1/22. Stewart-Treves syndrome: lymphangiosarcoma following mastectomy.lymphangiosarcoma (LAS) is an aggressive, malignant vascular tumor following long-lasting chronic lymphedema. patients with LAS demonstrate a history of breast cancer treated by radical mastectomy in the majority of patients. In the 1960s the incidence of LAS in patients with a 5-year survival after radical mastectomy varied from 0.07 to 0.45%. Today, due to changes in the operative techniques of breast cancer, less chronic lymphedema is seen with only a scant number of LAS patients. The etiology of this enigmatic tumor is not yet completely understood. Histologically, LAS arises from vascular endotheliocytes, and all vascular sarcomas originating in the setting of a chronic lymphedema are categorized as LAS. There is no standard treatment of LAS. The treatment options include radical ablative surgery, radiation therapy, and chemotherapy. The prognosis of LAS is poor; long-term survival is the exception. Only early recognition and radical surgery offer a chance of cure.- - - - - - - - - - ranking = 1keywords = lymphedema (Clic here for more details about this article) |
2/22. hemangiosarcoma of the left hand in a patient with the rare combination of Maffucci's and Stewart Treves syndrome.We describe a patient with the previously unseen combination of Maffucci's and Stewart Treves syndrome who presented with an angiosarcoma of the hand. Maffucci's syndrome is characterized by the presence of multiple enchondroma and soft tissue hemangioma. The syndrome is a rare nonhereditary condition with a usual onset in childhood. Malignant transformations are a common feature of this syndrome. In 1948, Stewart and Treves first described six cases of lymphangiosarcoma after radical mastectomy. This syndrome is an unusual form of angiosarcoma occuring as a complication of lymphedema. Chronic lymphedema and lymphangiectasia preceding lymphangiosarcoma may not only be induced by radical mastectomy with axillary lymph node dissection and postoperative radiation therapy. Posttraumatic, congenital or spontaneous chronic lymphedema may also be associated with lymphangiosarcoma. A time interval of many years seems to be required before malignant transformation develops. Generally the syndrome has a very poor prognosis. Both syndromes described above are of a rare frequency. We report this case because of prior unknown coincidence of both syndromes.- - - - - - - - - - ranking = 1keywords = lymphedema (Clic here for more details about this article) |
3/22. Two cases of Kaposi's sarcoma mimicking Stewart-Treves syndrome found to be human herpesvirus-8 positive.Although angiosarcoma is the most frequent tumor arising in the clinical setting of chronic lymphedema, as in Stewart-Treves syndrome, Kaposi's sarcoma has also been reported in this setting, although rarely. We describe two women who developed Kaposi's sarcoma in the lymphedematous arm many years after surgery for breast cancer. Case 1 is a 92-year-old and Case 2 is an 81-year-old; they underwent left total mastectomy and axillary node dissection for infiltrating breast carcinoma in 1981 and 1982 respectively. At that time, neither patient received further treatment. Except for persistent lymphedema, both women did well until over fourteen years later when each noted the development of several purple asymptomatic plaques on the edematous arm. In both, the clinical diagnosis at the time of biopsy was angiosarcoma. However, histologic findings in both cases were typical for Kaposi's sarcoma. In addition, a nested polymerase chain reaction (PCR) for the detection of a 233bp segment of KSHV/HHV8 was performed on dna extracted from the paraffin-embedded specimens and both cases were positive for this sequence. Histologic sections of both cases were also tested for KSHV by in situ hybridization and demonstrated a positive signal in the lesional cells in each case.- - - - - - - - - - ranking = 1keywords = lymphedema (Clic here for more details about this article) |
4/22. Long-term survival after radical operations for cancer treatment-induced sarcomas: how two survivors invite reflection on oncologic treatment concepts.Extent and radicality of surgical oncologic treatment has changed in the past 30 years. Two patients with node-positive breast cancer are presented, who underwent (total or radical) mastectomy with lymphadenectomy and postoperative radiation 24 and 40 years ago. A radiation-associated sarcoma of the parascapular soft tissue developed in one patient 9 years after treatment; the other one sought treatment for a lymphedema-associated Stewart-Treves lymphangiosarcoma 16 years after initial therapy. Both patients underwent a forequarter amputation for their treatment-associated high-grade sarcoma. Both are currently alive and cancer-free 15 and 24 