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Journal of Clinical Oncology, Vol 24, No 9 (March 20), 2006: pp. 1477-1478 © 2006 American Society of Clinical Oncology. DOI: 10.1200/JCO.2005.01.8473
CASE 3. Upper Limb Lymphangiosarcoma Following Breast Cancer TherapyDepartments of Medical Oncology, Dermatology, and Pathology, Hospital Universitario de Salamanca, Salamanca, Spain A 75-year-old woman with a history of hypertension, diabetes mellitus, and cardiac failure was referred to our institution for a second opinion after a diagnosis of lymphangiosarcoma of the upper left limb. In 1997, she was diagnosed with invasive ductal carcinoma of the left breast. Treatment consisted of breast-conserving surgery followed by axillary node dissection and local radiotherapy. No additional systemic treatment was administered. The patient did well except for residual lymphedema of the arm. Three months before admission to our hospital, edema and pain of the left arm was observed, which was associated with fever during the last 2 weeks. The patient was diagnosed with erysipelas and systemic antibiotic treatment was administered without resulting in any improvement. Physical examination showed striking lymphedema with purple-colored multifocal raised lesions, several of them progressing to ulceration (Fig 1). As no improvement was observed with antibiotic treatment, histologic study was performed. Biopsy showed a lymphovascular invasive pattern compatible with lymphangiosarcoma. There was proliferation of spindle cells and multiple vascular spaces (Fig 2). Immunohistochemistry was positive for endothelial cell markers (vimentin, keratin, and CD34). After carefully evaluating the extent of the disease, including thoracic and abdominal computed tomography that showed no involvement of chest wall or visceral metastasis, radical surgery was performed followed by a reconstructive procedure.
Lymphangiosarcoma secondary to upper extremity edema following postmastectomy was first described by Stewart and Treves in 19481 and since then, this association has been called Stewart-Treves syndrome. Lymphangiosarcoma following breast cancer surgery is a rare entity. The incidence among white women in the United States is 1.6 of 100,000 and only 25% of these sarcomas are located in the upper extremity.2 Since the original description, approximately 300 cases of Stewart-Treves syndrome have been reported worldwide. The pathogenic mechanism of this syndrome is unknown, but several hypotheses have been postulated. As proliferation of lymphatic vessels is often seen in areas of affected edematous tissue,3 it has been suggested that the block of the lymphatics represent a stimuli for growth factors and cytokines and for, thus, a proliferation of vessels and lymphatics. The association between radiotherapy and chest wall sarcomas is well known,4 but it is unclear if radiation therapy contributes to this entity, since most reported postradiation sarcomas are not lymphangiosarcomas. Although postmastectomy is the main predisposing factor for lymphedema development, other factors such as hypertension and cardiovascular disease have been described.5 In our patient, the association of cardiovascular disease could contribute to the lymphedema. The prognosis of these tumors is poor and wide surgical resection is the treatment of choice.6 Although amputation is usually indicated with a curative intention, there is also a role for palliation in bleeding, fungation, and severe pain.7 The role of chemotherapy is not clear and the response rate in advanced disease is poor.8 Stewart-Treves Syndrome is a rare entity that must be taken into account when skin abnormalities appear in an edematous extremity secondary to primary breast cancer surgery, especially if postoperative radiotherapy was used. Early diagnosis and radical surgical treatment appear to confer the best chance for improved survival. Authors' Disclosures of Potential Conflicts of Interest The authors indicated no potential conflicts of interest.
REFERENCES 1. Stewart FW, Treves N: Lymphangiosarcoma in postmastectomy lymphedema: A report of six cases in elephantiasis chirurgica. Cancer 1:64-81, 1948[CrossRef] 2. Mack TM: Sarcomas and other malignancies of soft tissue, retroperitoneum, peritoneum, pleura, heart, mediastinum and spleen. Cancer 75:211-245, 1995[CrossRef][Medline] 3. Woodward AH, Ivins JC, Soule EH: Lymphangiosarcoma arising in chronic lymphedematous extremities. Cancer 30:562-572, 1972[CrossRef][Medline] 4. Wiklund TA, Blomqvist CP, Raty J, et al: Postirradiation sarcoma: Analysis of nationwide cancer registry material. Cancer 68:524-531, 1991[CrossRef][Medline] 5. Bohler FK, Rhomberg W, Doringer W: Hypertention as a risk factor for increased rate of side effects in the framework of breast cancer irradiation. Strahlenther Onkol 168:344-349, 1992[Medline] 6. Stewart NJ, Pritchard DJ, Nascimento AG, et al: Lymphangiosarcoma following mastectomy. Clin Orthop Relat Res 320:135-141, 1995[Medline] 7. Clark MA, Thomas JM: Amputation for soft-tissue sarcoma. Lancet Oncol 4:335-342, 2003[Medline] 8. Kuten A, Sapir D, Cohen Y, et al: Postirradiation soft tissue sarcoma occurring in breast cancer patients: Report of seven cases and results of combination chemotherapy. J Surg Oncol 28:168-171, 1985[Medline]
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Copyright © 2006 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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