Management of intravenous leiomyomatosis: a case report illustrating two distinct surgical approaches.

Patient B was a nulliparous 45-year-old female who presented to the ED with palpitations, tachycardia, light-headedness, and a sensation of “bounding neck vessels.” Past medical history was significant for Raynaud’s disease. She had been on a combined oral contraceptive pill since age 19. Investigations demonstrated an elevated D-dimer with a right atrial filling defect on CT pulmonary embolism (CTPE) which was contiguous with the tumour in the IVC. CT of the abdomen and pelvis showed a multiloculated mass measuring 9 × 6 cm originating from the uterus. Imaging was highly suspicious of IVL arising from the right uterine vein, with growth into the right internal & external iliac veins, common iliac vein, and the IVC. A therapeutic dose of enoxaparin was initiated, and a biopsy of her pelvic mass confirmed leiomyoma. Her echocardiogram demonstrated a thrombus tracking into the RA and obstruction across the tricuspid valve. MRI of the pelvis and abdomen showed a 9 cm uterus with 9 cm exophytic fibroid originating from the posterior cervix and extending into the right pelvic sidewall. Tumour extension from this mass was seen in the right internal iliac vein, coursing into the right common iliac vein and IVC. Given the symptomatic and hemodynamic state of the patient, urgent operation to relieve cardiac obstruction was undertaken as an isolated procedure. The cardiac procedure began with a median sternotomy and opening of the pericardial sac, followed by heparinization and initiation of cardiopulmonary bypass (CPB) through an isolated SVC cannula using a 28 Pacifico cannula. The patient was cooled to 30 °C, and the heart–lung machine was turned off for a circulatory arrest, lasting 3 min, in order to facilitate tumor visualization and removal. After entry into the RA, traction was applied to the tumor to pull the IVC and hepatic portion of tumor into the atrium. However, this was not possible. The diaphragm was thus depressed towards the pelvis allowing for more tumour to be visible in the heart. Resection of the intra-cardiac portion was thus undertaken by transection of the tumour, after release of the diaphragm, the inferior resection margin was noted to be well into the infra-diaphragmatic, intrahepatic IVC ( Fig. 2 A). The total bypass time was 56 min, and no cross-clamp was required. Postoperatively, echocardiography showed mild physiological tricuspid regurgitation, which did not necessitate intervention. The procedure was completed with placement of two chest tubes, closure of the pericardial sac, and sternal fixation with seven wires. The patient tolerated the procedure well; 500 mL of blood loss was documented. The patient was in stable condition and discharged 1 week later on therapeutic anticoagulation. Fig. 2 A. Resection of intravenous leiomyomatosis intracardiac tumour component through sternotomy. Head to the left. Tumour being grasped by the forceps. B. Pelvic resection of the intravenous leiomyomatosis through incision into inferior vena cava. C. Both intravenous leiomyomatosis components resected in single-stage procedure. A. Resection of intravenous leiomyomatosis intracardiac tumour component through sternotomy. Head to the left. Tumour being grasped by the forceps. B. Pelvic resection of the intravenous leiomyomatosis through incision into inferior vena cava. C. Both intravenous leiomyomatosis components resected in single-stage procedure. One month later, Patient B underwent midline laparotomy, modified radical hysterectomy, bilateral salpingo-oophorectomy, para -aortic lymph node dissection, and an exploration of the IVC along with a patch cavoplasty to resect the remaining tumour. Intraoperatively, there was intravascular leiomyoma in the right uterine vein, right pelvic side wall, the uterine myometrium, and the IVC. First, the pelvic tumour was resected with a modified radical hysterectomy and resection of disease from pelvic side wall. To extract the remaining intracaval tumour, the retroperitoneum was mobilized off the anterior IVC, and the IVC was skeletonized for several centimetres above the confluence of the iliac veins. Intravenous heparin was administered (5000 units). Proximal and distal control was obtained, and a transverse cavotomy was performed ( Fig. 2 B). The tumour was grasped with a kelly clamp and pulled out of the IVC first. This was then repeated from the iliac veins. A tumour cast of the IVC, external iliac vein, and internal iliac vein was successfully extracted ( Fig. 2 C). A bovine pericardial patch was used to reconstruct the IVC. There were no surgical complications with 1150 mL blood loss and no gross residual tumour left. The patient was discharged home on acetylsalicylic acid (81 mg) for her bovine pericardial patch, deep vein thrombosis prophylaxis, and letrozole. The patient’s 3-month post-operative CT revealed a 4 cm mass resembling unresectable residual tumour in her right deep pelvic vein. Due to residual disease, letrozole was recommended but could not be tolerated due to side-effects.

