Pulm Med 2025 Apr 28;25(1):202. doi: 10.1186/s12890-025-03664-3. PMID: 40296055Free PMC Article
Zhao ZF, Kuang L, Zhang N, Ma SR, Yang Z, Han X, Zhao YF, Gao F, Gong ZJ, Yang L
Surg Laparosc Endosc Percutan Tech 2013 Jun;23(3):299-302. doi: 10.1097/SLE.0b013e31828b8810. PMID: 23751996
Urs AB, Shetty D, Praveen Reddy B, Sikka S
Minerva Stomatol 2011 Mar;60(3):149-53. PMID: 21270741
Quack Loetscher KC, Jandali AR, Garzoli E, Pok J, Beinder E
Gynecol Obstet Invest 2005;60(2):108-11. Epub 2005 May 2 doi: 10.1159/000085584. PMID: 15867472
Blair WF, Buckwalter JA, Mickelson MR, Omer GE
J Hand Surg Am 1983 Jul;8(4):399-405. doi: 10.1016/s0363-5023(83)80198-1. PMID: 6350419

Diagnosis

Usachev DY, Lukshin VA, Akhmedov AD, Shulgina AA, Ogurtsova AA, Pronin IN, Yakovlev SB
Zh Vopr Neirokhir Im N N Burdenko 2023;87(5):8-20. doi: 10.17116/neiro2023870518. PMID: 37830464
Lan J, Yang J, Lin A, Liu H, Liao J, Huang W
Pol Arch Intern Med 2023 Feb 26;133(2) Epub 2022 Dec 5 doi: 10.20452/pamw.16380. PMID: 36468694
Manekar AA, Janjala N, Sahoo SK, Tripathy BB, Mohanty MK
J Indian Soc Pedod Prev Dent 2022 Jan-Mar;40(1):86-89. doi: 10.4103/jisppd.jisppd_189_21. PMID: 35439889
Zhao ZF, Kuang L, Zhang N, Ma SR, Yang Z, Han X, Zhao YF, Gao F, Gong ZJ, Yang L
Surg Laparosc Endosc Percutan Tech 2013 Jun;23(3):299-302. doi: 10.1097/SLE.0b013e31828b8810. PMID: 23751996
Morris-Stiff G, Falk GA, El-Hayek K, Vargo J, Bronner M, Vogt DP
BMJ Case Rep 2011 May 12;2011 doi: 10.1136/bcr.03.2011.4022. PMID: 22696733Free PMC Article

Therapy

Kobayashi D, Kumagai H, Satsuma S
J Bone Joint Surg Br 2003 Aug;85(6):891-4. PMID: 12931814

Prognosis

Xu X, Liu W, Zheng C
Gastroenterology 2014 Apr;146(4):e11-3. Epub 2014 Feb 24 doi: 10.1053/j.gastro.2013.11.040. PMID: 24576729
Zhao ZF, Kuang L, Zhang N, Ma SR, Yang Z, Han X, Zhao YF, Gao F, Gong ZJ, Yang L
Surg Laparosc Endosc Percutan Tech 2013 Jun;23(3):299-302. doi: 10.1097/SLE.0b013e31828b8810. PMID: 23751996
Murase T, Tsuyuguchi Y, Doi T, Kawai H, Masada K
J Pediatr Orthop 1992 Jan;12(1):100-5. PMID: 1732286
Brown JV, Stenchever MA
Obstet Gynecol 1989 May;73(5 Pt 2):877-9. PMID: 2704522
Blair WF, Buckwalter JA, Mickelson MR, Omer GE
J Hand Surg Am 1983 Jul;8(4):399-405. doi: 10.1016/s0363-5023(83)80198-1. PMID: 6350419

Clinical prediction guides

Usachev DY, Lukshin VA, Akhmedov AD, Shulgina AA, Ogurtsova AA, Pronin IN, Yakovlev SB
Zh Vopr Neirokhir Im N N Burdenko 2023;87(5):8-20. doi: 10.17116/neiro2023870518. PMID: 37830464
Xu X, Liu W, Zheng C
Gastroenterology 2014 Apr;146(4):e11-3. Epub 2014 Feb 24 doi: 10.1053/j.gastro.2013.11.040. PMID: 24576729
Choi JY, Kim MJ, Chung JJ, Park SI, Lee JT, Yoo HS, Kim L, Choi JS
Abdom Imaging 2002 Jan-Feb;27(1):54-7. doi: 10.1007/s00261-001-0051-6. PMID: 11740609
Saito T, Terada K, Tsuchiya K, Oda Y, Tsuneyoshi M, Iwamoto Y
Spine (Phila Pa 1976) 1999 Jan 1;24(1):74-6. doi: 10.1097/00007632-199901010-00018. PMID: 9921595
Blair WF, Buckwalter JA, Mickelson MR, Omer GE
J Hand Surg Am 1983 Jul;8(4):399-405. doi: 10.1016/s0363-5023(83)80198-1. PMID: 6350419

Recent systematic reviews

Assaad M, Acharya R, Shamsi W, Springsted E, Foroozesh M, Del Mar Cirino-Marcano M
BMC Pulm Med 2025 Apr 28;25(1):202. doi: 10.1186/s12890-025-03664-3. PMID: 40296055Free PMC Article

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