Abstract
Objective
To propose an ultrasound-based classification system for intravenous leiomyomatosis (IVL) and to describe the sonographic features of each type using standardized terminology, with the aim of facilitating disease recognition and preoperative decision making.
Methods
A retrospective analysis was performed on 88 pathologically confirmed IVL cases. Ultrasound findings were compared with intraoperative observations, frozen section pathology, and postoperative pathology. Three experienced sonographers independently described the imaging features using standardized terms.
Results
Based on ultrasound findings, IVL was classified into four types: Type I (uterine type, 52/88, 59.1%), Type II (parauterine type, 22/88, 25.0%), Type III (combined uterine and parauterine type, 12/88, 13.6%), and Type IV (extrapelvic type, extending to heart/lungs, 2/88, 2.3%). The preoperative diagnostic accuracy was only 14.8% (14/88), with a misdiagnosis rate of 84.1% (74/88). All 52 Type I lesions were misdiagnosed, most commonly as uterine fibroids (47/74). Characteristic ultrasound signs included slit-like anechoic areas within the lesion (especially when internal blood flow was visible) and vermiform or articulated masses along the uterine/parauterine veins.
Conclusion
Ultrasound may play a useful role in the early identification, preoperative typing, and extent assessment of IVL. The proposed four-type classification and characteristic signs provide valuable imaging references for surgical planning and multidisciplinary care. When suspicious signs are detected, extended ultrasound to the iliac veins, inferior vena cava, and heart, along with CT/MRI, may be considered.
Keywords: intravenous leiomyomatosis, ultrasound diagnosis, ultrasound classification, preoperative assessment, misdiagnosis analysis, gynecological tumors
1. Introduction
Intravenous leiomyomatosis (IVL) is a rare, histologically benign smooth muscle tumor that exhibits invasive biological behavior, growing along venous channels. It can extend from the pelvic veins into the inferior vena cava and even reach the right heart (1). The onset of IVL is insidious, and clinical symptoms are non-specific (2). Once cardiovascular symptoms appear, the lesion has usually already involved the heart or pulmonary artery, significantly increasing surgical risk and threatening life (3).
The average age of IVL patients is approximately 45 years, and the disease predominantly affects premenopausal women, suggesting a possible estrogen-dependent mechanism (4, 5). Some studies have indicated that prior uterine surgery (e.g., myomectomy or cesarean section) may be a risk factor, possibly due to altered local vascular architecture that facilitates intravascular invasion (6, 7).
Ultrasound is widely used as a first-line screening modality for gynecological pelvic diseases because it is non-ionizing, repeatable, and allows dynamic observation. However, the sonographic features of IVL are highly variable and have not been systematically classified. Consequently, misdiagnosis and missed diagnosis remain frequent challenges in clinical practice. Most published studies are isolated case reports, and large−series systematic analyses remain relatively scarce.
In this study, we retrospectively analyzed 88 pathologically confirmed IVL cases to develop a practical ultrasound-based classification system using standardized terminology. Our aim is to provide a practical framework that may assist sonographers and clinicians in diagnosis and treatment planning.
2. Materials and methods
2.1. Patients
This study included 88 patients who were diagnosed with IVL by surgical pathology at our hospital from January 2012 to December 2023. Pathological diagnoses were based on the original surgical pathology reports from our institution. All cases were confirmed by experienced pathologists specializing in gynecological pathology at our institution, which is a tertiary referral center for obstetrics and gynecology, using established histopathological criteria for IVL, including the identification of benign smooth muscle tumor cells within vascular spaces. Frozen−section pathology was performed during surgery to guide intraoperative decision−making; however, final diagnoses were based on permanent section histopathology. Inclusion criteria were: ① preoperative gynecological ultrasound, combined abdominopelvic vascular ultrasound, and echocardiography performed at Women’s Hospital, School of Medicine, Zhejiang University; ② complete clinical and imaging data available; ③ diagnosis confirmed as IVL by postoperative histopathology; ④ age ≥ 18 years.
This retrospective study was approved by the Ethics Committee of Women’s Hospital, School of Medicine, Zhejiang University (approval no. IRB 20230322 R). All protocols adhered to the principles of the Declaration of Helsinki.
