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Case Study
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Stewart-Treves syndrome in long-term lymphedema Bonnie Sparks-DeFriese PT, RN, CWS, CWOCN, Lea Crestodina MSN, RN, GNP, CWOCN, CDE, Rhonda Holbrook BSN, RN, CWOCN CLINICAL PROBLEM:.A 79-year-old Caucasian woman with a long history of lower extremity lymphedema presented to the Vascular Surgery Clinic with a 15 cm x 12 cm raised area covering most of her distal anterior left lower extremity. The mass had been present for four months. The mass appeared necrotic with sanguineous drainage and satellite lesions that appeared necrotic. A biopsy was obtained and was positive for angiosarcoma PAST TREATMENT: Per chart review, past treatments for lymphedema have included H2O2 and Polysporin Ointment, Domeboro's Solution, lymphedema pumps, and compression stockings. Compression wraps were begun four months prior to this visit. ANGIOSARCOMA in LONG TERM LYMPHEDEMA (STEWART-TREVES SYNDROME): Angiosarcoma is a rare vascular malignancy, accounting for less than 1% of all sarcomas. It usually occurs in the skin, breast, liver and deep soft tissue. Angiosarcoma that arises in an area of chronic lymphedema is known as Stewart-Treves syndrome. It most often presents in postmastectomy lymphedema in the arm or the irradiated breast area. The prognosis is poor TREATMENT: After consultation with oncology, it was decided to proceed with a left above the knee amputation PATIENT OUTCOMES: A left above-knee amputation was performed and tolerated well. Patient was discharged 7 days post-operatively to a rehab facility. Rehab included PT, OT, SLP, TR, nutrition, social work, and rehab nursing. At discharge the patient required only contact-guard assistance for mobility, transfers and activities of daily living. She was able to propel her wheelchair 100 feet with supervision. After 13 days in the rehab, the patient was transferred to a skilled nursing facility for continued monitoring of co-morbid medical conditions and cognitive support CONCLUSION: .Although extremely rare, angiosarcoma can occur in patients with long-standing lymphedema and should be considered in the differential diagnosis of a new lesion. CLINICAL IMPLICATIONS: Patients with chronic lymphedema must be monitored for any atypical lesion, and any lesion must be promptly biopsied. These patients must be taught to promptly report any new lesions. LIMITATIONS: There is limited awareness of lymphedema treatment in general, with many patients untreated for years.. Allan A, Shoji T, Li N, Burlage A, Davis B, Bhawan J. Two cases of KaposiÕs sarcoma mimicking Stewart-Treves syndrome found to be human herpesvirus-8 positive. Am J Dermatopathol. 2001;23(5):431Ð445. Breidendbach M, Rein D, Schmidt T, et al. Intra-arterial mitoxantrone and paclitaxel in a patient with Stewart-Treves syndrome:selection of chemotherapy by an ex vivo ATP-based chemosensitivity assay. Anti Cancer Drugs. 2000;11(4):269Ð273 Komorowski A. Wysocki W, Mitus J. Angiosarcoma in a chronically lymphedematous leg: an unusual presentation of Stewart-Treves syndrome. South Med J. 2003;96(8):807Ð808. |
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