PMC4863127_01
Computed tomogram of the chest, abdomen, and pelvis showed a large mass within the inferior vena cava, extending into the right atrium and ventricle. (a) Coronal image demonstrates large filling defect in the right parametrium. (b, c) Coronal image presents large filling defect in the inferior vena cava extending from both the right uterine vein and common iliac and the right ovarian vein to the inferior vena cava and the right atrium. (d) Coronal image demonstrates large filling defect in the right atrium.
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fig1
We identified two extension pathways of intravenous tumor originated from the posterior uterine wall (Figure 1).
Magnetic resonance imaging (MRI): (a) on T2 weighted image, an irregular tumor is identified in the right posterior wall of the uterus, which exhibited heterogeneous high signal intensity (arrowheads); (b) the right uterine vein and ovarian vein are dilated, and low and high intensity masses are depicted in the veins and originated from the high intensity tumor in the posterior uterine wall (arrowheads); (c) sagittal view of dynamic contrast-enhanced MRI demonstrates large filling defect in the inferior vena cava with gradually increasing enhancement (upper arrowheads) and poor enhancement of tumor in the posterior uterine wall (lower arrowheads).
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fig2
Dynamic contrast-enhanced MRI using gadolinium with diethylenetriaminepentaacetate revealed the enhancement of the tumor in IVC (Figure 2).
Intraoperative findings. Tumor in (a) the right atrium (arrow heads), (b) the inferior vena cava (arrow heads), (c) the right ovarian vein (arrow heads), and (d) the right uterine vein (arrow heads).
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fig3
The operation was performed under general anesthesia (Figure 3).
Extirpated uterus, bilateral ovaries and tubes, and tumor in the inferior vena cava extending from both the right uterine vein and common iliac and the right ovarian vein to the inferior vena cava and the right heart.
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fig4
Complete tumor resection was achieved with an estimated blood loss of 1,045 mL (Figure 4).
(a) Histopathological and immunohistochemical analyses show low-grade endometrial stromal sarcoma. The primary tumor in the uterus comprises CD10-positive short spindle cells resembling the stromal cells of proliferative endometrial tissue. Also spindle cells with abundant cytoplasm and ellipsoidal nuclei (alpha-SMA-positive cells) are mixed with the tumor cells. (b) The intracardiac tumor shows extensive smooth muscle differentiation. Main part is occupied with the spindle cells (alpha-SMA-positive). Scattered aggregates of small darker cells (CD10-positive) are still recognizable.
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fig5
LG-ESS were still recognizable. LG-ESS was histologically confirmed in the right parametria and IV tumor (Figure 5).