2.2. Ultrasound examination
All patients underwent thorough transvaginal and/or transabdominal pelvic ultrasound before surgery, performed by experienced sonographers using color Doppler ultrasound devices (GE Voluson E8, Mindray Resona 6s, Sonoscape P6 PRO, etc.). Probe selection depended on lesion size and location. The examination included multi- planar and dynamic scans of the uterus and surrounding structures. Key assessment points were:
Uterine morphology and myometrial echogenicity
Lesion location (intramural, subserosal, parauterine)
Lesion morphology (size, margins, shape: “worm-like,” “beaded,” or “articulated”)
Internal echoes (uniformity, presence of “slit-like” anechoic zones)
Color Doppler flow imaging (CDFI) features: vascularity and pattern (ring-shaped, tree-like, or fissure pattern)
Relationship between the lesion, uterine serosa, and parametrial vessels (especially uterine and ovarian veins)
When major vessel or cardiac involvement was suspected, the examination was extended to include iliac vessels, inferior vena cava, and transthoracic echocardiography. All static and dynamic images of suspicious lesions were documented.
2.3. Image interpretation
Image analysis was performed by three experienced sonographers (each with >10 years of gynecological ultrasound experience) through an independent, blinded, consensus-driven process. First, the team created a standardized list of descriptive terms for IVL ultrasound features (e.g., “slit-like anechoic,” “worm-like,” “sieve-like,” “articulated”). Then, each sonographer independently reviewed the preoperative images of all 88 patients, blinded to the final pathological diagnosis, and recorded the ultrasound features. Any disagreements were resolved by discussion to reach consensus.
2.4. Data analysis
Descriptive statistics were used. Continuous variables (e.g., age) were expressed as mean ± standard deviation (SD), and categorical variables (e.g., clinical symptoms, diagnostic accuracy, misdiagnosis rates) as numbers and percentages. No inferential statistical tests (e.g., t-tests or chi-square tests) were applied, as this was a descriptive retrospective study without a control group.
3. Results
3.1. Patient characteristics
A total of 88 patients were included. Ages ranged from 24 to 58 years (mean 45.4 ± 7.0 years). Among them, 79 had a history of childbirth, and 24 had previously undergone cesarean section. Additionally, 24 patients had a history of myomectomy. Clinical symptoms were non-specific: 48 patients presented primarily with a “pelvic mass,” 25 with excessive menstrual bleeding, and the remainder with abdominal distension, vaginal discharge, or irregular menstruation (Table 1).
Table 1.
Basic information of IVL patients.
| Characteristic | IVL(n=88) |
|---|---|
| Age (years) | 45.4 ± 7.0 |
| Main complaint symptoms | |
| Pelvic mass | 48 (54.5%) |
| Excessive menstruation | 25 (28.4%) |
| Bloating | 1 (1.1%) |
| Vaginal discharge | 1 (1.1%) |
| Irregular menstruation | 2 (2.3%) |
| Frequent urination | 1 (1.1%) |
| Extended menstrual period | 3 (3.4%) |
| Lower abdominal pain | 2 (2.3%) |
| Menstrual cramps | 2 (2.3%) |
| Vaginal neoplasms | 1 (1.1%) |
| Vaginal bleeding | 2 (2.3%) |
| Previous surgical history | |
| History of uterine fibroid surgery | 24 (27.3%) |
| History of cesarean section | 24 (27.3%) |
| The extent of lesion involvement | |
| Limited to the pelvic cavity only | 86 (97.8%) |
| Affecting the heart or (and) lungs | 2 (2.3%) |
| Combined fibroids | 50 (56.8%) |
Values are expressed as the mean ± standard deviation or number (%).
Bold values indicate the clinical characteristics or symptoms presented by the patients; values in parentheses are percentages.
3.2. Ultrasound classification and features
Based on the location of IVL lesions as observed by ultrasound, we classified IVL into four types (Table 2).
Table 2.
