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Post-treatment sarcoma in breast cancer patients.

BACKGROUND: Many patients treated for breast cancer with radiotherapy will survive their disease and be at risk for treatment-related sarcoma for many years. METHODS: In order to identify patients with post-treatment sarcoma and define this disease, we examined the records of 99 patients treated for sarcoma with a history of antecedent breast carcinoma. Of these patients, 51 were felt to have a sarcoma unrelated to breast cancer treatment and 48 were felt to have a treatment-related sarcoma (secondary to lymphedema and/or radiation). RESULTS: Lymphangiosarcoma of the extremity was the most common histologic subtype of post-treatment sarcoma, accounting for 22 of 48 cases (46%). Twenty-six patients (54%) developed nonlymphangiosarcoma post-treatment sarcoma; all of these were radiation-associated sarcomas. The median latency interval between the diagnosis of breast cancer and the development of sarcoma was 11 years (range 4-44) and was not different between the two groups. However, patients with nonlymphangiosarcoma were significantly younger when diagnosed with breast cancer than were those with lymphangiosarcoma of the extremity (median 43 vs. 51 years, p < 0.001). The survival of all 48 patients was poor: 5-year survival was 29%. Five-year survival of patients with other types of post-treatment sarcoma was just as poor as those with lymphangiosarcoma of the extremity (30% vs. 28%, p = 0.98). CONCLUSIONS: Patients who develop sarcoma after treatment for breast cancer have a poor prognosis whether it occurs as Stewart-Treves syndrome or other types of post-treatment sarcoma. Younger patients may be at higher risk than are older patients for the development of nonlymphangiosarcoma post-treatment sarcoma.

Adult↗

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.

Amputation, Surgical↗

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.

Aged↗

Stewart-Treves syndrome. A report of two cases with a review of Japanese literature.

Two cases of postmastectomy lymphangiosarcoma seen at our department are reported. One case, treated by amputation, died 1 year 4 months later and the other case, treated by local excision followed by amputation, died 7 months later. Twenty cases from the Japanese literature, including our 2 cases, are reviewed herein. The initial treatment for the other 18 cases was as follows: amputation in 8 cases, local excision in 2, radiotherapy in 4 and chemotherapy in 4. Fifteen of 16 cases eligible for follow up were dead within 20 months after treatment. Although the knowledge available as to the results of the treatment of postmastectomy lymphangiosarcoma is still insufficient to indicate the optimal therapeutic approach, early recognition of the lesion and prompt radical ablative surgery seem to offer the best chance for survival.

Aged↗

The risk of angiosarcoma following primary breast cancer.

Lymphangiosarcoma of the upper extremity is a rare and aggressive tumour reported to occur following post-mastectomy lymphoedema (Stewart-Treves syndrome). Haemangiosarcoma, a related rare tumour, has occasionally been reported to occur in the breast following irradiation. We conducted a case-control study using the University of Southern California-Cancer Surveillance Program, the population-based cancer registry for Los Angeles County, to evaluate the relationship between invasive female breast cancer and subsequent upper extremity or chest lymphangiosarcoma and haemangiosarcoma together referred to as angiosarcoma. Cases were females diagnosed between 1972 and 1995 with angiosarcoma of the upper extremity (n = 20) or chest (n = 48) who were 25 years of age or older and residing in Los Angeles County when diagnosed. Other sarcomas at the same anatomic sites were also studied. Controls were females diagnosed with cancers other than sarcoma during the same time period (n = 266,444). Cases and controls were then compared with respect to history of a prior invasive epithelial breast cancer. A history of breast cancer increased the risk of upper extremity angiosarcoma by more than 59-fold (odds ratio [OR] = 59.3, 95% confidence interval [95% CI] = 21.9-152.8). A strong increase in risk after breast cancer was also observed for angiosarcoma of the chest and breast (OR = 11.6, 95% CI = 4.3-26.1) and for other sarcomas of the chest and breast (OR = 3.3, 95% CI = 1.1-1.7).

Adult↗

[Stewart-Treves syndrome arising in post-traumatic lymphedema].

The authors report a case-history of lymphangiosarcoma or Stewart-Treves syndrome which occurred in a patient with posttraumatic lymphedema of the arm. He presented two recurrences after surgery which led to the decision of amputation. He is now disease free nine years after this treatment. Initially defined as "lymphangiosarcoma arising in chronic lymphedematous extremities after mastectomy for breast cancer", Stewart-Treves syndrome complicates rarely traumatic, postsurgical, postradiation, idiopathic, congenital or filarial lymphedema. Clinical diagnosis is based on nodular, purple and frequently multiple skin lesions on chronic lymphedema of the limb. A proliferating malignant endothelium with affinity for anti-factor-VIII is found on histological slides. The tumor aggressivity explains the short survival if no treatment is given. Stewart-Treves syndrome prognosis is poor, with the occurrence of multiple local recurrences and pulmonary metastasis. On account of its rarity, there is no therapeutic consensus. In localized stage, radical surgery is usually performed. Conservative treatment with complete tumour removal and postoperative radiotherapy has not been yet evaluated. In metastatic or locally advanced tumours, it is necessary to study the benefit of cytotoxic drugs like anthacyclins and ifosfamide known to be effective on soft tissue sarcomas.

Amputation, Surgical↗

[Classification of vascular neoplasms].

