Clinical features and prognostic factors analysis of intravenous leiomyomatosis

Abstract

BackgroundThe treatment and prognostic factors of intravenous leiomyomatosis (IVL) remain lacking systematic evidence.MethodsA retrospective study was conducted on IVL patients from the Qilu Hospital of Shandong University, and IVL cases were published in PubMed, MEDLINE, Embase and Cochrane Library databases. Descriptive statistics were used for the basic characteristics of patients. The Cox proportional hazards regression analysis was used to assess the high-risk factors related to the progression-free survival (PFS). The comparison of survival curves was performed by Kaplan–Meier analysis.ResultsA total of 361 IVL patients were included in this study, 38 patients from Qilu Hospital of Shandong University, and 323 patients from the published literature. Age ≤45 years was observed in 173 (47.9%) patients. According to the clinical staging criteria, stage I/II was observed in 125 (34.6%) patients, and stage III/IV was observed in 221 (61.2%) patients. Dyspnea, orthopnea, and cough were observed in 108 (29.9%) patients. Completed tumor resection was observed in 216 (59.8%) patients, and uncompleted tumor resection was observed in 58 (16.1%) patients. Median follow-up period was 12 months (range 0–194 months), and 68 (18.8%) recurrences or deaths were identified. The adjusted multivariable Cox proportional hazard analysis showed age ≤45 years (vs. >45) (hazard ratio [HR] = 2.09, 95% confidence interval [CI] 1.15–3.80, p = 0.016), and uncompleted tumor resection (vs. completed tumor resection) (HR = 22.03, 95% CI 8.31–58.36, p < 0.001) were high-risk factors related to the PFS.ConclusionPatients with IVL have a high probability of recurrence after surgery and a poor prognosis. Patients younger than 45 years and with uncompleted tumor resection are at higher risk of postoperative recurrence or death.

Different clinical diagnosis end up in the same pathological diagnosis of intravascular leiomyomatosis: Two case reports

“…One-stage surgery and 2-stage surgery are optional according to patient conditions, and multidisciplinary cooperation is requisite in complex situations. [ 11 ] However, the size of IVL ranged from approximately 1.7 cm to 15.5 cm, and both of our patients were in stage I. To our knowledge, the size of IVL in case 1 is minimal according to the available English literature.…”

“…The incidence is low, but the recurrence rate postoperatively is very high. [9][10][11] Approximately 400 cases have been reported in the English literature since it first appeared in 1896. [10] Since a preoperative definite diagnosis could not be made by virtue of clinical symptoms and imaging analysis, the number of IVL cases remains underestimated due to its relatively insidious onset.…”

“…[10] Since a preoperative definite diagnosis could not be made by virtue of clinical symptoms and imaging analysis, the number of IVL cases remains underestimated due to its relatively insidious onset. [11][12][13] Following reasons such as asymptomatic IVL patients, undiagnosed early IVL patients, unreported limited lesions, etc, cause the morbidity to be underrated. Case 1, which was undiagnosed preoperatively, turned out to be an incidental IVL patient.…”

“…Some reports have postulated that the larger the mass is, the more likely it is to involve the cardiovascular system. [ 11 ] Patients with large lesions (≥7 cm) and lesions extending to the broad ligament may have an increased risk of recurrence, and parauterine metastasis is the first step toward extrapelvic involvement. [ 6 , 14 ] Although the mass filling abdominopelvic cavities of case 2 was very large, it was just expansive growth with no imaging evidence of iliac vein or inferior vena cava involvement.…”

Analysis of risk factors for post-operative recurrence or progression of intravenous leiomyomatosis

“… 7 Anti-estrogen treatment is currently the common post-operative adjuvant therapy. 11 Incomplete tumor resection is the main risk factor for recurrence or progression, 12 and thus, complete resection of the tumor is the goal of surgery. 13 …”