Ultrasound typing and diagnostic data.
| Parameter | Value |
|---|---|
| Ultrasound type distribution | |
| Type I (uterine) | 52 (59.1%) |
| Type II (parauterine) | 22 (25.0%) |
| Type III (combined) | 12 (13.6%) |
| Type IV (extrapelvic) | 2 (2.3%) |
| Overall diagnostic performance | |
| Preoperative correct diagnosis of IVL | 14 (14.8%) |
| Preoperative misdiagnosis | 74 (84.1%) |
| Misdiagnosis spectrum (n=74) | |
| Uterine fibroids | 47 (63.5%) |
| Fibroids with degeneration | 13 (17.6%) |
| Uterine sarcoma | 3 (4.1%) |
| Adnexal tumors | 2 (2.7%) |
| Undetermined | 9 (12.2%) |
| Misdiagnosis rate by type | |
| Type I (n=52) | 52 (100%) |
| Type II (n=22) | 17 (77.3%) |
| Type III (n=12) | 7 (58.3%) |
| Type IV (n=2) | 0 (0%)* |
*Type IV: both cases were correctly identified or strongly suspected as IVL preoperatively (see case descriptions above).
Type I: Uterine type (52 cases, 59.1%)(Figure 1). Lesions were confined within the myometrium. Two subtypes were observed:
Figure 1.
(A) The mass exhibits a “multinodular fused morphology”. (B) CDFI: Rich cord-like blood flow signals are seen internally. (C) The mass presents a “map-like appearance”. (D) CDFI: Linear vascular signals are identified inside the lesion. (E) A slit-like anechoic area is seen within the mass (indicated by the yellow arrow). (F) CDFI: No blood flow signal is present within the anechoic area.
Type Ia (Fused nodular type) (Figures 1A–D): Irregular or nearly round mass with clear but irregular borders, heterogeneous internal echoes, “multi-nodule fused” or “map-like” appearance, lacking the normal “whorled” structure of fibroids. CDFI showed mildly abundant, cord-like internal flow, without the typical peripheral ring-shaped flow of fibroids.
Type Ib (Fissure type) (Figures 1E, F): Single or multiple linear or branched “slit-like” anechoic areas within the myometrial lesion – a highly indicative sign. CDFI showed punctate and linear flow signals inside.
Type II: Parauterine type (22 cases, 25.0%) (Figure 2). Lesions were located in the parauterine region, within the broad ligament, or along the parauterine veins. Subtypes:
Figure 2.
(A) The lesion is located near the uterus and presents as “worm-like” high echogenicity. (B) Rich blood flow signals are seen around. (C, D) Contrast-enhanced ultrasound (CEUS): The lesion margin shows hyperperfusion, while the center shows hypoperfusion. (E) The lesion is located near the uterus and presents as “worm-like” hypoechoic. (F) CDFI shows a strip-like blood flow signal around it. (G) The lesion is located near the uterus and presents as an articulated hypoechoic mass. (H) CDFI shows a strip-like blood flow signal around it.
Type IIa (Vermiform type) (Figures 2A–F): Tortuous, cord-like hypoechoic or hyperechoic structures resembling “worm-like” or articulated shapes, often surrounded by tortuous dilated veins. CDFI showed linear venous signals around or within the lesion.
Type IIb (Articulated type) (Figures 2G, H): A series of nodular hypoechoic masses beside the uterus arranged in a string-like pattern. CDFI showed sparse internal flow.
Type III: Combined uterine and parauterine type (12 cases, 13.6%) (Figure 3). Lesions had features of both Type I and Type II, with direct continuity or close association between myometrial and parametrial components.
Figure 3.
(A) The lesion is located in the myometrium of the uterus, with a “slit-like” anechoic area locally (indicated by the yellow arrow). (B) CDFI: Rich blood flow signals are seen locally in the lesion. (C) The lesion is located beside the uterus, presenting as a strip-like hypoechoic area. (D) CDFI: Rich blood flow signals are seen around it. (E, F) The lesion is located in the myometrium with multiple “slit-like” anechoic areas (indicated by the yellow arrow), and rich blood flow signals are seen inside. (G, H) The lesion is located beside the uterus, presenting as a worm-like hypoechoic area, with blood flow signals around it. (I) The lesion is located in the myometrium of the uterus, presenting a nearly circular shape, with multiple irregular anechoic areas visible within (indicated by the yellow arrow). (J) There are a few blood flow signals seen around the periphery. (K) The lesion is located beside the uterus, with multiple irregular anechoic areas (indicated by the yellow arrows). (L) There are a few blood flow signals seen around the periphery.
