LEIOMYOSARCOMAS OF THE HEART AND GREAT-VESSELS

Leiomyosarcomas (LMS) of the heart are exceptional primary malignant t umours with a catastrophic prognosis and a mean survival measured in m onths. Extensive radical surgical resection clearly remains the most a ppropriate treatment. We report three cases observed over a 3-year per iod, consisting of an LMS of the inferior vena cava, an LMS of the pul monary artery trunk and an LMS of the left atrium. The first case was treated by radical resection and reconstruction by autologous Vein gra ft of the cavorenal junction, the second case was treated by extensive resection and prosthetic reconstruction of the pulmonary artery bifur cation and the third case was treated by a first radical resection of the left atrium, requiring total cardiectomy and orthotopic heart tran splantation for local recurrence at the sixth month. The survical was significantly improved compared to other treatment options (chemothera py, radiotherapy). The first patient is still alive without recurrence at two years; the second died 12.5 months after the surgical procedur e and the medium-term follow-up of the transplanted patient revealed c erebral and hepatic metastases nine months after transplantation. The authors review the literature concerning these extremely rare malignan t tumours. Recent progress of diagnostic investigations, such as spira l CT with reconstruction, MRI, positron emission tomography (PET), are now able to establish the diagnosis more rapidly and therefore allow more radical surgical resection. This resection, possibly combined wit h Venous reconstruction, must be associated with adjuvant therapies. H eart transplantation should be considered among the treatment options for leiomyosarcomas of the heart, in order to improve the poor prognos is of these lesions affections a young population.