PMC10313392_01
Preoperative assessment and surgical strategy formulation. (A-C) Echocardiography, computed tomography and magnetic resonance imaging showed intravenous leiomyomatosis (IVL) in different anatomical sites clearly. (D,E) 3-dimensional (3D) reconstruction using Materialise 3-matic (Leven, Belgium) software in the lateral view and front view, respectively. The red part is abdominal aorta, the transparent part is inferior vena cava (IVC), the purple part is IVL, the yellow part is bladder, and the fuchsia part is uterus. (F,G) The 3D printed model in the lateral view and front view, respectively. The red part is abdominal aorta, the transparent part is IVC, the green part is cardiac metastatic IVL, the blue part is uterine IVL, the pink part is bladder, and the yellow part is uterus. (H) After comprehensive evaluation, the surgical strategy was decided, transabdominal cardiac metastatic IVL and adnexal hysterectomy were proposed at the same time under off-cardiopulmonary bypass with the establishment of an external venous bypass (bilateral femoral vein-superior vena cava bypass) to maintain circulation.
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Echocardiography revealed a cord-like mass in IVC, RA, right ventricle (RV) and pulmonary artery (PA) that moved with cardiac contraction (Figure 1A). A 30 mm lesion could be seen in the right accessory area (Figure 1B). CTV (Figure 1C). Combined with the patient's hysteromyectomy history in 2020, the patient was finally diagnosed with cardiac metastatic uterine IVL and uterine fibroids. The 3D model was processed digitally, such as shell extraction, cutting, smoothing and repair, and the tissue structure and the shape of the false cavity were completely restored (Figures 1D,E). The Standard Tessellation Language (STL) file of the 3D reconstructed model was exported to Stratasys Polyjet 850 multi-material full color 3D printer. According to the 3D printed model, we could intuitively and fully understand the anatomical morphology of the right uterine adnexa, RIIV, RcIV, IVC, RA, RV and PA (Figures 1F,G). After multidisciplinary preoperative discussions, transabdominal cardiac metastatic IVL and adnexal hysterectomy were proposed at the same time under off-cardiopulmonary bypass with the establishment of an extracorporeal venous bypass (bilateral femoral vein-superior vena cava bypass) to maintain circulation (Figure 1H).
Main intraoperative steps and postoperative pathological examination results. (A) The uterus and the surrounding tissues were excised, and the arrow heads to the intravenous leiomyomatosis (IVL). (B,C) The tubula-like IVL was found in inferior vena cava (IVC). The arrow heads to IVL. (D) The excised tissues in uterus were sent to the pathological examination. The tumor cells are generated in the lobed shape in the vascular lumen. Immunohistochemical results: CD34 (+), Desmin (+), SMA (+), calponin (+), CD31 (+). This result was consistent with the preoperative diagnosis. (E) The excised tissues in IVL were sent to the pathological examination. Immunohistochemical results: CD34 (+), Desmin (+), SMA (+), calponin (+), CD31 (+). This result was consistent with the preoperative diagnosis.
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First of all, after the patient underwent general anesthesia, the gynecologist took the patient's lithotomy position to excise the uterus and the surrounding tissues, and the part of excised tissues were sent to the pathological examination (Figure 2A). The tumor was caught by angled clamps, and the blocking band near IVC was loosened to pull out the tumor in the heart cavity (Figures 2B,C). Postoperative pathological examinations verified IVL and uterine fibroids (Figures 2D,E).