Type IV: Extrapelvic type (2 cases, 2.3%) (Figures 4–6). Lesions extended beyond the pelvis. Characteristic findings included: (a) solid hypoechoic filling within the inferior vena cava and iliac veins, continuous with the pelvic lesion; (b) slit-like or multi-track anechoic areas within the intravascular mass, with detectable CDFI signals; (c) a hypoechoic mass in the right atrium or right ventricle that moved with the cardiac cycle.
Figure 4.
(A, B) The anterior wall of the uterus is significantly thickened with uneven echoes, resembling a worm-like hyperechoic mass with tortuous curves. (C) A “worm-like” hyperechoic mass with tortuous course (indicated by the yellow arrow) is seen on the left side of the uterine adnexa, with local slit-like anechoic areas inside. (D) CDFI: Slightly rich blood flow signals are seen in the slits. (E) On the right side of the parametrium, a “worm-like” hyperechoic mass with tortuous course was observed (indicated by the yellow arrow), and local slit-like anechoic areas were seen within it. (F) CDFI: Slightly rich blood flow signals were noted within the slits. (G) In the retrohepatic segment of the inferior vena cava, a hypoechoic filling was observed, with multiple slit-like anechoic areas (indicated by the yellow arrow), and the boundary between the tumor and the wall of the inferior vena cava was clear. (H) CDFI: Blood flow signals were seen within the tumor and in the gap between the tumor and the wall. (I) A hypoechoic area is seen filling the iliac vein, with multiple slit-like anechoic areas inside (indicated by the yellow arrow). (J) CDFI: Rich blood flow signals are seen within the tumor. (K) A hypoechoic mass is seen in the right atrium, with a clear boundary from the atrial wall. (L) The tumor in the inferior vena cava extends continuously into the right atrium. (In the image, RA represents the right atrium; IVC represents the inferior vena cava).
Figure 6.
(A) The right parametrial vein is tortuous and dilated. (B) A hypoechoic mass (indicated by the red arrow) is seen in the right ventricle, with an unclear boundary from the ventricular wall and no blood flow signal detected within. (C, D) show nodules of varying sizes in both lungs (indicated by the yellow arrow). Considering the medical history, metastatic leiomyoma is suspected.
Case A (continuous spread type) (Figure 4): A 49−year−old female with a pelvic mass and occasional chest tightness. Transvaginal ultrasound showed marked thickening of the anterior myometrial wall with heterogeneous echogenicity. Worm−like hyperechoic masses were seen bilaterally in the parametrium, continuous with the uterine lesion and coursing along the veins. These masses contained slit-like anechoic areas with rich internal blood flow on CDFI. Subsequent vascular and cardiac ultrasound revealed hypoechoic filling from the common iliac vein through the inferior vena cava into the right atrium. Enhanced CT confirmed filling defects in the inferior vena cava and bilateral ovarian veins. Postoperative pathology confirmed IVL.
Case B (skip metastasis type)( Figures 5, 6): A 39−year−old female with a history of myomectomy and subsequent lung nodules (pathology: leiomyoma). Pelvic ultrasound showed an enlarged uterus with heterogeneous myometrial echogenicity and multiple interconnecting slit-like anechoic areas. Echocardiography revealed a hypoechoic mass (4.0×4.3×2.7 cm) in the right ventricular outflow tract near the pulmonary valve. Based on the history and imaging, the cardiac and lung lesions were considered IVL. Postoperative pathology after hysterectomy and bilateral salpingo−oophorectomy confirmed IVL.
Figure 5.
(A) The echo of the uterine myometrium is uneven, with multiple slit-like anechoic and tortuous areas seen inside, and connections seem to exist among them (indicated by the yellow arrow). (B) Contrast-enhanced ultrasound shows contrast filling inside. (C) An irregular hypoechoic area is seen on the right parametrium with a clear boundary, seemingly closely related to the surrounding vessels, and a few blood flow signals are present inside. (D) Contrast-enhanced ultrasound shows the mass with low perfusion (the lesion is indicated by the yellow arrow).
