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Competing Interests: Disclosure The authors report no conflicts of interest in this work.

Angiosarcoma represents only 2.4% of soft tissue malignancies. Primary angiosarcoma of the breast is also a rare neoplasm. The incidence of this disease has been reported to be 0.04% of all breast malignancies. The aetiology is unknown. Angiosarcoma associated with chronic lymphoedematous arm of a postmastectomy woman was described by Stewart and Treves. In this paper we describe a 62-year-old female who underwent wide excision, axillary node dissection and adjuvant radiotherapy for primary breast cancer. After 5 years she noticed a purple discolouration of the treated left breast. Biopsy of these areas confirmed an angiosarcoma. No haematogenous dissemination was found. She underwent simple mastectomy without systemic therapy. A chest X-ray 3 months later revealed diffuse bilateral lung metastases. The incidence of breast conserving therapies is growing fast. Until now we have no evidence on the role of irradiation as an aetiological reason for angiosarcoma after breast-conserving surgery and axillary node dissection. Therefore, we have to pay increasing attention to these patients. A patient with history of radiotherapy after breast conserving therapy and skin discolouration must undergo a biopsy of these areas. The only therapy of angiosarcoma is an early mastectomy. The success of chemotherapy is uncertain. Possibly adjuvant chemotherapy with actinomycin D for the group with poorly differentiated tumours appears beneficial. Prospective studies might help to resolve this question.

The Stewart-Treves syndrome is an angiosarcoma that occurs due to chronic lymphoedema which in most cases is a complication after mastectomy with axillary node dissection and postoperative radiation. Prognosis for this rare tumour is poor. The best therapy is early and radical excision. Chronic lymphoedema seems to be an important pathogenetic factor. We report on a 70-year old patient with early stage lymphangiosarcoma who had a lumpectomy with axillary node dissection and postoperative radiation five years ago.

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.

A case of Stewart-Treves-syndrome is presented in which the sarcoma appeared seven years after mastectomy and irradiation.