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Lymphangioma

MedGen UID:
6153
Concept ID:
C0024221
Neoplastic Process
Synonym: Lymphangiomas
SNOMED CT: Lymphangioma (400178008); Benign lymphangioma (253057002); Congenital lymphangioma (254836000); Lymphangioendothelioma (253057002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
HPO: HP:0100764
Monarch Initiative: MONDO:0002013
Orphanet: ORPHA2415

Definition

Lymphangiomas are rare congenital malformations consisting of focal proliferations of well-differentiated lymphatic tissue in multi cystic or sponge like structures. Lymphangioma is usually asymptomatic due to its soft consistency but compression of adjacent structures can be seen due to the mass effect of a large tumor. [from HPO]

Conditions with this feature

Angioosteohypertrophic syndrome
MedGen UID:
9646
Concept ID:
C0022739
Disease or Syndrome
Klippel-Trenaunay syndrome is a condition that affects the development of blood vessels, soft tissues (such as skin and muscles), and bones. The disorder has three characteristic features: a red birthmark called a port-wine stain, abnormal overgrowth of soft tissues and bones, and vein malformations.\n\nMost people with Klippel-Trenaunay syndrome are born with a port-wine stain. This type of birthmark is caused by swelling of small blood vessels near the surface of the skin. Port-wine stains are typically flat and can vary from pale pink to deep maroon in color. In people with Klippel-Trenaunay syndrome, the port-wine stain usually covers part of one limb. The affected area may become lighter or darker with age. Occasionally, port-wine stains develop small red blisters that break open and bleed easily.\n\nKlippel-Trenaunay syndrome is also associated with overgrowth of bones and soft tissues beginning in infancy. Usually this abnormal growth is limited to one limb, most often one leg. However, overgrowth can also affect the arms or, rarely, the torso. The abnormal growth can cause pain, a feeling of heaviness, and reduced movement in the affected area. If the overgrowth causes one leg to be longer than the other, it can also lead to problems with walking.\n\nMalformations of veins are the third major feature of Klippel-Trenaunay syndrome. These abnormalities include varicose veins, which are swollen and twisted veins near the surface of the skin that often cause pain. Varicose veins usually occur on the sides of the upper legs and calves. Veins deep in the limbs can also be abnormal in people with Klippel-Trenaunay syndrome. Malformations of deep veins increase the risk of a type of blood clot called a deep vein thrombosis (DVT). If a DVT travels through the bloodstream and lodges in the lungs, it can cause a life-threatening blood clot known as a pulmonary embolism (PE).\n\nOther complications of Klippel-Trenaunay syndrome can include a type of skin infection called cellulitis, swelling caused by a buildup of fluid (lymphedema), and internal bleeding from abnormal blood vessels. Less commonly, this condition is also associated with fusion of certain fingers or toes (syndactyly) or the presence of extra digits (polydactyly).
Proteus syndrome
MedGen UID:
39008
Concept ID:
C0085261
Neoplastic Process
Proteus syndrome is characterized by progressive segmental or patchy overgrowth most commonly affecting the skeleton, skin, adipose, and central nervous systems. In most individuals Proteus syndrome has modest or no manifestations at birth, develops and progresses rapidly beginning in the toddler period, and relentlessly progresses through childhood, causing severe overgrowth and disfigurement. It is associated with a range of tumors, pulmonary complications, and a striking predisposition to deep vein thrombosis and pulmonary embolism.