years after amputation. These reports remind us that radical locoregional treatment can cure some solid cancers in the absence of systemic therapy; that such extensive treatment may induce significant disability or secondary malignancies long-term; that even advanced treatment-associated sarcomas can be cured with aggressive resection; that today's multimodality therapy approaches and appropriate patient selection have rendered such extensive locoregional treatment for many tumors obsolete or unnecessary; and that if no effective alternative treatment exists and organ or limb preservation is not feasible, an aggressive resection approach for high-grade cancer should not be discounted unless systemic failure is certain or imminent.- - - - - - - - - - ranking = 0.33333333333333keywords = lymphedema (Clic here for more details about this article) |
5/22. Stewart-Treves syndrome as a rare complication of a hereditary lymphedema.lymphangiosarcoma (LAS) may occur as a rare complication of primary lymphedema. A case of LAS in hereditary lymphedema of the lower extremity in a 36-year old female is reported. Despite of chemotherapy, local hyperthermia and later amputation of the extremity the patient died of progressive disease due to pulmonary metastasis. In respect to this case, the different therapeutic concepts, as reported in the literature, and their results are presented and discussed.- - - - - - - - - - ranking = 2keywords = lymphedema (Clic here for more details about this article) |
6/22. lymphangiosarcoma associated with lymphedema in a man with Maffucci's syndrome.A man with Maffucci's syndrome and lifelong lymphedema in the right lower extremity had a lymphangiosarcoma in the same extremity. Despite amputation of the limb and radiotherapy, he died several months later of pulmonary metastases. Malignancies of vascular origin rarely have been reported in this syndrome. Our patient's features suggest that lymphedema may predispose patients with Maffucci's syndrome to the development of lymphangiosarcoma.- - - - - - - - - - ranking = 2keywords = lymphedema (Clic here for more details about this article) |
7/22. Response of postmastectomy lymphangiosarcoma to radiotherapy: report of four cases.We have reported on four previously unpublished cases of postmastectomy lymphangiosarcoma. All of these cases had suffered from a long-lasting lymphedema of the upper extremity. Only one patient had survived over one year. After radiotherapy she lived four years from first lesion of lymphangiosarcoma. She died locally disease-free in spite of two earlier recurrences, which were also treated with radiotherapy. We believe that only in early cases can the radiotherapy give a good palliation in postmastectomy lymphangiosarcoma.- - - - - - - - - - ranking = 0.33333333333333keywords = lymphedema (Clic here for more details about this article) |
8/22. 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.- - - - - - - - - - ranking = 2.6666666666667keywords = lymphedema (Clic here for more details about this article) |
9/22. lymphangiosarcoma in chronic lymphedema. Reports of 3 cases and review of the literature.lymphangiosarcoma is an extremely rare but highly lethal complication of chronic lymphedema. The classic clinical picture is described and three cases are presented. Current concepts of etiology, histopathology, treatment and prognosis are discussed. Since early diagnosis may be the key to longer survival, all patients with chronic lymphedema should undergo careful examination at regular intervals throughout their lives.- - - - - - - - - - ranking = 2keywords = lymphedema (Clic here for more details about this article) |
10/22. lymphangiosarcoma in postmastectomy lymphedema (Stewart-Treves syndrome): ultrastructural and immunohistologic characteristics.Two cases of lymphangiosarcoma arising in a chronic lymphedematous extremity following mastectomy (Stewart-Treves syndrome) were reported with not only standard histology, but also special study on the ultrastructure and immunohistology. These cancers developed as a result of chronic lymphedema of the involved limb following mastectomy 15 and 16 years ago, respectively, for breast carcinoma. Immunohistologic and electron microscopic examinations proved that this tumor originates in the vascular endotheliocytes, even though they are clinically chronic lesions apparently derived from the lymphatic vessels. This sarcoma develops multicentrically in an edematous arm and spreads out rapidly, so prognosis is quite discouraging in those patients. They need to be radically surgically treated without hesitation; hence, amputation, not limb-saving surgery, should be indicated.- - - - - - - - - - ranking = 2keywords = lymphedema (Clic here for more details about this article) |
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