Intracardiac Leiomyomatosis Arising from a Uterine Leiomyoma – A Rare Case Requiring a Multidisciplinary Laparotomy–Thoracotomy Approach

ABSTRACT

A 37-year-old lady, when being evaluated in gynecology for pain in the abdomen, was found to have a pelvic mass suspicious of ovarian cancer with markers negative. There was an ovarian vein thrombosis extending to the right atrium in the contrast-enhanced computed tomography scan. A fluorodeoxyglucose positron emission tomography-computed tomography ruled out any other lesions. The patient underwent surgery under general anesthesia with transesophageal echocardiography to monitor the atrial thrombus. Intraoperatively, a retroperitoneal mass is seen arising from the right adnexal region of the uterus extending to the lumbar area. After the hysterectomy, bilateral salpingo-oophorectomy, tumor resection, and baring and looping of the retroperitoneal vessels, a sternotomy was done, and she was put on cardiopulmonary bypass. The tumor thrombus had two limbs both arising from the mass, one through the ovarian and the second through the iliac veins and joining together inside the inferior vena cava (IVC). With the excision of the ovarian vein at its junction, atrial incision, and incisions over the iliac veins and IVC, the thrombus was removed completely in a single sitting. Final histopathology revealed intravenous leiomyomatosis and no malignancy. We report this case as a rare disease, with both ovarian and iliac thrombus being a further rarity and a multidepartment joint effort with a successful outcome.

INTRODUCTION

Intravenous leiomyomatosis (IVL) is histologically benign and is characterized by the nodular linear growth of smooth muscle inside the vein. It has the potential to extend into the inferior vena cava (IVC), right heart, and pulmonary arteries. This is seen only in females, commonly in the 4^th decade, and can mimic malignancy.[1] This disease carries high morbidity and mortality.

CASE REPORT

A 37-year-old, with two living children, presented with complaints of right-sided lower abdominal pain for the past 3 months. On examination, the uterus was of normal size, a vague mass was tipped through the anterior fornix, and a cyst of 6 cm × 4 cm was felt in the left fornix. Contrast-enhanced computed tomography of the abdomen showed an abdominal-pelvic mass measuring 16 cm × 10.9 cm × 15 cm with nodular components noted in the right adnexa and extending to the infrarenal level. There was a right ovarian vein thrombus extending to the IVC and right atrium [Figure 1]. There was no evidence of disease in other organs in fluoro-deoxy-glucose positron emission tomography (PET). Tumor markers (Ca 125 – 15 units/ml, CEA – 0.89 ng/ml, and Ca 19-9 – 6.7 U/ml) were in the normal range.

Figure 1.

Axial and coronal section of CECT of the abdomen. The thick arrow shows IVL in the IVC. The red arrow shows the uterine leiomyoma. The blue arrow shows an enlarged ovarian vein with an IVL thrombus inside. CECT: Contrast-enhanced computed tomography, IVL: Intravenous leiomyomatosis, IVC: Inferior vena cava

After a multidisciplinary tumor board discussion (MDT), a team consisting of a surgical oncologist, cardiothoracic surgeons, cardiac anesthesiologist, and gynecologist performed the surgery. Intraoperative transesophageal echo was used to monitor tumor resection and guide fluid therapy. Additional monitors included nasopharyngeal temperature, hourly urine output, arterial blood gas, and activated clotting time.

She underwent a midline vertical laparotomy. A retroperitoneal mass of 16 cm × 15 cm × 10 cm arising from the right adnexal region attached to the uterus and extending into the right lumbar, right iliac, umbilical, and hypogastric area. There were flimsy adhesions between the mass, the bowels, and the bladder, and it extended retroperitoneally.

Proceeded with right adnexal/retroperitoneal mass removal, total abdominal hysterectomy, bilateral salpingo-oophorectomy, infracolic omentectomy, bilateral pelvic and retroperitoneal lymph nodes sampling. This was followed by median sternotomy and midline pericardiotomy. After heparinization, she was put on cardiopulmonary bypass with the aorta and bicaval cannulation. An aortic cross-clamp was applied, and antegrade root cardioplegic arrest was obtained. Venous return maintained with suction bypass. The superior vena cava looped, snugged, and the right atrium opened. There were two limbs for the thrombus both arising from the tumor, one was through the right ovarian vein, and the other limb was arising from the same mass but through the iliac vein and both joined inside the IVC and reached atria [Figure 2].

Figure 2.

(a) Gross specimen of TAH BSO, (b) IVL. IVL: Intravenous leiomyomatosis, TAH BSO: Total abdominal hysterectomy bilateral salpingo-oophorectomy

The tumor thrombus was removed by incising the IVC at the ovarian vein junction along with an incision over the atrium and iliac vein. Ovarian vein ligated. Anteromedial commissuroplasty of the tricuspid valve was done given moderate tricuspid regurgitation at the anteroseptal commissure related to the pressure changes related to the thrombus. Right atrium (RA) closure was done after de-airing and rewarming. The cross-clamp was taken off. The heart picked up in sinus rhythm. The thrombus was not infiltrating the vein wall, and its physical appearance was that of a tumor thrombus, and did not rupture.

The postoperative period was uneventful; the patient was extubated the next day and discharged on postoperative day 7 with aspirin and acenocoumarol.

Ascitic fluid cytology was negative for malignant cells. Histopathological diagnosis of the specimen was leiomyoma - suberous,broad ligament and left paraovarian with extensive hydropic change and intravenous extension [Figure 3]. The cells are smooth muscle actin-positive. All lymph nodes showed only reactive change, and the omentum showed only congestion.

Figure 3.

Histopathology specimen of IVL. H and E, ×40, shows elongated spindle tumor cells invaginating the vascular channels with no evidence of atypia. IVL: Intravenous leiomyomatosis

The MDT decided to follow up only for her.

DISCUSSION

The first description of this rare disease was by Birch-Hirschfeld in 1896.[2] 0.25% of uterine leiomyomas can have IVL as suggested by Ma et al., in their series of 76 cases.[3] Although IVL is histologically benign, it can grow through the iliac or ovarian veins into the vena cava ultimately reaching the heart. This mass of tissue extending through the veins does not adhere to the wall and usually is only partially occlusive.[4] A systematic review published in December 2022 describes 748 clinical cases, and no prospective studies were identified.[1] The average age of presentation is 47.6 years in the 187 patients’ data from Li et al.[5]

Depending on the maximum reached level of the IVL, four stages are described. Stage I is confined to the pelvic cavity, Stage II is when the tumor has extended into the abdominal cavity but has not reached the renal vein, Stage III reaches the atrium, and Stage IV is a pulmonary artery or lung metastasis.[3] Accordingly, our case comes in Stage III. In this lady, the thrombus had two limbs, one from the ovarian vein and another from the internal iliac vein both joining together above the renal vein junction inside the IVC. In the literature, only 5% of the IVL extends through both the iliac and ovarian veins simultaneously.[6] Magnetic resonance imaging, computed tomography (CT) angiogram, and echocardiography can help evaluate the location, size, and full extension pathway of IVL lesions. In PET CT, there can be mild or absent uptake as the histology is similar to benign leiomyoma.[7]

Complete excision is the treatment, and this can be done with a double approach – laparotomy–thoracotomy in a single-stage or two-stage procedure. Incomplete excision can result in recurrence.[5] Total hysterectomy is needed for those arising from the uterine leiomyoma, and removal of bilateral salpingo-oophorectomy may help as it is estrogen dependent. Adjuvant antiestrogen therapy may not add any benefit after complete resection, and it is to be used in incomplete resection and when surgery is not contemplated.[4,5]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.