Clinical characteristics and prognostic features of intravenous leiomyomatosis with inferior vena cava or intracardiac extension

• Published in: Journal of vascular surgery. Venous and lymphatic disorders (New York, NY) Vol. 5; no. 4; pp. 485 - 492
• Main Authors: Zhang, Guorui, MD, Yu, Xin, MD, Shi, Honghui, MD, Fan, Qingbo, MD, Lang, Jinghe, MD, Liu, Bao, MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.07.2017
• Subjects: Adult; Estrogen Receptor Modulators - therapeutic use; Female; Follow-Up Studies; Heart Neoplasms - diagnostic imaging; Heart Neoplasms - pathology; Heart Neoplasms - surgery; Hospitals, University; Humans; Leiomyomatosis - diagnostic imaging; Leiomyomatosis - pathology; Leiomyomatosis - surgery; Middle Aged; Neoplasm Invasiveness; Neoplasm Recurrence, Local - diagnosis; Neoplasm Recurrence, Local - pathology; Neoplasm Recurrence, Local - surgery; Neoplasm Staging; Prognosis; Retrospective Studies; Risk Factors; Surgery; Treatment Outcome; Uterine Neoplasms - surgery; Vascular Neoplasms - diagnostic imaging; Vascular Neoplasms - pathology; Vascular Neoplasms - surgery; Vascular Surgical Procedures - methods; Vena Cava, Inferior - diagnostic imaging; Vena Cava, Inferior - pathology; Vena Cava, Inferior - surgery
• ISSN: 2213-333X; 2213-3348
• DOI: 10.1016/j.jvsv.2016.12.015

Abstract Objective The aim of this study was to explore the clinical characteristics and prognostic features of intravenous leiomyomatosis (IVL) with inferior vena cava extension (IVCE) or intracardiac extension (ICE). Methods A retrospective analysis was conducted of the clinical data of 38 patients with pathologically diagnosed IVL with IVCE or ICE. Results The mean age of the patients was 44.05 ± 6.31 years. Twenty-two patients (57.9%) had a history of uterine leiomyoma. Nine patients (23.7%) had a history of IVL without IVCE or ICE. The most common symptoms were chest tightness (seven patients), lower limb swelling (five patients), palpitation (four patients), dizziness (two patients), and abdominal distention (two patients). Preoperative diagnoses were achieved in 28 patients (73.7%); 23 patients (60.5%) underwent one-stage surgery, whereas 15 (39.5%) underwent two-stage surgery. Eighteen patients (72%) received postoperative antiestrogen hormone therapy. Regular follow-up was available in 30 patients, with a median follow-up time of 12.0 months; 15 patients (50.0%) showed evidence of recurrence. Factors such as postoperative antiestrogen hormone therapy (9/20 compared with 6/10; P  = .601), duration of postoperative hormonal therapy (10/15 in >6 months compared with 5/15 in ≤6 months; P  = .862), and heart involvement (10/22 compared with 5/8; P  = .669) were not significantly associated with recurrence. Conclusions IVL with IVCE or ICE is a rare disease with nonspecific manifestations. Surgery is the primary treatment. The postoperative recurrence rate is high, and postoperative antiestrogen hormone therapy is not significantly correlated with recurrence.