Abstract
BACKGROUND: Intravascular leiomyomatosis (IVL) of the uterus is extremely rare in clinical practice, often involving the pelvic and abdominal veins. Involvement of the heart and pulmonary arteries is rarer but can lead to severe clinical outcomes. It is difficult to diagnose the disease before operation, and most of them are found accidentally during the operation or postoperative pathology of uterine fibroids. This article will report a special case of IVL involving the heart, and comprehensively analyze and discuss the relevant domestic and foreign literature. Through real clinical cases, we hope to understand the core characteristics of this disease to guide clinical diagnosis and treatment. CASE DESCRIPTION: Here, we present the case of a 47-year-old woman with intrauterine venous leiomyomatosis whose tumor had spread to the right atrium. The patient lacked any distinct clinical symptoms. During uterine fibroid surgery, the tumor bolt was unintentionally discovered. The combined utility of multiple disciplines resulted in total removal of the tumor thrombus in the patient's venous system and right atrium during the same procedure. Following surgery, the patient achieved a significant recovery. After a follow-up of 2 years, there was no sign of neoplasm recurrence. CONCLUSIONS: IVL mainly occurs in premenopausal women. The early clinical manifestations are non-specific, and preoperative diagnosis is difficult. In order to improve the preoperative diagnosis rate, preoperative examination should be strengthened for patients with large uterine fibroids, long duration, chest tightness, shortness of breath, syncope and other medical history. During the operation, the pelvic and abdominal vessels should be comprehensively explored, and all intravascular lesions should be removed at one time, especially for those involving the heart, which often requires multidisciplinary collaboration. Complete resection of the lesion is the key factor affecting the recurrence. Endocrine therapy is an option for postoperative treatment, and it is used selectively according to the specific condition of the patient to reduce the risk of recurrence.