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.

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The paper presents the results of treating 14 patients, namely eight patients with visceral artery aneurysms and six patients with visceral artery pseudoaneurysms. In 64.3% of the patients, the initial diagnosis was made based on the ultrasound examination. All the patients (100%) underwent CT angiography, while angiography was performed in 71.4% of the cases. Five (35.7%) patients with visceral artery pseudoaneurysms were emergently hospitalized; among them, the signs of bleeding were observed in 2 patients. In 9 patients, pathology was detected during tests for other conditions. Five (35.7%) patients underwent endovascular treatment, while 9 (64.3%) patients received surgical treatment. Endovascular interventions and open surgery demonstrated a nil mortality rate. After endovascular treatment, stent thrombosis was found in 1 patient. In the case of surgical treatment, visceral artery aneurysm was observed in 1 patient who underwent the resection of superior mesenteric artery pseudoaneurysm. Conclusions. The choice of the method of treating visceral artery aneurysms and visceral artery pseudoaneurysms depends on the location, size, anatomic features of the visceral arteries and the clinical course of the disease. Both endovascular and surgical treatment demonstrate good postoperative outcomes. Visceral ischemia is one of the most serious complications in the postoperative period, which can complicate both the diagnosis and the choice of treatment tactics.

The authors conclude that life long control is mandatory in order to detect late complications in patients who underwent operation of aortic coarctation in childhood.

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