Management and prognosis comparison between incidental and nonincidental intravenous leiomyomatosis: a retrospective single-center real-life experience

Abstract

Background: Intravenous leiomyomatosis (IVL) is a rare, difficult-to-treat type of smooth muscle tumor that originates from the uterine myoma. However, its clinical characteristics, management, and prognosis are not clearly understood. Moreover, the 2 different methods used to diagnose IVL-incidental and nonincidental-result in completely different treatments. Methods:We conducted a single-center, retrospective study. Our real-life case series included patients pathologically diagnosed with IVL between July 2011 and December 2020. All patients with IVL were divided into 2 groups: an incidental group and a nonincidental group. Medical records of patients, including clinical characteristics, primary treatment, treatment after recurrence, and prognosis, were reviewed.Results: A total of 39 patients were included in the study, with a median patient age of 47 years. Of the 39 cases, 15 (38.5%) were incidentally diagnosed with only intrapelvic tumors. Among the 24 patients with IVL in the nonincidental group, tumor spread in the inferior vena cava, right heart, and pulmonary artery was identified in 4, 17, and 3 patients, respectively. The most common symptoms were abnormal uterine bleeding in the incidental group and chest distress and dyspnea in the nonincidental group. Among the 15 patients in the incidental group, ovary-preserving surgery was performed in 6 young women (≤40 years old), of whom 3 underwent myomectomy. All 24 patients with IVL in the nonincidental group underwent thrombectomy without uterine or ovary preservation by multidisciplinary surgical treatment.Only 1 patient in each group underwent postoperative adjuvant therapy. During the median follow-up of 36.0 months, recurrence was recorded in 5 (12.8%) cases in the incidental group, with no deaths recorded.Only 1 patient was lost to follow-up. No recurrence was noted in the cases in the nonincidental group.Among the 5 patients who experienced recurrence, 4 received secondary surgical treatment and 1 received hormone therapy. All patients were alive as of this report.Conclusions: Patients with IVL who are diagnosed incidentally have a higher recurrence risk than those who are diagnosed nonincidentally and undergo complete tumor resection. However, patients incidentally diagnosed with IVL can still experience long disease-free survival rates following secondary surgical treatment after recurrence.

Molecular pathological approach of uterine intravenous leiomyomatosis

“…Shi et al . reported that patients with IVL diagnosed incidentally seemed to be at a higher risk of recurrence than those who were diagnosed non-incidentally and underwent complete tumor resection ( 5 ). However, incidentally diagnosed patients with IVL can still experience extended disease-free survival after receiving secondary surgical treatment for recurrence ( 5 ).…”

“…reported that patients with IVL diagnosed incidentally seemed to be at a higher risk of recurrence than those who were diagnosed non-incidentally and underwent complete tumor resection ( 5 ). However, incidentally diagnosed patients with IVL can still experience extended disease-free survival after receiving secondary surgical treatment for recurrence ( 5 ). The medical evidence obtained from this clinical study provides medical staff with important information for selecting the appropriate treatments for patients with IVL.…”

Magnetic resonance imaging findings of intravascular leiomyomatosis of the uterus: a case report

“…Through conducting a PubMed search with the specific keywords “intravenous leiomyomatosis,” we identified 2,338 studies. However, through conducting a PubMed search with the specific keywords “intrapelvic intravenous leiomyomatosis,” we identified two studies ( 6 , 7 ). IVL is a benign tumor caused by the proliferation of smooth muscle cells within the vasculature.…”

“…However, research indicates that for certain stage I patients who are young women (≤40 years old), it is possible to preserve fertility by undergoing tumor excision alone as the main treatment. Hysterectomy or BSO (bilateral salpingo-oophorectomy) is not necessary in these cases ( 7 ). All patients graded stage II or above received total tumor resection, abdominal hysterectomy, and bilateral salpingo-oophorectomy.…”

Safety and efficacy of sirolimus in recurrent intravenous leiomyomatosis, pulmonary benign metastatic leiomyomatosis, and leiomyomatosis peritonealis disseminata: a pilot study

“…Depending on the range of the lesions, surgeries might be achieved through multidisciplinary collaboration, including gynecologists, vascular surgeons, and cardiac surgeons, with the aim of complete resection [ 3 ]. However, the postoperative recurrence rate is as high as 14–31.0% [ 1 , 4 , 5 ]. Benign metastatic leiomyomatosis refers to the presence of benign leiomyomas outside the uterus, which often occurs in patients who have previously undergone myomectomy or hysterectomy.…”