95:, as a treatment for breast cancer. The classical radical mastectomy was abandoned in most areas of the world in the late 1960s to early 1970s, being replaced by the much more conservative modified radical mastectomy and, more recently, by segmental breast tissue excision and radiation therapy. Because of this change in clinical practice lymphedema is now a rarity following breast cancer treatment—and post-mastectomy lymphangiosarcoma is now vanishingly rare. When it occurs following mastectomy it is known as
42:
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Lymphangiosarcoma may present as a purple discoloration or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting to an extensive necrotic focus involving the skin and subcutaneous tissue. It metastasizes quickly. Lymphangiosarcoma mostly
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Gambini D, Visintin R, Locatelli E, Galassi B, Bareggi C, Runza L, Onida F, Tomirotti M.Tumori. 2009 Nov–Dec;95(6):828–31. Paclitaxel-dependent prolonged and persistent complete remission four years from first recurrence of secondary breast angiosarcoma. Medical
Oncology Unit, IRCCS Foundation
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occurs in the upper arm, forearm, elbow, and anterior chest wall. Furthermore, in post-mastectomy patients, lymphangiosarcoma is associated with sarcomatous degeneration, especially in patients who have survived five or more years and who have severe lymphedema.
99:. The pathogenesis of lymphangiosarcoma has not been resolved, however several vague mechanisms have been proposed. Stewart and Treves, proposed that a cancer causing agent is present in lymphedematous limbs. Schreiber
158:. Evidence supporting the effectiveness of chemotherapy is, in many cases, unclear due to a wide variety of prognostic factors and small sample size. However, there is some evidence to suggest that drugs such as
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Schreiber H, Barry FM, Russell WC, Macon IV WL, Ponsky JL, Pories WJ. Stewart–Treves
Syndrome: a lethal complication of postmastectomy lymphedema and regional immune deficiency. Arch Surgery 1979;114:82–5.
91:
It was previously a relatively common complication of the massive lymphedema of the arm which followed removal of axillary (arm pit) lymph nodes and lymphatic channels as part of the classical
66:
obstruction). It involves either the upper or lower lymphedematous extremities but is most common in upper extremities. Although its name implies lymphatic origin, it is believed to arise from
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Verdier E, Carvalho P, Young P, Musette P, Courville P, Joly P. Lymphangiosarcoma treated with liposomal doxorubicin (Caelyx)] Ann
Dermatol Venereol. 2007 Oct;134 (10 Pt 1):760–3. French.
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Sher T, Hennessy BT, Valero V, Broglio K, Woodward WA, Trent J, Hunt KK, Hortobagyi GN, Gonzalez-Angulo AM.Primary angiosarcomas of the breast. Cancer. 2007 Jul 1;110(1):173–8.
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proposed that local immunodeficiency as a result of lymphedema results in a "immunologically privileged site" in which the sarcoma is able to develop.
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Stewart FW, Treves N. Lymphangiosarcoma in postmastectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.
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Chopra, S, Ors, F, Bergin, D MRI of angiosarcoma associated with chronic lymphoedema: Stewart Treves syndrome Br J Radiol 2007 80: e310–313
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Hellman S, DeVita VT, Rosenberg S (2001). Cancer: principles & practice of oncology. Philadelphia: Lippincott-Raven. p. 1853.
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The most successful treatment for lymphangiosarcoma is amputation of the affected limb if possible.
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Chung, Kevin C.; Kim, Huyi Jin
Elizabeth; Jeffers, Lynn L. C. (2000-11-01).
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351:"Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena", Milan, Italy.
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279:"Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients"
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which occurs in long-standing cases of primary or secondary
244:. Academy of Lymphatic Studies 2014. 2014. pp. 26–30.
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may be administered if there is evidence or suspicion of
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Cancer of blood/lymph vessel lining in swollen body parts
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679:Intravascular papillary endothelial hyperplasia
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70:and may be more accurately referred to as
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737:Multifocal lymphangioendotheliomatosis
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793:Connective and soft tissue neoplasms
534:Proliferating angioendotheliomatosis
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711:Acquired progressive lymphangioma
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486:Blue rubber bleb nevus syndrome
1:
93:Halstedian radical mastectomy
581:Immunosuppression-associated
544:Infantile hemangiopericytoma
174:exhibit antitumor activity.
706:Lymphangioma circumscriptum
283:The Journal of Hand Surgery
814:
788:Lymphatic vessel diseases
725:Lymphangioleiomyomatosis
566:African lymphadenopathic
465:Tumours of blood vessels
295:10.1053/jhsu.2000.18490
204:Stewart–Treves syndrome
97:Stewart–Treves syndrome
652:Universal angiomatosis
618:Targeted hemosiderotic
501:Endovascular papillary
123:This section is empty.
241:LYMPHEDEMA MANAGEMENT
491:Hemangioendothelioma
674:Pyogenic granuloma
539:Hemangiopericytoma
156:metastatic disease
78:Signs and symptoms
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742:Lymphangiomatosis
701:lymphangiosarcoma
561:African cutaneous
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68:endothelial cells
62:(swelling due to
52:Lymphangiosarcoma
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25:Lymphangiosarcoma
19:Medical condition
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588:Hemangioblastoma
556:Kaposi's sarcoma
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289:(6): 1163–1168.
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258:on 26 April 2014
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125:You can help by
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571:AIDS-associated
481:Hemangiosarcoma
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657:Angiokeratoma
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134:November 2021
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767:Angiofibroma
762:angiosarcoma
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697:Lymphangioma
613:Microvenular
527:Spindle cell
473:Blood vessel
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260:. Retrieved
256:the original
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194:Lymphangioma
184:Angiosarcoma
152:Chemotherapy
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131:
127:adding to it
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72:angiosarcoma
51:
50:
667:Angiolipoma
635:Seriginosum
608:Glomeruloid
551:Venous lake
506:Epithelioid
172:gemcitabine
164:doxorubicin
782:Categories
662:of Mibelli
593:Hemangioma
511:Kaposiform
417:DiseasesDB
210:References
168:ifosfamide
160:paclitaxel
60:lymphedema
54:is a rare
689:Lymphatic
603:Cavernous
598:Capillary
516:Infantile
496:Composite
303:0363-5023
146:Treatment
107:Diagnosis
31:Specialty
521:Retiform
401:: 9170/3
311:11119680
262:25 April
178:See also
36:Oncology
798:Sarcoma
758:Angioma
625:Angioma
576:Classic
411:D008204
199:Sarcoma
751:Either
719:PEComa
645:Tufted
640:Spider
630:Cherry
309:
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170:, and
101:et al.
87:Causes
56:cancer
38:
422:29095
398:ICD-O
406:MeSH
307:PMID
299:ISSN
264:2014
246:ISBN
223:ISBN
291:doi
129:.
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287:25
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390:D
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Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