Professional guidelines

PubMed

Jiao-Ling L, Hai-Ying W, Wei Z, Jin-Rong L, Kun-Shan C, Qian F
Eur J Obstet Gynecol Reprod Biol 2018 Dec;231:274-279. Epub 2018 Oct 15 doi: 10.1016/j.ejogrb.2018.10.031. PMID: 30482553
Li JL, Hai-Ying W, Liu JR, He QM, Chen KS, Yang J, Qian F
Eur J Obstet Gynecol Reprod Biol 2018 Dec;231:268-273. Epub 2018 Oct 9 doi: 10.1016/j.ejogrb.2018.10.018. PMID: 30343933
Ha J, Yu YC, Lannigan F
Curr Pediatr Rev 2014;10(3):238-48. doi: 10.2174/1573396309666131209210751. PMID: 25088344

Recent clinical studies

Etiology

Chiang J, Woo JE, Vu AH, Sirsi S
BMJ Case Rep 2024 May 8;17(5) doi: 10.1136/bcr-2023-258099. PMID: 38719266Free PMC Article
Hathaway BA, Radu S, Wilson J, Nauta AC
Lymphat Res Biol 2021 Feb;19(1):41-50. Epub 2021 Jan 25 doi: 10.1089/lrb.2020.0100. PMID: 33493408
Jiao-Ling L, Hai-Ying W, Wei Z, Jin-Rong L, Kun-Shan C, Qian F
Eur J Obstet Gynecol Reprod Biol 2018 Dec;231:274-279. Epub 2018 Oct 15 doi: 10.1016/j.ejogrb.2018.10.031. PMID: 30482553
Kyogoku M, Inata Y, Takeuchi M
J Pediatr 2017 Oct;189:238. Epub 2017 Jul 20 doi: 10.1016/j.jpeds.2017.06.065. PMID: 28735978
Flanagan BP, Helwig EB
Arch Dermatol 1977 Jan;113(1):24-30. PMID: 831620

Diagnosis

Chiang J, Woo JE, Vu AH, Sirsi S
BMJ Case Rep 2024 May 8;17(5) doi: 10.1136/bcr-2023-258099. PMID: 38719266Free PMC Article
Mede A, Chotai PN, Huh WJ, Tan M
J Surg Res 2023 May;285:197-204. Epub 2023 Jan 23 doi: 10.1016/j.jss.2022.12.026. PMID: 36696706
Hoang VT, Nguyen MD, Van HAT, Hoang DT
Jpn J Radiol 2023 Mar;41(3):283-301. Epub 2022 Nov 3 doi: 10.1007/s11604-022-01356-0. PMID: 36327088
Jiao-Ling L, Hai-Ying W, Wei Z, Jin-Rong L, Kun-Shan C, Qian F
Eur J Obstet Gynecol Reprod Biol 2018 Dec;231:274-279. Epub 2018 Oct 15 doi: 10.1016/j.ejogrb.2018.10.031. PMID: 30482553
Mordehai J, Kurzbart E, Shinhar D, Sagi A, Finaly R, Mares AJ
Pediatr Surg Int 1998 Mar;13(2-3):208-10. doi: 10.1007/s003830050295. PMID: 9563054

Therapy

Yu J, Tang J, Chen M, Ren Q, He J, Tang M, Zhang X, Liu Z, Ding H
J Clin Neurosci 2023 Jan;107:23-33. Epub 2022 Nov 30 doi: 10.1016/j.jocn.2022.11.010. PMID: 36462413
Wiegand S, Dietz A, Wichmann G
Eur Arch Otorhinolaryngol 2022 Aug;279(8):3801-3810. Epub 2022 May 8 doi: 10.1007/s00405-022-07378-8. PMID: 35526176Free PMC Article
Ashack KA, Kuritza V, Visconti MJ, Ashack L
Am J Clin Dermatol 2020 Aug;21(4):541-555. doi: 10.1007/s40257-020-00519-x. PMID: 32410134
Patel SR, Rosenberg JB, Barmettler A
Cochrane Database Syst Rev 2019 May 15;5(5):CD013000. doi: 10.1002/14651858.CD013000.pub2. PMID: 31094450Free PMC Article
Bagrodia N, Defnet AM, Kandel JJ
Curr Opin Pediatr 2015 Jun;27(3):356-63. doi: 10.1097/MOP.0000000000000209. PMID: 25888145

