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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

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVLymphangiosarcoma

Phenotypic abnormality
- Abnormality of the immune system
  - Abnormality of the lymphatic system
    - Abnormal lymphatic vessel morphology
      - Lymphatic vessel neoplasm
        Lymphangiosarcoma
        Skin lymphangiosarcoma

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

See all (6)

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

Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients.

Chung KC, Kim HJ, Jeffers LL
J Hand Surg Am 2000 Nov;25(6):1163-8. doi: 10.1053/jhsu.2000.18490. PMID: 11119680

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

See all (39)

Diagnosis

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

Lymphangiosarcoma following mastectomy.

Stewart NJ, Pritchard DJ, Nascimento AG, Kang YK
Clin Orthop Relat Res 1995 Nov;(320):135-41. PMID: 7586817

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

See all (93)

Therapy

Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment.

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 following mastectomy.

Stewart NJ, Pritchard DJ, Nascimento AG, Kang YK
Clin Orthop Relat Res 1995 Nov;(320):135-41. PMID: 7586817

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

See all (73)

Prognosis

Kaposi Sarcoma and Cutaneous Angiosarcoma: Guidelines for Diagnosis and Treatment.

Lymphangiosarcoma of the scalp.

Mamelak AJ, Collie MR, Kroll R, Hanson ML, Harris HM
J Cutan Med Surg 2014 Mar-Apr;18(2):132-6. doi: 10.2310/7750.2013.13025. PMID: 24636440

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

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

See all (80)

Clinical prediction guides

Secondary angiosarcoma: A fatal complication of chronic lymphedema.

Farzaliyev F, Hamacher R, Steinau Professor HU, Bertram S, Podleska LE
J Surg Oncol 2020 Jan;121(1):85-90. Epub 2019 Jun 24 doi: 10.1002/jso.25598. PMID: 31236970

Morphofunctional basis of the different types of angiogenesis and formation of postnatal angiogenesis-related secondary structures.

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: 26617830 Free PMC Article

Complex karyotype in a case of cutaneous lymphangiosarcoma associated with chronic lymphedema of the lower limb.

Marando A, Bernasconi B, Sabatino D, Militti L, Capella C
Pathol Res Pract 2014 Dec;210(12):1138-41. Epub 2014 May 16 doi: 10.1016/j.prp.2014.04.021. PMID: 24947410

See all (21)

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: 32721135 Free PMC Article

See all (2)

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Lymphangiosarcoma

Definition

Term Hierarchy

Professional guidelines

PubMed

Recent clinical studies

Etiology

Diagnosis

Therapy

Prognosis

Clinical prediction guides

Recent systematic reviews

Supplemental Content

Table of contents

Clinical resources

Practice guidelines

Consumer resources

Reviews

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