Uterine Intravenous Leiomyomatosis Research Articles

Update on clinical characteristics and molecular insights for uterine intravenous leiomyomatosis (Review).

Intravenous leiomyomatosis (IVL) is a rare benign disease, which typically develops along vascular vessels and extends to the inferior vena cava and right atrium of the heart. In the early stages of the disease, the clinical manifestations and the results of imaging examinations are not uniform among patients. Thus, a high rate of misdiagnosis and missed diagnosis is common. When the tumor extends along the venous system to the pelvic floor vein or through the inferior vena cava involving the right atrium of the heart or the pulmonary artery, severe symptoms occur, such as ascites, dyspnea, heart failure and even sudden mortality. Improving the understanding of IVL to identify and evaluate this disease in its early stages is important. Complete tumor resection remains the primary treatment option for IVL. The recurrence rate of the disease varies depending on multiple factors, such as type of surgical procedure performed. Therefore, long-term follow-up is necessary for patients with IVL. The review of recent findings on the molecular and clinicopathological characterization of IVL is important to understand the pathogenesis of IVL. In the present study, the clinical manifestations, pathogenesis, differential diagnosis, treatment and prognosis of IVL are summarized in order to provide a single source of insightful information on IVL.

A contrast-enhanced CT-based radiomic nomogram for the differential diagnosis of intravenous leiomyomatosis and uterine leiomyoma.

Uterine intravenous leiomyomatosis (IVL) is a rare and unique leiomyoma that is difficult to surgery due to its ability to extend into intra- and extra-uterine vasculature. And it is difficult to differentiate from uterine leiomyoma (LM) by conventional CT scanning, which results in a large number of missed diagnoses. This study aimed to evaluate the utility of a contrast-enhanced CT-based radiomic nomogram for preoperative differentiation of IVL and LM. 124 patients (37 IVL and 87 LM) were retrospectively enrolled in the study. Radiomic features were extracted from contrast-enhanced CT before surgery. Clinical, radiomic, and combined models were developed using LightGBM (Light Gradient Boosting Machine) algorithm to differentiate IVL and LM. The clinical and radiomic signatures were integrated into a nomogram. The diagnostic performance of the models was evaluated using the area under the curve (AUC) and decision curve analysis (DCA). Clinical factors, such as symptoms, menopausal status, age, and selected imaging features, were found to have significant correlations with the differential diagnosis of IVL and LM. A total of 108 radiomic features were extracted from contrast-enhanced CT images and selected for analysis. 29 radiomics features were selected to establish the Rad-score. A clinical model was developed to discriminate IVL and LM (AUC=0.826). Radiomic models were used to effectively differentiate IVL and LM (AUC=0.980). This radiological nomogram combined the Rad-score with independent clinical factors showed better differentiation efficiency than the clinical model (AUC=0.985, p=0.046). This study provides evidence for the utility of a radiomic nomogram integrating clinical and radiomic signatures for differentiating IVL and LM with improved diagnostic accuracy. The nomogram may be useful in clinical decision-making and provide recommendations for clinical treatment.

Intravenous leiomyomatosis of the uterus: Preoperative and intraoperative assessment.

To assess factors influencing preoperative diagnosis and hemorrhage during surgery with uterine intravenous leiomyomatosis. This retrospective single-institution study used univariate analysis and multivariate models to investigate potential factors contributing to preoperative diagnosis and hemorrhage during surgery associated with intravenous leiomyomatosis in 135 patients from January 2012 to April 2022. Risk factors for disease recurrence were also investigated. The SPSS statistical analysis package was used for data analysis. Previous myomectomy or fibroid ablation and tumor location on color Doppler were related to preoperative diagnosis (P = 0.031 and P = 0.003, respectively). Multivariate regression analysis demonstrated that lesions extending to the broad ligament were the only factors affecting preoperative diagnosis (odds ratio [OR] 5.383, 95% confidence interval [CI] 1.49-19.47). Univariate analysis showed that previous myomectomy or fibroid ablation (P = 0.017), tumor location (P = 0.027), and parauterine involvement (P = 0.014) were associated with intraoperative hemorrhage. Parauterine involvement was an independent risk factor for increased bleeding (OR 1.36, 95% CI 1.14-3.92). Six patients (4.4%) relapsed. The present study demonstrated that age (P = 0.031) and surgical type (P < 0.001) might be associated with disease recurrence. Treatment emphasis should focus on lesions extending to the broad ligament. Intraoperative bleeding associated with parauterine involvement should be stopped as effectively as possible.

