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.

Keywords: Intravenous leiomyomatosis (IVL); incidentally diagnosed; management; prognosis.

Figure 1

The clinical symptoms of IVL patients. AUB, abnormal uterine bleeding; IVL, intravenous leiomyomatosis.

Figure 2

The hematoxylin and eosin staining of IVL (×40/×200). Benign smooth muscle cells present within venous vascular spaces. The black arrow shows the venous wall. IVL, intravenous leiomyomatosis.

Figure 3

Representative Immunohistochemical images of typical markers in IVL lesions (×200). IVL, intravenous leiomyomatosis; ER, estrogen receptor; PR, progesterone receptor; SMA, smooth muscle actin.

Figure 4

Management and prognosis in the incidental group. TH, total hysterectomy; TH + BSO, total hysterectomy with bilateral salpingo-oophorectomy; MIS, minimally invasive surgery; AWoD, alive without disease; AWD, alive with disease; IVC, inferior veno cava.