Surgical Strategy for Intravenous Cardiac Leiomyomatosis

Intravenous-cardiac leiomyomatosis (IVCL) is a rare, histologically benign but biologically aggressive tumour. Accurate diagnosis and appropriate treatment choices are important for prognosis. The best surgical approach remains unclear owing to limited evidence. This study aimed to assess surgical strategies for treating IVCL and to propose individualised surgical strategies. We searched PubMed, Web of Science, and the Cochrane Library to identify case reports and case series published in English over the last 10 years. Two (2) reviewers independently screened the literature according to the inclusion and exclusion criteria, and subsequently extracted data. One hundred and ten (110) cases were included. According to our analysis, IV cardiac leiomyomatosis is most common in the fifth decade, and the mean age at detection is 45.71±7.67 years. Most patients had undergone previous hysterectomy/myomectomy, or had a co-existing uterine leiomyoma when admitted. The most common clinical presentations were dyspnoea, palpitation, pelvic mass, and leg oedema. More patients benefited more from one-stage surgery. Seventy-eight per cent (78%) of cases experienced a surgical approach of thoracic and abdominal incisions. Extractions of IVCL were varied. Eighty-seven (87) patients experienced cardiopulmonary bypass and deep hypothermic circulatory arrest was performed on 64.4% of them. Total hysterectomy and bilateral oophorectomy, together with pelvic leiomyoma excision, were done in 76.0% of patients. Overall, surgical strategies for IVCL are varied; the optimal strategy required consideration of multiple factors. Complete resection is recommended for both single- and two-stage operations. Once complete resection is achieved, recurrence is rare.