Neoplasms of blood and lymph vessels differ from angiectatic and angiokeratotic nevi by real proliferating growth. According to their features of growth and their wall structures, they are classified into three main groups: angiomas, glomangiomas and malignant vascular tumors. Within the angiomas on the one hand, capillary angiomas are classified into: planotuberous and tuberonodous angiomas of childhood and Kasabach-Merritt syndrome, multilocular hemangiomatosis, progressive multiple angiomas, tardive ("senile") angiomas, eruptive angiomas (granulomata pediculata), papular angioplasia, gemmangioma, and benign juvenile hemangioendothelioma. On the other hand, cavernous angiomas, i.e. arterial and venous cavernomas, as well as blue rubberbleb nevus, Mafucci's syndrome, angioleiomyoma, benign juvenile hemangiopericytoma and cavernous lymphangioma, form thick walled structures without involution. Glomangiomas occur as solitary, multiple systematized, and multiple disseminated and familiar forms. Within the group of malignant vascular tumors--Kaposi sarcoma, lymphangiosarcoma in lymphedema, hemangioendothelioma and angioplastic reticulosarcoma, hemangio- or lymphangiosarcoma, angioendotheliomastosis proliferans, rarity and increasing loss of characteristic differentiated structures give rise to difficulties in nosologic classification.

Diagnosis, Differential↗

[Stewart-Treves syndrome of the edematous arm following breast cancer operation].

In 1948, Stewart and Treves (158) described a lymphangiosarcoma on the edematous arm for the first time with reference to six cases involving arm edema after breast cancer operation. Since their publication, a further 313 cases have become known. In 1982, with communication of one case we published a statistical compilation comprising of 248 cases (Zeitschrift für Lymphologie VI, 1982, 51-66). A statistical compilation totalling 290 observations is now presented on the basis of further studies of the older literature supplemented by observations of the last six years including two of our own cases. So far, a total of 319 single observations has become known: 21 cases of Yap et al. (182), eight cases of Oshmianskaia et al. (123) and 290 cases in the statistical compilation we have drawn up and which is the basis of this publication. By histological investigation of the tissue material taken from a female patient within six weeks (exploratory excision, radical excision), detailed information could be obtained on the histological development of the Stewart-Treves-Syndrome (STS) from a preneoplasia to a mature lymphangiosarcoma. The second female patient is a relatively rare observation of an STS of fulminant course which remained restricted to the extremity, chest wall and neighboring parts of the back during the entire course.

Aged↗

[Malignant tumors of lymphatic vessels in children].

Lymphangiosarcomas were found in 5 out of 82 observations of vascular malignant tumours in children, according to the data of the pathological department of Children's Clinical Hospital No. 1, for 60 years. Differential diagnosis of relapsing lymphangiomas and rarely found malignant lymphangioendetheliomas presents considerable difficulties for morphologists because of poorly manifest cell polymorphism in the primary node of neoplasia and the presence of areas of lymphangioma and malignant hemangioendothelioma among fields of lymphangiosarcoma. Cytological studies assist in specification of the degree of malignancy and histologenesis of the neoplasias.

Child, Preschool↗

A rare late complication of breast cancer treatment.

In 1948 Stewart and Treeves recognised and described six cases of lymphangiosarcoma arising in the oedematous upper limb of patients who had previously undergone radical mastectomy and radiotherapy for breast carcinoma. The incidence of lymphangiosarcoma in such patients is 0.45% and is uniformly aggressive and fatal, with a 5-year survival of less than 5% regardless of the treatment modality. We present a case report to increase the clinical awareness of this rare and fatal iatrogenic complication occurring subsequent to breast cancer treatment, and speculate on ways of improving survival.

Aged↗

Basement membrane in Kaposi's sarcoma: an immunohistochemical and ultrastructural study.

Basement membranes were investigated in early angiomatous and late sarcomatoid stages of Kaposi's sarcoma (KS). Seven frozen skin biopsies of KS from five elderly Mediterranean people and one renal allograft recipient were labelled, using an immunoperoxidase technique, for basement membrane-specific macromolecules, laminin and type IV-collagen. Twenty-seven other frozen cutaneous lesions including haemangio and lymphangiosarcomas, benign vascular tumours, and various epithelial, melanocytic, fibrohistiocytic, fibrosarcomatous and muscular tumours were processed in the same way. In addition an ultrastructural study was performed in two cases of KS, one haemangiosarcoma and one lymphangiosarcoma. Intense labelling was observed for both type IV-collagen and laminin, which appeared closely co-distributed, in all areas of KS. Staining pattern was often regular and continuous around neoplastic vessels in early lesions of KS, as in benign vascular lesions, whereas in late nodular lesions large amounts of basement membrane components were present in intercellular spaces between densely aggregated spindle cells. In contrast, ultrastructural examination disclosed early disruption of basement membranes around neoplastic vessels, and occasional fragments of external lamina were seen at the interface between KS spindle cells and collagen. Similar results were obtained in angiosarcomas both by immunohistological and ultrastructural study. In the comparative group, laminin and type IV-collagen were present, in significantly fewer quantities and in various distinctive patterns, in epithelial, melanocytic, fibrohistiocytic and muscular tumors. This study deals with basement membrane modifications in early and late lesions of KS and provides further evidence in favour of the endothelial nature of the spindle cells of KS.

Adult↗