Primary venous leiomyosarcoma: A rare but lethal disease

Abstract

Primary venous leiomyosarcoma is rare, only 197 patients have been reported. To examine progress in diagnosis, treatment, and clinical outcome, we retrospectively reviewed data of 13 patients, 12 women and one man (mean age, 55 years; range, 19 to 75 years), who in the last 35 years underwent surgical treatment for primary venous leiomyosarcoma at our institution. The tumor arose from the inferior vena cava in eight, iliac vein in two, ovarian vein in one, and greater saphenous vein in two patients. Primary venous leiomyosarcoma was detected by physical examination in nine patients, symptoms were present in six. The 13 patients underwent 16 surgical procedures to resect primary (12), recurrent (2), or metastatic (2) tumors. Local excision alone was performed in seven, and local excision with reconstruction of the inferior vena cava or iliac vein was performed in six patients. The tumor was greater than 10 cm in eight patients. The perioperative mortality rate was 15% (2 of 13). Median survival of the 11 early survivors was 3.5 years (range, 6 months to 17 years). Currently five patients are alive (four of them free of known tumor), with a median survival of 3 years (6 months to 17 years) after surgery. Of the eight deceased patients, primary venous leiomyosarcoma recurred after resection in six. Tumor recurrence was not affected by tumor grade, size, or adjuvant treatment. Although early detection with modern imaging techniques could potentially be of benefit, wide local excision with selective venous reconstruction affords the only hope for prolonged survival.

Leiomyosarcoma of vascular origin: case report

“…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.…”

“…Most of them were high grade histologically, and 7/16 patients (44%) at the time of diagnosis. Dzsinich et al (1992) [5] reported on 13 cases of vascular LMS, which 8/13 arose from the inferior cava vein 2/13 from the iliac vein, and 2/13 cases from the saphenous vein. Svarvar et al (2007) [6] reported on 225 patients with LMS of all types from the Scandinavian Sarcoma Group with a cumulative survival of 49% at 10 years.…”

“…The definitive diagnosis of vascular LMS is performed by histological examination and immunohistochemistry techniques [4][5][6][7][8] . Macroscopic appearance or gross aspect of the tumor, it usually attains large size with rubbery consistency and soft if necrotic and usually show hemorrhage and bigger tumors often showed cystic structures and myxoid degeneration [3] .…”

“…Local [1,2] . It discus follow-up, and to three types a on, followed by LMS are a rare enous or arteria ors that origina ds to the femora MS could be c needs histolog or biopsy [5][6][7] . T cepted.…”

“…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] .…”