Background Intravenous leiomyomatosis is a benign and rare smooth muscle cell tumor. Extension to the right heart is exceptional. Among the reported cases, the tumor is usually known to enter through the lumen of the iliac vein and grows into the inferior vena cava; involvement of bilateral gonadal veins is rarely reported. Complete tumor resection is the key to therapy. Case presentation A 25 year old female Chinese patient suffering from abdominal distention for 1 month, who was diagnosed with intravenous leiomyomatosis extending to the heart, from pre-operative imaging studies, is presented. A one stage procedure with complete excision of the tumor was performed. Histopathological findings confirmed the diagnosis of intravenous leiomyomatosis. The patient's post-operative recovery was uneventful, without recurrence and re-stenosis at 1 year follow up. Conclusion Intravenous leiomyomatosis may grow within veins along various routes. This case demonstrated intravenous leiomyomatosis with tumor extension through bilateral gonadal veins extending to the heart. It is believed that one stage radical resection can be a practical and effective alternative for the patients in good clinical condition. Long-term follow up is recommended because of the possibility of recurrence and metastases. Highlights ? Intravenous leiomyomatosis is a benign and rare smooth muscle cell tumor. ? Intravenous leiomyomatosis may grow within veins along various routes. ? Complete tumor resection is the key to the therapy for intravenous leiomyomatosis. ? One-stage radical resection can be a practical and effective alternative for patients who are in good condition.
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