Leiomyosarcomas of Vascular Origin in the Extremity

Abstract

Between 1996 and 2006 a total of 278 patients with soft tissue Leiomyosarcoma were treated at our centre. We identified 16 patients (5.8%) where the tumour directly arose from the blood vessels. These tumours were studied to determine their prognosis and behaviour. All tumors were in the lower limbs: 11 from the femoral vein, 3 popliteal vein, and 2 from the posterior tibial vein. Mean tumour size was 10.4 cm (3 to 33). Histological grade was high in all patients. Surgical treatment was amputation in one, excision with or without vascular reconstruction in 12 followed by radiotherapy, and 3 patients had no surgery because of advanced disease at diagnosis. Seven out of the 16 patients (44%) had metastasis at diagnosis, and five patients without metastasis at diagnosis rapidly developed metastases at a median time of 5 months from diagnosis (2–30 months). The overall survival of the patients at 5 years was 25% which was considerably worse than those with nonvascular leiomyosarcoma. We conclude that patients with leiomyosarcoma of vascular origin have a very high risk of metastases and poor prognosis when treated in the conventional way.

Leiomyosarcoma of vascular origin: case report

“…Regarding to the follow-up, the results of Abet series [4] showed a bad outcome in vascular LMS with 75% of patients died with metastatic disease within the first 3 years of finding, while good local control by surgery and radiotherapy are achieved, while, those results are very similar to the series of Berlin et al (1984) [8] Leu and Makek (1986) [9] and Hadju et al (1979) [10] .…”

“…Vascular LMS is a rare tumor origin the media of vessel walls, low extremities veins are the most commonly veins affected, furthermore, the popliteal and tibial veins even less frequently, some few cases have been published [1,[4][5][6] . The diagnostic of leiomyosarcoma, it must first be distinguished from other types of tumors, that differentiate these tumors from other soft tissue sarcomas it's not easy to do, a good histological and immunohistochemical study using markers are useful for the diagnosis.…”

“…There have been only a few hundred published reports of vascular LMS. This type of lesions blocks the veins by either intraluminal or extra luminal participation or by external pressure, due to tumors growing in the surrounding tissues [4] . Abed R et al…”

“…Sarcomas are generally classified according to the normal cell line that they most closely look like, which stands up from mesenchymal cell lines. Of all soft tissue sarcomas, around 5%-10% are leiomyosarcoma (LMS) [1][2][3][4] . Sarcomas are rare malignant tumors with a large variety of histologic subtypes, approximately 1% of adult malignancies [1] , for high-risk factor, known by tumor grade, size and location, local control is enhanced with postoperative adjuvant radiation.…”

“…The CT show , it displaced th Figure 1). T ed a polypoida sistency, light emorrhagic foc mposed of diff Figure 3A, 3B itotic figures w chemistry reve in (SMA [4][5][6] . A correc hy [4] .…”