|Classification and external resources|
Lymphangiosarcoma is a rare malignant tumor which occurs in long-standing cases of primary or secondary lymphedema. It involves either the upper or lower lymphedemateous extremities but is most common in upper extremities.
Signs and Symptoms
The sarcoma first appears as a bruise mark, a purplish discolorization or a tender skin nodule in the extremity, typically on the anterior surface. It progresses to an ulcer with crusting, and finally to an extensive necrosis involving the skin and subcutaneous tissue. It metastasizes quickly.
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 Halstedian radical mastectomy, 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 Stewart-Treves syndrome (which can be both lymphangiosarcoma and hemangiosarcoma following mastectomy). 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 et al. proposed that local immunodeficiency as a result of lymphedema results in a "immunologically privileged site" in which the sarcoma is able to develop.
The most successful treatment for angiosarcoma is amputation of the affected limb if possible. Chemotherapy may be administered if there is metastatic disease. If there is no evidence of metastasis beyond the lymphedematous limb, adjuvant chemotherapy may be given anyway due to the possibility of micrometastatic disease. 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 paclitaxel, doxorubicin, ifosfamide, and gemcitabine exhibit antitumor activity.
Recently, there has been interest in evaluating the effectiveness of anti-angiogenic drugs in the treatment of lymphangiosarcoma. Early evidence suggests that treatment with one such drug, Bevacizumab, may be effective in treating lymphangiosarcoma. Investigation of bevacizumab in combination with other chemotherapy agents is underway.
- Hellman S, DeVita VT, Rosenberg S (2001). Cancer: principles & practice of oncology. Philadelphia: Lippincott-Raven. p. 1853. ISBN 0-7817-2229-2.
- Stewart FW, Treves N. Lymphangiosarcoma in postmas- tectomy lymphedema: a report of six cases in elephantiasis chirurgica. Cancer 1948;1:64–81.
- Chopra, S, Ors, F, Bergin, D MRI of angiosarcoma associated with chronic lymphoedema: Stewart Treves syndrome Br J Radiol 2007 80: e310-313
- Schreiber H, Barry FM, Russell WC, Macon IV WL, Ponsky JL, Pories WJ. Stewart–Treves Syndrome: a lethal complica- tion of postmastectomy lymphedema and regional immune deficiency. Arch Surgery 1979;114:82–5.
- 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 "Ospedale Maggiore Policlinico, Mangiagalli e Regina Elena", Milan, Italy.
- 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.
- 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.
- M. Agulnik, S. H. Okuno, M. Von Mehren, B. Jovanovic, B. Brockstein, R. S. Benjamin and A. M. Evens. An open-label multicenter phase II study of bevacizumab for the treatment of angiosarcoma. Northwestern University, Chicago, IL; Mayo Clinic, Rochester, MN; Fox Chase Cancer Center, Philadelphia, PA; Evanston Northwestern Healthcare, Chicago, IL; University of Texas M. D. Anderson Cancer Center, Houston, TX