Lymphangiosarcoma
Encyclopedia
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.
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.
(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.
, 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.
Lymphedema
Lymphedema , also known as lymphatic obstruction, is a condition of localized fluid retention and tissue swelling caused by a compromised lymphatic system....
. It involves either the upper or lower lymphedemateous extremities but is most common in upper extremities.
Signs and Symptoms
The sarcomaSarcoma
A sarcoma is a cancer that arises from transformed cells in one of a number of tissues that develop from embryonic mesoderm. Thus, sarcomas include tumors of bone, cartilage, fat, muscle, vascular, and hematopoietic tissues...
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.
Causes
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 syndromeStewart-Treves syndrome
Stewart-Treves syndrome refers to an angiosarcoma arising from chronic lymphedema . It typically occurs as a complication of long-lasting lymphedema of the arm after mastectomy and/or radiotherapy for breast cancer. It can rarely occur on the leg...
(which can be both lymphangiosarcoma and hemangiosarcoma
Hemangiosarcoma
Hemangiosarcoma is a rare, rapidly growing, highly invasive variety of cancer. It is a sarcoma arising from the lining of blood vessels; that is, blood-filled channels and spaces are commonly observed microscopically...
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.
Treatment
The most successful treatment for angiosarcoma is amputation of the affected limb. 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 paclitaxelPaclitaxel
Paclitaxel is a mitotic inhibitor used in cancer chemotherapy. It was discovered in a U.S. National Cancer Institute program at the Research Triangle Institute in 1967 when Monroe E. Wall and Mansukh C. Wani isolated it from the bark of the Pacific yew tree, Taxus brevifolia and named it taxol...
, doxorubicin
Doxorubicin
Doxorubicin INN is a drug used in cancer chemotherapy. It is an anthracycline antibiotic, closely related to the natural product daunomycin, and like all anthracyclines, it works by intercalating DNA....
, ifosfamide
Ifosfamide
Ifosfamide is a nitrogen mustard alkylating agent used in the treatment of cancer.It is sometimes abbreviated "IFO".-Uses:It is given as a treatment for a variety of cancers, including:...
, 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
Bevacizumab
Bevacizumab is a drug that blocks angiogenesis, the growth of new blood vessels. It is commonly used to treat various cancers, including colorectal, lung, breast, kidney, and glioblastomas....
, may be effective in treating lymphangiosarcoma. Investigation of bevacizumab in combination with other chemotherapy agents is underway.
See also
- LymphangiomaLymphangiomaLymphangiomas are malformations of the lymphatic system, which is the network of vessels responsible for returning to the venous system excess fluid from tissues. These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and...
- AngiosarcomaAngiosarcomaAngiosarcoma is a malignant neoplasm of endothelial-type cells that line vessel walls. This may be in reference to blood or lymphatic vessels ....
- HemangiosarcomaHemangiosarcomaHemangiosarcoma is a rare, rapidly growing, highly invasive variety of cancer. It is a sarcoma arising from the lining of blood vessels; that is, blood-filled channels and spaces are commonly observed microscopically...
- Stewart-Treves SyndromeStewart-Treves syndromeStewart-Treves syndrome refers to an angiosarcoma arising from chronic lymphedema . It typically occurs as a complication of long-lasting lymphedema of the arm after mastectomy and/or radiotherapy for breast cancer. It can rarely occur on the leg...
- SarcomaSarcomaA sarcoma is a cancer that arises from transformed cells in one of a number of tissues that develop from embryonic mesoderm. Thus, sarcomas include tumors of bone, cartilage, fat, muscle, vascular, and hematopoietic tissues...
External links
- http://www.lymphedemapeople.com/thesite/lymphedema_lymphangiosarcoma.htm