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Lymphangiosarcoma

MedGen UID:
6154
•Concept ID:
C0024224
•
Neoplastic Process
Synonyms: Lymphangioendothelioma, Malignant; Lymphangioendotheliomas, Malignant; Lymphangiosarcomas; Malignant Lymphangioendothelioma; Malignant Lymphangioendotheliomas
SNOMED CT: Lymphangiosarcoma (63373002); Lymphangioendothelial sarcoma (63373002); Lymphangioendothelioma, malignant (63373002); Malignant lymphangioendothelioma (63373002); Lymphangiosarcoma (403986008)
 
HPO: HP:6000004
Monarch Initiative: MONDO:0006282

Definition

A malignant neoplasm arising from the endothelial cells of the lymphatic vessels. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLymphangiosarcoma

Professional guidelines

PubMed

Stewart-Treves syndrome: pathogenesis and management.
Sharma A, Schwartz RA
J Am Acad Dermatol 2012 Dec;67(6):1342-8. Epub 2012 Jun 8 doi: 10.1016/j.jaad.2012.04.028. PMID: 22682884
Prevention and management of arm lymphedema in the patient with breast cancer.
Golshan M, Smith B
J Support Oncol 2006 Sep;4(8):381-6. PMID: 17004511
The integrated and interdisciplinary treatment of chronic lymphedema.
Doś J, Górska-Doś M, Szuba A
Rocz Akad Med Bialymst 2005;50 Suppl 1:141-4. PMID: 16119649

Recent clinical studies

Etiology

Lymphatic-type "Angiosarcoma" With Prominent Lymphocytic Infiltrate.
Martinez AP, Zapata M, North PE, Folpe AL, Weiss SW
Am J Surg Pathol 2020 Feb;44(2):271-279. doi: 10.1097/PAS.0000000000001398. PMID: 31688141
Morphofunctional basis of the different types of angiogenesis and formation of postnatal angiogenesis-related secondary structures.
Díaz-Flores L, Gutiérrez R, García-Suárez MP, Sáez FJ, Gutiérrez E, Valladares F, Carrasco JL, Díaz-Flores L Jr, Madrid JF
Histol Histopathol 2017 Dec;32(12):1239-1279. Epub 2017 Aug 1 doi: 10.14670/HH-11-923. PMID: 28762232
Prevention and management of arm lymphedema in the patient with breast cancer.
Golshan M, Smith B
J Support Oncol 2006 Sep;4(8):381-6. PMID: 17004511
Lymphangiosarcoma.
Sordillo PP, Chapman R, Hajdu SI, Magill GB, Golbey RB
Cancer 1981 Oct 1;48(7):1674-9. doi: 10.1002/1097-0142(19811001)48:7<1674::aid-cncr2820480733>3.0.co;2-h. PMID: 7284967
Cutaneous lymphangioma.
Flanagan BP, Helwig EB
Arch Dermatol 1977 Jan;113(1):24-30. PMID: 831620

Diagnosis

A retrospective analysis of Stewart-Treves syndrome in the context of chronic lymphedema.
Hao K, Sun Y, Zhu Y, Xin J, Zhang L, Li B, Shen W
An Bras Dermatol 2023 May-Jun;98(3):287-295. Epub 2023 Feb 4 doi: 10.1016/j.abd.2022.04.011. PMID: 36746732Free PMC Article
Stewart-Treves Syndrome.
Bernia E, Rios-Viñuela E, Requena C
JAMA Dermatol 2021 Jun 1;157(6):721. doi: 10.1001/jamadermatol.2021.0341. PMID: 33909015
Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment.
Requena C, Alsina M, Morgado-Carrasco D, Cruz J, Sanmartín O, Serra-Guillén C, Llombart B
Actas Dermosifiliogr (Engl Ed) 2018 Dec;109(10):878-887. Epub 2018 Sep 24 doi: 10.1016/j.ad.2018.06.013. PMID: 30262126
Stewart-Treves syndrome: pathogenesis and management.
Sharma A, Schwartz RA
J Am Acad Dermatol 2012 Dec;67(6):1342-8. Epub 2012 Jun 8 doi: 10.1016/j.jaad.2012.04.028. PMID: 22682884
Lymphedema: classification, diagnosis and therapy.
Szuba A, Rockson SG
Vasc Med 1998;3(2):145-56. doi: 10.1177/1358836X9800300209. PMID: 9796078