3.3. Diagnostic accuracy and misdiagnosis
The overall preoperative ultrasound diagnostic accuracy was 14.8% (14/88), and the misdiagnosis rate was 84.1% (74/88). The most common misdiagnosis was uterine fibroids (47/74, 63.5%), followed by “fibroids with degeneration” (13/74, 17.6%), uterine sarcoma (3/74, 4.1%), adnexal tumors (2/74, 2.7%), and undetermined diagnoses (9/74, 12.2%).
Misdiagnosis rates varied by type: Type I 100% (52/52), Type II 77.3% (17/22), Type III 58.3% (7/12), and Type IV 0% (0/2) (Table 2).
4. Discussion
The principal contribution of this study is to provide a practical, ultrasound−based classification tool for the preoperative evaluation of IVL. To our knowledge, this is the first study to systematically categorize IVL according to sonographic lesion location and to describe the corresponding features using standardized terminology. Our classification visually reflects the anatomical extent of the lesion (from uterine−confined to cardiac involvement) and organizes the diverse sonographic manifestations into logical categories, which may help sonographers establish a clear diagnostic approach in clinical practice.
Our data show that the preoperative ultrasound diagnostic rate of IVL is extremely low (14.8%), consistent with the difficulty emphasized by previous studies (2, 4). Misdiagnosis was mainly concentrated in Type I cases (100% misdiagnosed), highlighting the challenge of differentiating early IVL from multiple uterine fibroids and even uterine sarcoma.
The mean age of our IVL patients was 45.4 years, and all were premenopausal, consistent with previously reported demographic characteristics (5, 6), further supporting a possible estrogen−dependent mechanism. In addition, 24 patients had a history of myomectomy and 24 had a history of cesarean section. Some studies have indicated that prior uterine surgery may be a risk factor for IVL (7, 8). Previous fibroid resection may cause local vascular changes that facilitate intravascular invasion and spread along the lumen (9). Therefore, for patients with recurrent fibroids after surgery or with rapidly growing fibroids, if characteristic ultrasound findings are present, IVL should raise suspicion and prompt further evaluation.
4.1. Differentiation from fibroids and sarcoma
Type I IVL is frequently misdiagnosed as fibroids, especially cellular or degenerated fibroids. The following clues may aid identification: a. Morphology: IVL lesions are often irregular, multinodular, and fused, lacking the smooth, round outline and typical whorled structure of fibroids. b. Internal structure: The presence of “slit−like” anechoic areas is a key sign; when CDFI shows blood flow within them, it strongly suggests vascular channels rather than simple cystic change (10, 11). c. Blood flow pattern: IVL typically shows internal cord−like flow, whereas fibroids usually have peripheral ring−shaped flow. In contrast, uterine sarcomas usually grow rapidly, have extremely disordered internal echoes with irregular cystic−solid areas, and exhibit extremely rich, disordered “sea of fire” flow with a possibly low resistance index (RI). Although final identification relies on pathology, ultrasound clues are valuable for surgical planning (11).
4.2. The “slit−like” anechoic area
This study found that the slit−like anechoic area is an important ultrasonographic feature of IVL. In Type I and Type III patients, some uterine lesions exhibited multiple slit−like or irregular anechoic areas. CDFI showed no significant internal blood flow in some, suggesting dilated lymphatic channels or degeneration. However, in the inferior vena cava lesions of Type IV cases, the same slit−like anechoic areas were observed, but venous blood flow signals could be seen filling them. Previous studies have suggested that IVL may induce vascular endothelial proliferation and neovascularization (10). Thus, such anechoic areas may represent intratumoral vascular structures, a view supported by Ge et al. (11). In Type IV Case A, blood flow signals were observed within the slit−like anechoic areas in the parametrial lesions. Of note, in both Type IV cases, the myometrium showed tortuous slit−like anechoic areas, and the parametrial lesions followed venous channels and involved the heart or lungs. These imaging features differ significantly from Types I−III, suggesting that Type IV may represent a distinct subtype, a hypothesis that warrants further investigation.
4.3. Role of contrast−enhanced ultrasound and MRI
In the diagnosis of IVL, CEUS can provide more microvascular perfusion information than color Doppler ultrasound. In Type IIa cases, CEUS of parametrial lesions shows characteristic high perfusion at the margins and low perfusion in the center. This appearance helps differentiate IVL from intravascular tumor thrombus (which often shows heterogeneous enhancement) (12) and bland thrombus (usually no enhancement) (13). For slit−like anechoic areas not clearly characterized by two−dimensional ultrasound, CEUS can provide additional reference information (as shown in Figure 5B). Although MRI is contraindicated in patients with intrauterine metal devices or pacemakers, its main advantage lies in its high soft−tissue resolution (14). Wang et al. (15) suggested using MRI to assess parametrial vessel involvement. Combined use of our ultrasound classification and MRI may improve early diagnostic accuracy.
