DIAGNOSIS, TREATMENT AND PROGNOSIS OF THE LEIOMYOSARCOMA OF THE INFERIOR VENA-CAVA

Objective: To describe our experience with three cases of leiomyosarcoma of the inferior vena cava, and summarise current methods of diagnosis and treatment. Design. Descriptive study. Setting: University hospital, Germany. Subjects: 3 Patients with histologically confirmed leiomyosarcoma of the inferior vena cava. Interventions: Resection, with or without vascular reconstruction. Main outcome measures: Morbidity and mortality. Results: Case 1 was diagnosed on magnetic resonance imaging, the tumour was resected, and the vena cava replaced with a polytetrafluoroethylene (PTFE) graft. She made an uncomplicated recovery and was well and free of symptoms 10 months later. Case 2 was diagnosed at operation (the differential diagnosis on computed tomography was a retroperitoneal tumour), the tumour was resected, and the defect on oversewn. She made an uneventful recovery and was well and free of symptoms 14 months later. Case 3 was diagnosed at operation (a diagnosis of pancreatic cancer had been made preoperatively), the tumour a as resected, and the defect oversewn. She was well and free of symptoms IO months after operation. Conclusions: Magnetic resonance imaging is superior to computed tomography in the diagnosis of leiomyosarcoma of the inferior vena cava. The treatment of choice is resection; small defects can be closed by suture or PTFE patch. and large defects by PTFE prostheses. Reported resectability is 40% to 60%, but the prognosis is poor: the local recurrence rate is about 36%, most patients are dead within 2.5 years, and the 5-year survival is 30%. Chemotherapy and radiotherapy will give some degree of palliation. but do not affect the outcome.