Intravenous leiomyomatosis is often seen in perimenopausal women, which is clinically rare and often associated with a history of uterine leiomyomas. It can grow along the vasculature in a very aggressive fashion. Uterine venous leiomyomas involving the right heart are extremely rare and available literature is mostly individual cases [5, 6].

IVL confined to the inferior vena cava has no obvious symptoms, but at the time symptoms occur, most tumors have invaded the heart. There is lack of effective standards for etiology, classification, and treatment methods. Regarding the origin of the ICL, one view is that the tumor originates from the venous vascular wall in the uterus or pelvis. Other holds the view that the ICL is a further extension of the uterine leiomyoma to the vasculature [7]. Most ICLs extend from the uterus to the heart but interestingly, although some patients have a history of uterine leiomyoma but uterine leiomyoma and cardiac leiomyoma do not have anatomical continuity and ICL arises from the heart walls itself [2]. Due to its extension from pelvis to the heart, IVL is considered a benign tumor with malignant behavior [3]. In this case, there was a history of uterine leiomyomectomy, however, the pedicle of cardiac leiomyoma originated from the inferior vena cava. Li et al. [4] found that ICL originating from the uterus usually has a serpentine appearance macroscopically, with IVL extends from ovarian or iliac vein into the IVC and RA. In contrast, some cases with ICL arising from the venous smooth muscle wall itself have been reported [4].

The tumor is generally smooth and rubbery, with a greyish-white or rusty color, with or without an intumescent intracardiac head. In patients with a long course of this disease, the tumor may be calcified and extended more than 50 cm [4]. Immunohistochemical staining is positive for SMA, a smooth muscle markers [8]. Evidence suggests that the etiology of cardiac leiomyoma is not only related to uterine leiomyomatosis but may also be related to other factors. Current literature reports that the vast majority of ICLs occur in perimenopausal women [4], which suggests that the tumor is closely related to the estrogen changes in the body. Generally, echocardiography is a more convenient method and can provide real-time information about an intracardiac tumor. CT and MRI can clarify the tumor anatomy and its relation with adjacent structures.

In terms of ICL morphological characteristics, to achieve the goal of complete tumor resection, the general surgical procedure needs to involve the pelvic cavity, abdominal cavity, thoracic cavity, and great vessels. It requires multidisciplinary team cooperation involving cardiovascular surgeon, general surgeon, and gynecology and obstetrics surgeon. Generally, a combined thoracoabdominal incision under extracorporeal circulation is used to remove the tumor [3, 9, 10]. In this case, according to the location of the pedicel of cardiac leiomyoma which originated about 3 cm from the inferior vena cava opening into the RA, a single median sternotomy was done. After the establishment of CPB, the silk suture was used to make a snare to cover and ligate the pedicel of the tumor, and then a scalpel was used to remove the tumor, avoiding an abdominal incision. This approach theoretically improves the feasibility of successful single sternotomy incision surgery and eliminating the need for an abdominal incision even if the pedicle of the tumor is far but within a reachable location from the opening of IVC. A single abdominal incision approach with peripheral CPB has been performed for ICL without intracardiac portion dilation of the tumor [6]. One-stage and two-stage operation for ICL has also been reported [3, 7, 11]. An individualized and reasonable surgical plan is required on the premise of fully assessing the patient history, imaging data, and surgical tolerance. Research shows that complete resection has good short-term and long-term results, with no reports of recurrence or postoperative death [4, 12]. However, in 33.3% of patients, recurrence occurs after incomplete resection [4]. Recurrence of ICL is not associated with favorable outcomes [5]. Therefore, close postoperative follow-up is necessary.

Combined with medical history, echocardiography, CT, and other tools can help in diagnosis, and diagnosis is generally not difficult. Once ICL is diagnosed, curative care should be carried out, and the surgical plan should be fully evaluated as far as possible. Individual incisions should be formulated according to the scope and origin of the tumor, and multiple and large incisions should be avoided as far as possible. Follow-up and observation should be carried out to detect tumor recurrence at an early stage.