4.4. Extension pathways and clinical implications
IVL extension within the vascular system mainly follows three pathways (4): ① proximal spread via uterine veins → internal iliac veins → common iliac veins → inferior vena cava; ② extension along ovarian veins → inferior vena cava, possibly reaching the right atrium, right ventricle, and pulmonary artery; ③ skip lesions, with discontinuous distribution within pelvic veins or inferior vena cava, presenting as isolated intracardiac mass adherent to the chamber wall (16) or as metastatic dissemination with multiple pulmonary nodules. High expression of HMGA2 protein in IVL may be associated with its vascular invasive ability (17). Case A in our series followed the first pathway, and its imaging features (cobra−head or flared−tip shape, clear border with the atrial wall, movement with heartbeat) help distinguish it from primary cardiac myxoma. Case B showed the skip pattern with discontinuous spread and pulmonary involvement.
4.5. Recommendations for clinical practice
If a ‘worm−like’ mass is found in the uterine area during pelvic ultrasound, and similar lesions are seen along the parametrial veins or solid masses are found within parametrial veins, IVL should be considered in the differential diagnosis. Therefore, we suggest that extended ultrasound examination to the iliac vessels, inferior vena cava, and heart should be considered when clinically indicated, and chest CT may also be useful (16).Establishing this complete preoperative evaluation protocol may help reduce missed diagnoses.
4.6. Treatment and follow−up
Precise preoperative assessment of IVL is valuable for treatment decisions. Ultrasound can provide useful information on the morphology, size, relationship with the vessel wall, and mobility of intravascular tumors, which may help guide surgical approach selection (16). Echocardiography can provide detailed information on the origin, extent, and adherence to valvular structures of intracardiac tumor thrombus. Because residual recurrence risk persists after surgery, long−term systematic follow−up is recommended (17). Ultrasound, owing to its accessibility and repeatability, remains a practical surveillance modality for follow−up. The mainstay of IVL treatment is complete tumor resection and recurrence prevention, while considering patient age and fertility requirements (18). Treatment options may include surgical resection and hormonal therapy, depending on individual patient circumstances (19). For patients with disease extending to the inferior vena cava or beyond, a multidisciplinary team approach is recommended. For women who have completed childbearing, hysterectomy with bilateral salpingo−oophorectomy is recommended to reduce recurrence risk (16). In patients with fertility preservation requirements, individualized treatment strategies should be discussed in a multidisciplinary setting. In our Type IV cases, residual or metastatic lesions remained stable without significant progression during follow−up (24 and 20 months), highlighting the importance of individualized treatment strategies based on precise preoperative assessment.
4.7. Comparison with existing classification systems
Two established classification systems for IVL have been reported. The traditional anatomical staging (Stages I−IV) is based on the extent of tumor spread from the pelvis to the heart and lungs and is typically determined postoperatively or by CT/MRI. The cardiac−great vessel morphological classification applies only to advanced cases with intracardiac involvement, categorizing tumors based on the relationship between tumor diameter and inferior vena cava caliber. Our ultrasound classification is designed for preoperative evaluation using widely accessible ultrasound. It may help address the early diagnostic challenge (especially Type I) and can inform the need for extended vascular and cardiac imaging. Thus, our classification is intended to complement, rather than replace, existing systems. Of note, the numerical labels (Type I to Type IV) in our ultrasound classification are based solely on sonographic lesion location and do not correspond to the traditional anatomical Stages I to IV.
4.8. Limitations
Several limitations should be acknowledged. This single−center retrospective study included only Chinese women, limiting generalizability. The lack of a non−IVL control group prevented calculation of sensitivity or specificity. A centralized histopathological review of all cases was not performed. The sample size for Type IV was small (n=2), and inter−observer agreement was not assessed. Formal inter−observer agreement analysis was not performed, and the reproducibility of the proposed classification has not yet been established. Therefore, the classification should currently be considered hypothesis−generating rather than validated, and its clinical utility remains to be confirmed in future studies. Long−term follow−up data are also lacking. Future multicenter prospective studies with standardized protocols are needed to validate our classification.
