Lymphangiosarcoma
Lymphangiosarcomas are rare and aggressive malignant tumors that originate in the lymphatic vessels, primarily affecting individuals with a history of lymphedema. These tumors are most commonly observed in the upper extremities, such as the arms, and often arise in patients who have undergone classical radical mastectomy for breast cancer, typically five to fifteen years post-surgery. The condition, known as Stewart-Treves syndrome, predominantly affects women and is linked to chronic lymphedema—90 percent of cases occur in those with this condition.
Symptoms of lymphangiosarcoma include the appearance of purplish, bruised areas on the skin that can progress to painful sores with necrosis. These tumors are known for their rapid metastasis, frequently spreading to the lungs. Diagnosis involves a histological examination following the identification of concerning skin changes. Unfortunately, effective treatment options are limited, often necessitating surgical removal of the tumor, and in severe cases, amputation of the affected limb. The prognosis is generally poor, with a high recurrence rate and a typical survival time of around 2.5 years post-diagnosis, highlighting the serious nature of this malignancy.
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Subject Terms
Lymphangiosarcomas
ALSO KNOWN AS: Lymphatic vessel tumors, angiosarcomas, lymphangioendothelioma, Stewart-Treve syndrome, hemangiosarcomas
RELATED CONDITIONS: Primary or secondary lymphedemas, classical radical mastectomy
DEFINITION: A lymphangiosarcoma is a rare, aggressive, malignant soft tissue neoplasm tumor that begins in the cells of the lymph vessels, usually in the upper extremities of individuals with lymphedema.
Risk factors: Risk factors for lymphangiosarcomas include a history of primary or secondary lymphedema, having had a classical radical mastectomy, or having radiation or chronic infections in a lymphodemous limb. Women are impacted more often than men.
Etiology and the disease process: Lymphangiosarcoma begins in the cells of the lymphatic vessels. This tumor is seen in the upper arms approximately five to fifteen years after a classical radical mastectomy, specifically in individuals who have long-standing lymphedema. Although the breast cancer may have been cured with the radical mastectomy, a secondary cancer diagnosis of lymphangiosarcoma has a poor prognosis. The radical mastectomy procedure is now outmoded and has been replaced with a more conservative surgical procedure. Around 90 percent of cases of lymphangiosarcomas arise in individuals with long-standing idiopathic lymphedema. This is called Stewart-Treves syndrome. The signs of lymphangiosarcoma are a purple or bruised area on an extremity (usually the upper arm) that becomes a sore that does not heal with necrosis (breakdown) of the skin and underlying tissue. There are often satellite spots from the original site. The tumor metastasizes quickly, usually impacting the lungs.
Incidence: Lymphangiosarcoma is rare; the occurrence in patients who had a radical mastectomy for breast cancer is less than 1 percent. It occurs most often in chronic lymphedema patients.
Symptoms: Symptoms of lymphangiosarcoma are purple, bruised areas or tender growths of abnormal tissue. Around 60 percent occur on the skin of the arms or legs, especially the forearm and elbow. Other common areas of development include the scalp of older men and the head and neck of older women.
Screening and diagnosis: The appearance of purplish, bruised-looking areas on the extremities of an individual with long-standing lymphedema is an indication for further evaluation. A is taken from the site; diagnosis is made by histologic examination and rules out metastatic disease from a or another sarcoma (such as Kaposi sarcoma).
Treatment and therapy: There is not an effective treatment or therapy for lymphangiosarcoma. The lymphangiosarcoma site is removed surgically, and amputation of the affected limb may be necessary. may also be given after surgical treatment, usually the drugs paclitaxel, doxorubicin, or gemcitabine.
Prognosis, prevention, and outcomes: The rate of recurrence is high, and the long-term survival rate is poor, in large part because of the rapid spread of the tumor to the chest wall, the liver, and to bone. Most individuals survive for around 2.5 years following diagnosis.
Bibliography
Brennan, Murray F., Cristina R. Antonescu, and Robert G. Maki. Management of Soft Tissue Sarcoma. Springer, 2013.
Kelati, Awatef, et al. "Dermoscopy in a Lymphangiosarcoma of Stewart–Treves Syndrome." Breast Cancer Management, vol. 7, no. 2, 2018. Taylor & Francis Online, doi.org/10.2217/bmt-2017-0022.
Mamelak, Adam J., et al. "Lymphangiosarcoma of the Scalp." Journal of Cutaneous Medicine and Surgery, vol. 18, no. 2, 2014, pp. 132–36. doi:10.2310/7750.2013.13025.
Murgia, Robert D., and Gary P. Gross. "Stewart-Treves Syndrome." National Library of Medicine, 14 Aug. 2023, www.ncbi.nlm.nih.gov/books/NBK507833. Accessed 20 June 2024.
Sepah, Yasir J., et al. “Lymphangiosarcoma of the Arm Presenting with Lymphedema in a Woman 16 Years after Mastectomy: A Case Report.” Cases Journal, vol. 2, 1 Sept. 2009. doi:10.4076/1757-1626-2-6887.
Yang, Fuchun, et al. "Autophagy Inhibition Prevents Lymphatic Malformation Progression to Lymphangiosarcoma by Decreasing Osteopontin and Stat3 Signaling." Nature Communications, vol. 14, no. 1, 2023, p. 978. doi.org/10.1038/s41467-023-36562-5.