Therapy

Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment.
Requena C, Alsina M, Morgado-Carrasco D, Cruz J, Sanmartín O, Serra-Guillén C, Llombart B
Actas Dermosifiliogr (Engl Ed) 2018 Dec;109(10):878-887. Epub 2018 Sep 24 doi: 10.1016/j.ad.2018.06.013. PMID: 30262126
Stewart-Treves syndrome.
Stanczyk M, Gewartowska M
Indian J Med Res 2014 Jan;139(1):179. PMID: 24604055Free PMC Article
Lymphedema-induced lymphangiosarcoma.
Janse AJ, van Coevorden F, Peterse H, Keus RB, van Dongen JA
Eur J Surg Oncol 1995 Apr;21(2):155-8. doi: 10.1016/s0748-7983(95)90270-8. PMID: 7720889
Lymphangiosarcoma.
Sordillo PP, Chapman R, Hajdu SI, Magill GB, Golbey RB
Cancer 1981 Oct 1;48(7):1674-9. doi: 10.1002/1097-0142(19811001)48:7<1674::aid-cncr2820480733>3.0.co;2-h. PMID: 7284967
Vascular and lymphatic tumors in infancy, childhood and adulthood: challenge of diagnosis and treatment.
Edgerton MT, Hiebert JM
Curr Probl Cancer 1978 Jan;2(7):1-44. doi: 10.1016/s0147-0272(78)80001-4. PMID: 373997

Prognosis

Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment.
Requena C, Alsina M, Morgado-Carrasco D, Cruz J, Sanmartín O, Serra-Guillén C, Llombart B
Actas Dermosifiliogr (Engl Ed) 2018 Dec;109(10):878-887. Epub 2018 Sep 24 doi: 10.1016/j.ad.2018.06.013. PMID: 30262126
Stewart-Treves syndrome: pathogenesis and management.
Sharma A, Schwartz RA
J Am Acad Dermatol 2012 Dec;67(6):1342-8. Epub 2012 Jun 8 doi: 10.1016/j.jaad.2012.04.028. PMID: 22682884
Lymphedema-induced lymphangiosarcoma.
Janse AJ, van Coevorden F, Peterse H, Keus RB, van Dongen JA
Eur J Surg Oncol 1995 Apr;21(2):155-8. doi: 10.1016/s0748-7983(95)90270-8. PMID: 7720889
Vascular and lymphatic tumors in infancy, childhood and adulthood: challenge of diagnosis and treatment.
Edgerton MT, Hiebert JM
Curr Probl Cancer 1978 Jan;2(7):1-44. doi: 10.1016/s0147-0272(78)80001-4. PMID: 373997
Cutaneous lymphangioma.
Flanagan BP, Helwig EB
Arch Dermatol 1977 Jan;113(1):24-30. PMID: 831620

Clinical prediction guides

Morphofunctional basis of the different types of angiogenesis and formation of postnatal angiogenesis-related secondary structures.
Díaz-Flores L, Gutiérrez R, García-Suárez MP, Sáez FJ, Gutiérrez E, Valladares F, Carrasco JL, Díaz-Flores L Jr, Madrid JF
Histol Histopathol 2017 Dec;32(12):1239-1279. Epub 2017 Aug 1 doi: 10.14670/HH-11-923. PMID: 28762232
Clinicopathological features of breast angiosarcoma.
Masai K, Kinoshita T, Jimbo K, Asaga S, Hojo T
Breast Cancer 2016 Sep;23(5):718-23. Epub 2015 Aug 5 doi: 10.1007/s12282-015-0630-y. PMID: 26243043
Angiosarcoma (Stewart-Treves syndrome) in postmastectomy patients: report of 10 cases and review of literature.
Cui L, Zhang J, Zhang X, Chang H, Qu C, Zhang J, Zhong D
Int J Clin Exp Pathol 2015;8(9):11108-15. Epub 2015 Sep 1 PMID: 26617830Free PMC Article
Histological differentiation, histogenesis and prognosis of cutaneous angiosarcoma.
Kamo R, Ishii M
Osaka City Med J 2011 Jun;57(1):31-44. PMID: 22106765
The risk of angiosarcoma following primary breast cancer.
Cozen W, Bernstein L, Wang F, Press MF, Mack TM
Br J Cancer 1999 Oct;81(3):532-6. doi: 10.1038/sj.bjc.6690726. PMID: 10507781Free PMC Article

Recent systematic reviews

Stewart-Treves syndrome and other cutaneous malignancies in the context of chronic lymphedema: a systematic review.
Kim PJ, Mufti A, Sachdeva M, Lytvyn Y, Zabihi-Pour D, Zaaroura H, Yeung J
Int J Dermatol 2022 Jan;61(1):62-70. Epub 2021 Jul 1 doi: 10.1111/ijd.15736. PMID: 34196958
Unsuspected Stewart-Treves syndrome clinically mimicked by apparent bullous erysipelas and a systematic review of dermatological presentations of the classical Stewart-Treves syndrome.
da Costa Vieira RA, de Araujo Silva I, de Oliveira-Junior I, de Almeida Santos Yamashita ME, da Silva SRM
Cancer Rep (Hoboken) 2019 Apr;2(2):e1143. Epub 2018 Oct 25 doi: 10.1002/cnr2.1143. PMID: 32721135Free PMC Article

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