5. Conclusion
Ultrasound may play a useful role in the preoperative evaluation of IVL. The proposed classification provides an initial descriptive framework, but it should be considered hypothesis−generating at this stage. Prospective multicenter studies with formal validation are needed before its clinical utility can be established. When suspicious signs are detected, extended scanning to the iliac vessels, inferior vena cava, and heart may be considered, combined with CT/MRI as clinically appropriate.
Funding Statement
The author(s) declare that financial support was received for the research and/or publication of this article. This work was supported by grants from the National Natural Science Foundation of China (NO. 2272004) and the Natural Science Foundation of Zhejiang Province of China(NO. LZ22H180001).
Footnotes
Edited by: Ayse Ayhan, Johns Hopkins University, United States
Reviewed by: Maruti Haranal, U.N. Mehta Institute of Cardiology and Research, India
Qun Wang, Shanghai AM-SINO Women and Children’s Hospital, China
Data availability statement
The datasets presented in this study can be found in online repositories. The names of the repository/repositories and accession number(s) can be found in the article/supplementary material.
Ethics statement
The studies involving humans were approved by Ethics Committee of Women’s Hospital School of Medicine Zhejiang University. The studies were conducted in accordance with the local legislation and institutional requirements. Written informed consent for participation was not required from the participants or the participants’ legal guardians/next of kin in accordance with the national legislation and institutional requirements. Written informed consent was not obtained from the individual(s) for the publication of any potentially identifiable images or data included in this article because As this study was retrospective, 88 patients who had been discharged from the hospital were contacted by telephone. They gave verbal consent to the matter.
Author contributions
QW: Writing – original draft, Writing – review & editing, Conceptualization, Data curation, Formal analysis, Funding acquisition, Investigation, Methodology, Project administration, Resources, Software, Supervision, Validation, Visualization. YL: Formal analysis, Conceptualization, Data curation, Project administration, Funding acquisition, Investigation, Methodology, Resources, Software, Supervision, Validation, Visualization, Writing – original draft, Writing – review & editing. JZ: Conceptualization, Investigation, Project administration, Supervision, Visualization, Writing – original draft, Writing – review & editing.
Conflict of interest
The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
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References
- 1. Andrade D, Vinck EE, Torres LN, Citarella D. Surgical removal of intravascular leiomyomas with intracardiac extension: A two-case report. J Card Surg. (2020) 35:1094–7. doi: 10.1111/jocs.14525 [DOI] [PubMed] [Google Scholar]
- 2. Clay TD, Dimitriou J, Mcnally OM, Russell PA, Newcomb AE, Wilson AMJ. Intravenous leiomyomatosis with intracardiac extension – a review of diagnosis and management with an illustrative case. (2013) 22:e44–52. doi: 10.1016/j.suronc.2013.03.004 [DOI] [PubMed] [Google Scholar]
- 3. Lim WH, Lamaro VP, Sivagnanam V. Manifestation and management of intravenous leiomyomatosis: A systematic review of the literature. Surg Oncol. (2022) 45:101879. doi: 10.1016/j.suronc.2022.101879 [DOI] [PubMed] [Google Scholar]
