Cases reported "Lymphangiosarcoma"

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1/3. Primary malignant lymphoma arising in postmastectomy lymphedema. Another facet of the Stewart-Treves syndrome.

    In rare cases, primary malignant lymphomas may arise in the soft tissues. Only one previous case has arisen in the context of chronic lymphedema. Because of the clinical appearance of such lesions, which resemble violaceous nodular or plaquelike tumors, they may be confused clinically with lymphedema-associated angiosarcomas occurring after radical mastectomy (Stewart-Treves syndrome). Furthermore, the histologic appearance of some lymphomas and angiosarcomas may also be similar. We studied two women with primary postmastectomy lymphedema-related malignant lymphoma in the soft tissues of the upper arm. These tumors arose 11 and 30 years, respectively, after radical removal of ductal mammary carcinomas. Histologically, one neoplasm mimicked metastatic carcinoma or epithelioid angiosarcoma; whereas the other was initially confused with a variety of pathologic entities, including vasculitis, epithelioid hemangioma, and malignant fibrous histiocytoma. The lymphoid nature of both lesions was confirmed by immunoreactivity for leukocyte common antigen in addition to the B-lymphocyte marker, L26. Conversely, vascular and epithelial determinants were absent. One patient's disease pursued an indolent course; she died of unknown causes but with no evidence of lymphoma at last follow-up. The second patient is currently in remission on chemotherapy. awareness of the existence of lymphedema-related malignant lymphoma and familiarity with methods used for its distinction from epithelioid vascular sarcomas should prevent unnecessary surgery.
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2/3. Stewart-Treves syndrome: an histogenetic (ultrastructural and immunohistological) study.

    One case of the so-called "Stewart-Treves syndrome" (STS), appearing on a lymphoedematous arm complicating radical mastectomy for breast cancer, was characterized electronmicroscopically and immunohistologically, in order to elucidate its disputed (epithelial vs endothelial) histogenesis. Epithelial and endothelial differentiation markers used comprised: antibodies against keratin, vimentin, factor viii-related antigen (F VIII-RA), hla-dr antigens and the lectin ulex europeaus agglutinin I (UEA I). At the ultrastructural level, neoplastic cells were found to contain typical weibel-palade bodies, whereas by immunohistological techniques they proved to be keratin-negative/vimentin , F VIII-RA , UEAI , HLA-DR . These results rule out a possible epithelial differentiation and strongly favour an endothelial one for STS.
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3/3. Stewart-Treves syndrome. A lethal complication of postmastectomy lymphedema and regional immune deficiency.

    lymphangiosarcoma is a fatal complication of postmastectomy lymphedema. The pathogenesis of lymphangiosarcoma in chronic lymphedema is a combination of two factors. First, the edematous region responds in a manner similar to "immunologically privileged sites." Second, because of its anatomic and physiologic properties, it is a favorable site for the development of mutant cell populations for reasons that are not fully understood. As a result, these mutant cells, with their genetically nonidentical antigens, escape recognition by the host's impaired immune surveillance mechanism. The failure to promote a sufficient immune response allows unrestricted tumor growth to take place, resulting in the ultimate death of the patient. Available therapeutic measures are equally unsatisfactory. Emphasis is placed on periodic examination of the lymphedematous extremity, aggressive treatment of established lymphedema and infections, and surgical preservation of lymphatic channels during breast cancer surgery.
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