Michal Moshkovich: Conceptualization, Data curation, Formal analysis, Investigation, Methodology, Resources, Writing – original draft, Writing – review & editing. Emily Volfson: Data curation, Formal analysis, Methodology, Writing – original draft, Writing – review & editing. Robert J. Cusimano: Writing – review & editing. Miranda Witheford: Writing – review & editing. Marcus Q. Bernardini: Writing – review & editing. Johannes Koen: Writing – review & editing. Rachel Soyoun Kim: Conceptualization, Data curation, Formal analysis, Investigation, Methodology, Project administration, Resources, Supervision, Writing – original draft, Writing – review & editing.

When imaging clearly shows a mobile tumour with no adherence to cardiac or vascular structures, an abdominal-only approach may be considered. In such cases, intraoperative echocardiography can confirm tumour size and mobility within the heart and IVC, allowing removal through an incision into the IVC. It also confirms no intraoperative dislodgement of the superior aspect of the tumour. If there is pre or intra-operative concern for tumour adherence to vessels or the heart, combined laparotomy and sternotomy may be required; however, trying to accomplish both at the same time may prolong hospital stay by imposing large physiologic demands on the patient. A single-stage operation involves complete resection of the tumour along with reconstruction of associated vascular structures in the same procedure. Unlike a two-stage operation, a single-stage procedure eliminates the risks of hemodynamic complications and tumour progression during the interval between two separate operations ( Zhang et al., 2010 , Lee et al., 2011 , Deng et al., 2021 ). However, this approach places significant physiological demands on the patient, making it less suitable for individuals with critical conditions such as heart failure, syncope, or other forms of hemodynamic compromise ( Wu et al., 2009 ). The combined effects of sternotomy and laparotomy contribute to reduced functional residual capacity, slower mobilization, and extended hospital stays due to their impact on respiratory mechanics and postoperative recovery. Furthermore, a single-stage operation may typically involve longer CPB durations, which are associated with an increased risk of adverse effects, including coagulopathy, systemic inflammatory responses, and organ dysfunction ( Paparella et al., 2002 , Bartoszko and Karkouti, 2021 ). Therefore, careful patient selection and meticulous perioperative planning are critical to mitigating these risks and optimizing surgical outcomes. A two-stage operation with cardiac involvement includes resection of the intracardiac and abdominal/pelvic tumor components in separate surgical procedures. This approach allows for a more controlled and tailored management of complex cases, particularly for patients with significant comorbidities or critical conditions such as heart failure, significant tricuspid regurgitation, or right ventricular obstruction ( Deng and Song, 2020 , Liu et al., 2009 , Peruyera and Vázquez, 2023 ). Two operations may also be more appropriate for extensive tumours that are intimately attached to intracardiac or intravenous structures ( Deng and Song, 2020 ). Furthermore, a two-staged procedure, or rarely a palliative approach, might be required for tumours with high adhesion or complex morphology. IVLs are hypothesized to be indolent, leading experts to comment that the treatment interval in a staged intervention is unlikely to impact completeness of tumour resection. Other studies conclude the interval may introduce a threat of embolization ( Zhang et al., 2010 , Liu et al., 2009 ). Two-stage procedures are associated with longer postoperative stays and a higher risk of tumour recurrence, yet they show no significant differences in morbidity, mortality, or postoperative complications compared to one-stage operations ( Deng and Song, 2020 , Wang et al., 2012 ). Managing IVL with cardiac involvement requires a carefully coordinated multidisciplinary approach to optimize surgical outcomes while minimizing risks. Decisions should be guided by a thorough preoperative assessment, including advanced imaging modalities such as CT, MRI, PET, and TEE, to evaluate tumour mobility and involvement of cardiac and vascular structures. Cases should be discussed in a multidisciplinary tumour board involving oncologists, radiologists, anesthetists, surgeons, and critical care specialists. This team approach ensures accurate patient selection for surgery and the development of an individualized surgical strategy, considering both the anatomical feasibility of complete resection and the patient’s surgical risk profile. Ultimately, the decision-making process must balance the potential benefits of tumour resection against the risks of surgery, with patient safety and optimal recovery as primary considerations. Ongoing intraoperative collaboration between surgical teams is essential to adapt the approach based on real-time findings, ensuring that management is both effective and patient centered.