Prognosis

Mede A, Chotai PN, Huh WJ, Tan M
J Surg Res 2023 May;285:197-204. Epub 2023 Jan 23 doi: 10.1016/j.jss.2022.12.026. PMID: 36696706
Jiao-Ling L, Hai-Ying W, Wei Z, Jin-Rong L, Kun-Shan C, Qian F
Eur J Obstet Gynecol Reprod Biol 2018 Dec;231:274-279. Epub 2018 Oct 15 doi: 10.1016/j.ejogrb.2018.10.031. PMID: 30482553
Li JL, Hai-Ying W, Liu JR, He QM, Chen KS, Yang J, Qian F
Eur J Obstet Gynecol Reprod Biol 2018 Dec;231:268-273. Epub 2018 Oct 9 doi: 10.1016/j.ejogrb.2018.10.018. PMID: 30343933
Chandrasekaran A
Pediatr Neonatol 2018 Feb;59(1):65-70. Epub 2017 Jul 11 doi: 10.1016/j.pedneo.2016.12.007. PMID: 28736178
Ioannidis I, Kahn AG
Arch Pathol Lab Med 2015 Feb;139(2):278-82. doi: 10.5858/arpa.2013-0656-RS. PMID: 25611113

Clinical prediction guides

Patel SR, Rosenberg JB, Barmettler A
Cochrane Database Syst Rev 2019 May 15;5(5):CD013000. doi: 10.1002/14651858.CD013000.pub2. PMID: 31094450Free PMC Article
Zhang J, Li J, Saucier JB, Feng Y, Jiang Y, Sinson J, McCombs AK, Schmitt ES, Peacock S, Chen S, Dai H, Ge X, Wang G, Shaw CA, Mei H, Breman A, Xia F, Yang Y, Purgason A, Pourpak A, Chen Z, Wang X, Wang Y, Kulkarni S, Choy KW, Wapner RJ, Van den Veyver IB, Beaudet A, Parmar S, Wong LJ, Eng CM
Nat Med 2019 Mar;25(3):439-447. Epub 2019 Jan 28 doi: 10.1038/s41591-018-0334-x. PMID: 30692697
Ha J, Yu YC, Lannigan F
Curr Pediatr Rev 2014;10(3):238-48. doi: 10.2174/1573396309666131209210751. PMID: 25088344
Sanger C, Wong L, Wood J, David LR, Argenta LC
J Craniofac Surg 2011 Jul;22(4):1271-4. doi: 10.1097/SCS.0b013e31821c6cf1. PMID: 21772195
Wright JE, Sullivan TJ, Garner A, Wulc AE, Moseley IF
Ophthalmology 1997 Jun;104(6):905-13. doi: 10.1016/s0161-6420(97)30208-5. PMID: 9186428

Recent systematic reviews

Yu J, Tang J, Chen M, Ren Q, He J, Tang M, Zhang X, Liu Z, Ding H
J Clin Neurosci 2023 Jan;107:23-33. Epub 2022 Nov 30 doi: 10.1016/j.jocn.2022.11.010. PMID: 36462413
Wiegand S, Dietz A, Wichmann G
Eur Arch Otorhinolaryngol 2022 Aug;279(8):3801-3810. Epub 2022 May 8 doi: 10.1007/s00405-022-07378-8. PMID: 35526176Free PMC Article
Patel SR, Rosenberg JB, Barmettler A
Cochrane Database Syst Rev 2019 May 15;5(5):CD013000. doi: 10.1002/14651858.CD013000.pub2. PMID: 31094450Free PMC Article
Cheng J
J Pediatr Surg 2015 Dec;50(12):2143-6. Epub 2015 Sep 4 doi: 10.1016/j.jpedsurg.2015.08.051. PMID: 26421368
Adams MT, Saltzman B, Perkins JA
Otolaryngol Head Neck Surg 2012 Oct;147(4):627-39. Epub 2012 Jul 11 doi: 10.1177/0194599812453552. PMID: 22785242

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