Retrograde growing intravenous leiomyomatosis—a case report

Introduction

Leiomyomatosis is a benign tumor that usually originates from smooth muscle cells and can appear at any location (1). Intravenous leiomyomatosis (IVL) usually grows along blood vessels and extends toward the iliac vein and the inferior vena cava (IVC). It extends toward the right heart cavity and the main pulmonary artery in extreme cases. IVL is a rare smooth muscle tumor that is histologically benign but clinically aggressive (2,3). It is not typical for IVL to develop metastases, but rather to grow progressively and cause mechanical compression in surrounding tissues (3). The current literature supports two theoretical hypotheses. The first hypothesis is that IVL is formed by the growth of the pre-existing uterine leiomyoma and invasion into the vascular lumen, while the second is that IVL is considered to be the formation of smooth muscle cells in the uterine vein wall; growing evidence supports the former. All patients with IVL had a combination of uterine smooth muscle tumors or a history of smooth muscle disease. Immunohistochemical staining revealed that the tumor-involved vascular endothelial and subendothelial layer cells were estrogen and progesterone receptor-negative. In contrast, tumor cells of IVL are positive for estrogen and progesterone receptors (4).

Early diagnosis and surgical treatment of IVL are important because this condition can cause cardiac symptoms as well as pulmonary embolism and sudden death (4). We report a case of retrogradely growing IVL, originating in the uterus and extending along the venous system to the right internal iliac vein, common iliac vein, IVC, and right heart. At the same time, this leiomyomatosis retrogrades into the right external iliac vein (4,5). We present this case in accordance with the CARE reporting checklist (available at https://acr.amegroups.com/article/view/10.21037/acr-24-196/rc).

Case presentation

A 48-year-old woman with a mass in the IVC was admitted to the Wuhan Union Hospital. Her medical history included three pregnancies, two deliveries, and one miscarriage. She had no history of hormonal contraceptive exposure. Vital signs on admission were: blood pressure 110/86 mmHg, temperature 36.5 ℃, respiratory rate 20 breaths per minute, and pulse 86 breaths per minute. She had undergone a hysterectomy for fibroids 2 years previously. She had repeated syncope in the past three months, with a loss of consciousness that could be relieved on its own, along with palpitations and chest distress after physical activity. The patient presented to our hospital with an outpatient diagnosis of an IVC mass. Her medical and family histories were unremarkable, and physical examination and vital signs were normal.

Doppler ultrasound examination excluded deep vein thrombosis, and blood flow from the legs was normal. Computed tomography venography and three-dimensional reconstruction revealed filling defects in the IVC, right internal and external iliac veins, right common iliac vein, right renal vein, and right atrium. The filling defect was suggestive of IVL. Magnetic resonance venography of IVL (Figures 1-3) revealed filling of the IVC, left external iliac veins, right internal and external iliac veins, bilateral common iliac veins, and right atrium. IVL were taken into consideration.

Figure 1 Magnetic resonance imaging of the case. Inferior vena cava tumor shown by arrows.

Figure 2 Magnetic resonance imaging of the case (coronal section). Inferior vena cava tumor shown by arrows.

Figure 3 Magnetic resonance imaging of the case (cross section). Inferior vena cava tumor shown by arrows.

All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

The patient underwent resection of the vena cava tumor, cardiac masses, and iliac vein lesions. During surgery, the entire IVC, right internal and external iliac veins, part of the right common iliac vein, and right atrium were almost completely filled with white masses (Figure 4). The tumor retrograded towards the right common iliac vein. After a multidisciplinary consultation, IVC leiomyomatosis was considered, and surgical treatment was performed. The surgeons performed laparotomy and resection of the tumor in the IVC, right common iliac vein, right external iliac vein, right internal iliac vein, left common iliac vein, and left external iliac vein. The tumor envelope remained intact after removal (Figure 5).

Figure 4 Vein leiomyoma growth path. The black arrows show the direction of inferior vena cava tumour extension. Tumour of the inferior vena cava is shown in the red area.

Figure 5 Gross images and intraoperative findings of the case.

Tumor cells were positive for CD34, smooth muscle actin (SMA), desmin, and H-caldesmon, but negative for S100, MelanA, and HMB45 on immunostaining. Ki-67 index was approximately 5%. Based on the surgical, imaging, and pathological findings, the final diagnosis was IVC leiomyoma (Figure 6).

Figure 6 Typical histopathology of intravenous leiomyomatosis (hematoxylin & eosin staining). The original magnification is 20×, 40× and 40× respectively.

Discussion

IVL is a rare and specific type of uterine smooth muscle tumor. To date, fewer than 400 cases of IVL have been reported in the English literature worldwide (5). Currently, most of the literature at home and abroad consists of case reports, and analyses of the clinical features of IVL at different sites are lacking. However, the etiology of IVL is not clear (6). The clinical manifestations of IVL depend mainly on its location and extent. Early symptoms resemble typical uterine fibroid, which can lead to fainting or sudden death if large blood vessels, the heart, or the lungs are involved.

Three tumor extension routes exist from the uterus to the IVC: the internal iliac, ovarian, and anterior sacral veins (4,7). All these routes are prograde-growing. Here, we report a case of retrogradely growing IVL. It originates in the uterus, extends along the venous system to the right internal iliac vein, and grows retrogradely into the external iliac vein (8). The authors believe that this is a new growth path that has rarely been mentioned. Previously, prograde IVL was thought to have progressed. When we noticed an abnormality in the external iliac vein on imaging, we assumed that it was due to a thrombus. After a complete surgical resection, we realized that the IVL could grow in a retrograde manner. There is limited literature related to the retrograde growth of IVC tumors (9). Most studies have reported anterograde growth of venous leiomyoma tumors invading the IVC as well as more distant heart. For example, Price et al. reported that venous leiomyomas extend through the uterine channel into the venous system and then into the cardiac chamber (10). This case provides a better understanding of the extent and pathway of IV leiomyomatosis. Therefore, a larger anatomical scope should be considered when performing surgery.

Conclusions

we report a case of retrogradely growing IVL. It originates in the uterus, extends along the venous system to the right internal iliac vein, and grows retrogradely into the external iliac vein. This is a new growth path that has rarely been mentioned.

Acknowledgments

None.

Footnote

Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://acr.amegroups.com/article/view/10.21037/acr-24-196/rc

Peer Review File: Available at https://acr.amegroups.com/article/view/10.21037/acr-24-196/prf

Funding: None.

Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://acr.amegroups.com/article/view/10.21037/acr-24-196/coif). The authors have no conflicts of interest to declare.

Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration (as revised in 2013). Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.

Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.

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