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Case Reports
. 2018 Mar;97(11):e0051.
doi: 10.1097/MD.0000000000010051.

One-stage surgery for removal of intravascular leiomyomatosis extending to right ventricle

Affiliations
Case Reports

One-stage surgery for removal of intravascular leiomyomatosis extending to right ventricle

Ching-Shu Chiang et al. Medicine (Baltimore). 2018 Mar.

Abstract

Rationale: Intravascular leiomyomatosis (IVL) is a rare nonmalignant tumor that can be fatal if untreated.

Patient concerns: A 49-year-old nulliparous Asian woman who underwent hysterectomy and left salpingo-oophorectomy for multiple uterine leiomyomas 18 months prior presented complaining of intermittent palpitation and chest tightness for approximately 1 month. Echocardiography revealed a large mobile tumor mass extending from the inferior vena cava (IVC) to the right atrium that partially obstructed IVC flow and tricuspid inflow. Thoracicabdominopelvic computed tomography revealed a left adnexal tumor (4.8 × 2.5 cm) causing intravascular obstruction extending from the left internal iliac vein to the IVC, right atrium, and right ventricle.

Diagnosis: IVL with right heart involvement INTERVENTIONS:: Under cardiopulmonary bypass, a one-stage surgery combining sternotomy and laparotomy was performed. The tumor was approached and extracted via sternotomy, and tumor detachment and removal of residual tumors was accomplished via laparotomy.

Outcomes: A firm, smooth, and regularly shape tumor 15.5 × 5.5 × 2.5 in size was completely removed and histopathologically confirmed as IVL. The patient tolerated the surgical procedure well and no postoperative complication was noted.

Lessons: We describe a one-stage surgical approach to completely remove an IVL extending to the right ventricle.

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Conflict of interest statement

The authors have no funding and no conflicts of interest to disclose.

Figures

Figure 1
Figure 1
(A, B) Computed tomography showed a mass extending from left internal iliac vein to right ventricle. (C) The intracardiac tumor extended to right atrium and right ventricle. (D) The intracaval tumor. (E) The left internal iliac vein was totally occluded. (F) A tumor mass about 4.8 × 2.5 cm in size at the left adnexa. IVC = inferior vena cava, LCIV = left common iliac vein, LIIV = left internal iliac vein, RA = right atrium, RV = right ventricle, T = left adnexa tumor.
Figure 2
Figure 2
(A) Sternotomy and laparotomy were performed to expose the right atrium, right ventricle, and inferior vena cava. (B) Atriotomy was performed and the intracardiac part of the tumor mass was exposed. (C) The intracaval part of the tumor mass was detached through an inferior cava venotomy. A = aorta, IVC = inferior vena cava, RA = right atrium, RV = right ventricle, SVC = superior vena cava.
Figure 3
Figure 3
A firm, smooth, regularly shaped tumor 15.5 × 5.5 × 2.5 in size, originated from left internal iliac vein and extended to inferior vena cava, right atrium, and right ventricle. IVC = inferior vena cava, RA = right atrium, RV = right ventricle.

References

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