- 4. Ma G, Miao Q, Liu X, Zhang C, Sun LJM. Different surgical strategies of patients with intravenous leiomyomatosis. (2016) 95:. doi: 10.1097/md.0000000000004902 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 5. Li B, Chen X, Chu YD, Li RY, Li WD, Ni YM. Intracardiac leiomyomatosis: A comprehensive analysis of 194 cases. Interact Cardiovasc Thorac Surg. (2013) 17:132–8. doi: 10.1093/icvts/ivt117 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 6. Cassol DF, Junior F, Dias do Couto Netto S, Rengel LC, Ragazzo L, Gaiotto FA, et al. Symptomatic uterine leiomyomatosis with intracaval and intracardiac invasion: Video case report. Gynecol Oncol Rep. (2023) 45:101127. doi: 10.1016/j.gore.2022.101127 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 7. Peng J, Zhong F, Zhu Y, Zhang M, Zhang M, Lu C, et al. Clinical analysis of uterine intravenous leiomyomatosis: A retrospective study of 260 cases. J Obstet Gynaecol Res. (2021) 47:4357–64. doi: 10.1111/jog.15013 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8. Stilidi I, Paianidi J, Bokhian V, Andreeva J, Shevchuk A, Ramirez PT. Intracardiac intravenous leiomyomatosis: Diagnosis and management. Int J Gynecol Cancer. (2020) 30:1243–7. doi: 10.1136/ijgc-2020-001614 [DOI] [PubMed] [Google Scholar]
- 9. Dang M, Cai S, Yao J, Zhang B, Zhang L, Xu W, et al. Uterine intravenous leiomyomatosis with internal iliac artery involvement. Int J Womens Health. (2025) 17:3699–706. doi: 10.2147/IJWH.S549134 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 10. Sun L, Zhou F, Benjoed BENJOED, Zhu C, Lyu W, Li Y. Expression characteristics and diagnostic value of angiogenic molecules in intravenous leiomyomatosis. (2022) 102:2005–10. [DOI] [PubMed] [Google Scholar]
- 11. Ge Z, Wang Y, Wang Y, Li W, Yang X, Li J, et al. Diagnostic experience of intravenous leiomyomatosis with emphasis on conventional ultrasonography imaging: A single-center study. Front Oncol. (2023) 13:1203591. doi: 10.3389/fonc.2023.1203591 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 12. Swan RZ, Hanna EM, Sindram D, Iannitti DA, Martinie JB. Adrenocortical carcinoma with intracaval extension to the right atrium: Resection on cardiopulmonary bypass. Ann Surg Oncol. (2012) 19:1275. doi: 10.1245/s10434-011-2203-4 [DOI] [PubMed] [Google Scholar]
- 13. Luo X, Li R, Li Z. Combined transthoracic echocardiography and contrast‐enhanced ultrasonography to trace intravenous leiomyomatosis with intracardiac extension. Echocardiography. (2019) 36:1573–6. doi: 10.1111/echo.14424 [DOI] [PubMed] [Google Scholar]
- 14. Gui T, Qian Q, Cao D, Yang J, Peng P, Shen K. Computerized tomography angiography in preoperative assessment of intravenous leiomyomatosis extending to inferior vena cava and heart. BMC Cancer. (2016) 16:73. doi: 10.1186/s12885-016-2112-9 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 15. Wang Q, Li Y, Gu X, Zhang N, Xie J, Niu B, et al. Imaging diagnosis of intravenous leiomyomatosis: An institutional experience. Clin Radiol. (2023) 78:655–60. doi: 10.1016/j.crad.2023.03.017 [DOI] [PubMed] [Google Scholar]
- 16. Wen YL, Ma GT, Miao Q. Diagnosis and treatment of intravenous leiomyomatosis. Zhonghua Wai Ke Za Zhi. (2023) 61:1051–7. doi: 10.3760/cma.j.cn112139-20230310-00099 [DOI] [PubMed] [Google Scholar]
- 17. Ordulu Z, Nucci MR, Dal Cin P, Hollowell ML, Otis CN, Hornick JL, et al. Intravenous leiomyomatosis: An unusual intermediate between benign and Malignant uterine smooth muscle tumors. Mod Pathol. (2016) 29:500–10. doi: 10.1038/modpathol.2016.36 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 18. Wen Y, Ma G, Miao Q, Shao J, Lu W, Liu X, et al. The largest single-center report on intravenous leiomyomatosis and development of a classification to guide surgical management. J Vasc Surg Venous Lymphat Disord. (2025) 13:101989. doi: 10.1016/j.jvsv.2024.101989 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 19. Aleksandrov A, Lyubenov A, Damyanova P. Intravascular leiomyomatosis with cardiac and pelvic involvement in a postmenopausal woman: A case report of multidisciplinary team management. Case Rep Womens Health. (2023) 40:e00557. doi: 10.1016/j.crwh.2023.e00557 [DOI] [PMC free article] [PubMed] [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Data Availability Statement
The datasets presented in this study can be found in online repositories. The names of the repository/repositories and accession number(s) can be found in the article/supplementary material.