Radiology Service, Internal Medicine Department, Hospital Metropolitano, Quito, Ecuador.
Oncological Surgery, Surgery Department, Hospital Metropolitano, Quito, Ecuador.
J Cardiothorac Surg. 2023 Sep 1;18(1):256. doi: 10.1186/s13019-023-02344-9.
Intravascular leiomyomatosis (IVL) is a histologically benign smooth muscle tumor arising from the uterus that can spread through the pelvic veins and, on rare occasions, extend as far as the heart via the inferior vena cava. Despite its benign characteristics, it can behave like a malignant tumor leading to significant morbidity and even mortality if left untreated.
The patient is a 42-year-old woman with a past medical history of uterine leiomyomas. She presented with heavy bleeding and frequent spotting; therefore, she went to her gynecologist. After further evaluation, a mass within the uterus that expanded into the pelvic veins, inferior vena cava, and right atrium was discovered. After the complete removal of the mass, the patient underwent full recovery. IVL with cardiac extension was the final diagnosis.
Although IVL is rare, it must be considered in women who underwent previous hysterectomies or myomectomies and present with symptoms of right heart failure. The ideal therapy will need the aid of a multidisciplinary team and will depend on the patient's symptoms, previous operative history, the tumor's extension, and resectability.
血管内平滑肌瘤病(IVL)是一种起源于子宫的组织学良性平滑肌肿瘤,可通过盆腔静脉扩散,在极少数情况下,通过下腔静脉延伸至心脏。尽管它具有良性特征,但如果不治疗,它可能表现得像恶性肿瘤一样,导致严重的发病率,甚至死亡。
患者为 42 岁女性,既往有子宫肌瘤病史。她因大量出血和频繁点滴出血就诊。进一步评估发现,子宫内有一个肿块,已扩展至盆腔静脉、下腔静脉和右心房。肿块完全切除后,患者完全康复。最终诊断为血管内平滑肌瘤病伴心内延伸。
尽管 IVL 较为罕见,但对于既往接受过子宫切除术或子宫肌瘤切除术且出现右心衰竭症状的女性,必须考虑到这种疾病。理想的治疗方法需要多学科团队的协助,将取决于患者的症状、既往手术史、肿瘤的延伸范围和可切除性。