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Lymphangioendothelioma

MedGen UID:
7411
•Concept ID:
C0024217
•
Neoplastic Process
Synonyms: Endothelioma, Lymphatic; Endotheliomas, Lymphatic; Lymphangioendotheliomas; Lymphatic Endothelioma; Lymphatic Endotheliomas
SNOMED CT: Acquired progressive lymphangioma (403975006); Benign lymphangioendothelioma (403975006)
 
Monarch Initiative: MONDO:0006841

Definition

A lymphangioma characterized by the presence of collagen bundle formation. It has an indolent clinical course and may be associated with skin plaques. [from NCI]

Professional guidelines

PubMed

Head and neck lymphatic malformation treatment: a systematic review.
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

Recent clinical studies

Etiology

Post-mastectomy benign lymphangioendothelioma of the skin following chronic lymphedema for breast carcinoma: a teaching case mimicking low-grade angiosarcoma and masquerading as Stewart-Treves syndrome.
Yamada S, Yamada Y, Kobayashi M, Hino R, Nawata A, Noguchi H, Nakamura M, Nakayama T
Diagn Pathol 2014 Oct 29;9:197. doi: 10.1186/s13000-014-0197-5. PMID: 25358645Free PMC Article
Vascular proliferations of the skin after radiation therapy for breast cancer: clinicopathologic analysis of a series in favor of a benign process: a study from the French Sarcoma Group.
Gengler C, Coindre JM, Leroux A, Trassard M, Ranchère-Vince D, Valo I, Michels JJ, Guillou L
Cancer 2007 Apr 15;109(8):1584-98. doi: 10.1002/cncr.22586. PMID: 17357996
Vascular anomalies of the female external genitalia.
Vogel AM, Alesbury JM, Burrows PE, Fishman SJ
J Pediatr Surg 2006 May;41(5):993-9. doi: 10.1016/j.jpedsurg.2005.12.069. PMID: 16677899
Cutaneous vascular proliferation. Part II. Hyperplasias and benign neoplasms.
Requena L, Sangueza OP
J Am Acad Dermatol 1997 Dec;37(6):887-919; quiz 920-2. doi: 10.1016/s0190-9622(97)70065-3. PMID: 9418757

Diagnosis

Unilateral swelling and hyperpigmented papules.
Ajayi A, Sokumbi O
Dermatol Online J 2021 Dec 15;27(12) doi: 10.5070/D3271256716. PMID: 35499446
Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series.
Guillou L, Fletcher CD
Am J Surg Pathol 2000 Aug;24(8):1047-57. doi: 10.1097/00000478-200008000-00002. PMID: 10935645
Benign lymphangioendothelioma.
Herron GS, Rouse RV, Kosek JC, Smoller BR, Egbert BM
J Am Acad Dermatol 1994 Aug;31(2 Pt 2):362-8. doi: 10.1016/s0190-9622(94)70173-3. PMID: 7518477
Recently characterized vascular tumours of skin and soft tissues.
Tsang WY, Chan JK, Fletcher CD
Histopathology 1991 Dec;19(6):489-501. doi: 10.1111/j.1365-2559.1991.tb01497.x. PMID: 1786935
Benign lymphangioendothelioma.
Jones EW, Winkelmann RK, Zachary CB, Reda AM
J Am Acad Dermatol 1990 Aug;23(2 Pt 1):229-35. doi: 10.1016/0190-9622(90)70203-t. PMID: 2212118

Therapy

Pediatric case of acquired progressive lymphatic anomaly treated with sirolimus.
Komamizu S, Ozeki M, Hayashi D, Endo S, Hori-Hirose Y, Sasaki S, Ohnishi H
Pediatr Int 2023 Jan;65(1):e15497. doi: 10.1111/ped.15497. PMID: 36749179
A case of extensive acquired progressive lymphangioma.
Larkin SC, Wentworth AB, Lehman JS, Tollefson MM
Pediatr Dermatol 2018 Jul;35(4):486-489. Epub 2018 Apr 6 doi: 10.1111/pde.13486. PMID: 29633311
Acquired progressive lymphangioma: Case report with partial response to imiquimod 5% cream.
Salman A, Sarac G, Can Kuru B, Cinel L, Yucelten AD, Ergun T
Pediatr Dermatol 2017 Nov;34(6):e302-e304. Epub 2017 Sep 21 doi: 10.1111/pde.13283. PMID: 28940760
Head and neck lymphatic malformation treatment: a systematic review.
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
Vascular proliferations of the skin after radiation therapy for breast cancer: clinicopathologic analysis of a series in favor of a benign process: a study from the French Sarcoma Group.
Gengler C, Coindre JM, Leroux A, Trassard M, Ranchère-Vince D, Valo I, Michels JJ, Guillou L
Cancer 2007 Apr 15;109(8):1584-98. doi: 10.1002/cncr.22586. PMID: 17357996

Prognosis

Unilateral swelling and hyperpigmented papules.
Ajayi A, Sokumbi O
Dermatol Online J 2021 Dec 15;27(12) doi: 10.5070/D3271256716. PMID: 35499446
Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series.
Guillou L, Fletcher CD
Am J Surg Pathol 2000 Aug;24(8):1047-57. doi: 10.1097/00000478-200008000-00002. PMID: 10935645
Lymphangioma-like variant of Kaposi's sarcoma: clinicopathologic study of seven cases with review of the literature.
Cossu S, Satta R, Cottoni F, Massarelli G
Am J Dermatopathol 1997 Feb;19(1):16-22. doi: 10.1097/00000372-199702000-00004. PMID: 9056649

Clinical prediction guides

Giant benign lymphangioendothelioma with positive expression of Wilms tumor 1: A case report.
Teixeira D, Canelhas Á, Costa M, Magalhães C, Ferreira EO, César A
J Cutan Pathol 2022 Jan;49(1):86-89. Epub 2021 Sep 6 doi: 10.1111/cup.14125. PMID: 34423872
Benign lymphangioendothelioma presenting as a giant flank mass.
Schnebelen AM, Page J, Gardner JM, Shalin SC
J Cutan Pathol 2015 Mar;42(3):217-221. Epub 2014 Dec 23 doi: 10.1111/cup.12453. PMID: 25410589
Vascular anomalies of the female external genitalia.
Vogel AM, Alesbury JM, Burrows PE, Fishman SJ
J Pediatr Surg 2006 May;41(5):993-9. doi: 10.1016/j.jpedsurg.2005.12.069. PMID: 16677899
Benign lymphangioendothelioma (acquired progressive lymphangioma): a lesion not to be confused with well-differentiated angiosarcoma and patch stage Kaposi's sarcoma: clinicopathologic analysis of a series.
Guillou L, Fletcher CD
Am J Surg Pathol 2000 Aug;24(8):1047-57. doi: 10.1097/00000478-200008000-00002. PMID: 10935645

Recent systematic reviews

Head and neck lymphatic malformation treatment: a systematic review.
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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