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[Leiomyosarcoma of the heart and great vessels]
G Babatasi1, M Massetti, D Agostini
1Département d'Anatomie Pathologique, CHU Caen Côte de Nacre, France.
Insights
Radical surgical resection offers improved survival for rare heart leiomyosarcomas (LMS). Advanced diagnostics and potential heart transplantation are crucial for managing these aggressive tumors.
Area of Science:
- Cardiovascular Surgery
- Surgical Oncology
- Medical Imaging
Background:
- Primary cardiac tumors are rare, with leiomyosarcomas (LMS) presenting a particularly poor prognosis and short survival.
- Extensive radical surgical resection is the primary treatment modality for cardiac LMS.
Observation:
- Three cases of cardiac LMS are presented: inferior vena cava, pulmonary artery trunk, and left atrium.
- Treatments included radical resection with autologous vein graft, prosthetic reconstruction, and radical resection with orthotopic heart transplantation for recurrence.
Findings:
- Surgical resection, particularly when combined with reconstruction or transplantation, demonstrated improved survival compared to chemotherapy or radiotherapy.
- One patient remained recurrence-free at two years post-surgery; another survived 12.5 months.
- The heart transplant patient developed metastases nine months post-transplantation.
Implications:
- Advanced imaging (CT, MRI, PET) aids in earlier diagnosis and more radical surgical planning for cardiac LMS.
- Adjuvant therapies and venous reconstruction should complement surgical resection.
- Heart transplantation is a viable option to improve outcomes for younger patients with cardiac LMS.
Abstract:
Leiomyosarcomas (LMS) of the heart are exceptional primary malignant tumours with a catastrophic prognosis and a mean survival measured in months. Extensive radical surgical resection clearly remains the most appropriate treatment. We report three cases observed over a 3-year period, consisting of an LMS of the inferior vena cava, an LMS of the pulmonary artery trunk and an LMS of the left atrium. The first case was treated by radical resection and reconstruction by autologous vein graft of the cavorenal junction, the second case was treated by extensive resection and prosthetic reconstruction of the pulmonary artery bifurcation and the third case was treated by a first radical resection of the left atrium, requiring total cardiectomy and orthotopic heart transplantation for local recurrence at the sixth month. The survical was significantly improved compared to other treatment options (chemotherapy, radiotherapy). The first patient is still alive without recurrence at two years; the second died 12.5 months after the surgical procedure and the medium-term follow-up of the transplanted patient revealed cerebral and hepatic metastases nine months after transplantation. The authors review the literature concerning these extremely rare malignant tumours. Recent progress of diagnostic investigations, such as spiral 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 with venous reconstruction, must be associated with adjuvant therapies. Heart transplantation should be considered among the treatment options for leiomyosarcomas of the heart, in order to improve the poor prognosis of these lesions affections a young population.