Clinical analysis of uterine intravenous leiomyomatosis: A retrospective study of 260 cases.

MethodsWe collected the clinical data of 260 patients admitted to the hospital from April 2003 to September 2019 with pathologically confirmed intravenous leiomyomatosis (IVL) and followed up with these patients regularly. Univariate and multivariate logistic regression analyses were carried out on the relevant recurrence factors.ResultsA total of 166 patients were regularly followed up, the median follow‐up time was 36 (range 2–168) months, 14 (5.4%) patients eventually relapsed, and the median recurrence time was 8.5 (range 2–42) months. The univariate analysis showed that age (p = 0.003) and surgical type (p < 0.001) were associated with recurrence, and multivariate regression analysis demonstrated that surgical type was the only factor associated with recurrence (p < 0.001, OR 20.01).ConclusionsThe use of gonadotrophin releasing hormone agonist (GnRHa) cannot reduce the postsurgical recurrence rate of patients with UIVL. Compared to total hysterectomy and bilateral salpingo‐oophorectomy (TH‐BSO), total hysterectomy (TH) does not increase the odds of recurrence, but the chance of recurrence with tumorectomy (TE) is 20 times higher than that of TH‐BSO.

Intravenous Leiomyomatosis of the Uterus: A Retrospective Single-Center Study in 14 Cases.

Purpose This study aimed to retrospectively review the diagnosis and surgical treatment of uterine intravenous leiomyomatosis (IVL). Methods The clinical data of 14 patients with uterine IVL admitted to our hospital between 2013 and 2018 were retrospectively analyzed, including their demographics, imaging results, surgical procedures, perioperative complications, and follow-up results. Results The tumors were confined to the pelvic cavity in 7 patients, 1 into the inferior vena cava, 4 into the right atrium, and 2 into the pulmonary artery (including 1 into the superior vena cava). Only one case was misdiagnosed as right atrial myxoma before the operation, which was found during the surgery and was treated by staging surgery; all the other patients underwent one-stage surgical resection. Three patients underwent complete resection of the right atrial tumor through the abdominal incision, and one patient died of heart failure in the process of resection of heart tumor without abdominal surgery. During the 6–60 months of follow-up, 4 patients developed deep venous thrombosis of the lower extremity, and 1 patient developed ovarian vein thrombosis and pulmonary embolism. After anticoagulation treatment, the symptoms disappeared. One patient refused hysterectomy and the uterine fibroids recurred 4 years after the operation. Conclusion Specific surgical plans for uterine IVL can be formulated according to cardiac ultrasound and computed tomography (CT). For the first type of tumor involving the right atrium, the right atrium tumor can be completely removed through the abdominal incision alone to avoid thoracotomy. The disease is at high risk of thrombosis and perioperative routine anticoagulation is required.

Uterine intravenous leiomyomatosis with an isolated large metastasis to the right atrium: a case report

BackgroundAn intravenous leiomyomatosis is a special type of uterine leiomyoma characterized by the formation of benign leiomyomatous tissue within the vascular wall. Although histologically benign, intracardiac metastasis can lead to circulatory failure, and death, if untreated. Herein, we report on a case of a uterine intravenous leiomyomatosis with an isolated large adherent metastasis in the right atrium of the heart.Case PresentationA 52-year-old Japanese woman sought medical attention at our hospital for lower abdominal pain. A 27-cm uterine mass was detected on clinical imaging, with a 78 × 47-mm mass in the right atrium detected on preoperative echocardiography. Intracardiac mass resection and tricuspid annuloplasty were performed as the first-stage surgery. The pedicle of the tumor was adherent to the wall of the atrium. On histological examination, the tumor was found to consist of spindle-shaped cells with eosinophilic cytoplasm, without atypia, but with a myxoid change, and rich microvascularization of the pedicle. Total abdominal hysterectomy was performed as the second-stage surgery, with confirmation of the diagnosis as uterine intravenous leiomyomatosis with an isolated metastasic lesion to the right atrium. There has been no evidence of tumor recurrence in the 15 months since surgery.ConclusionWe report a unique case in which a large right atrial leiomyoma was identified following a uterine intravenous leiomyomatosis. Our case exemplifies that intravenous leiomyomatosis metastatic tumors have the potential to grow via their vascularization.

Intravenous leiomyomatosis: molecular analysis of 17 cases

Intravenous leiomyomatosis (IVL) is a rare smooth muscle tumour with a benign histology but with a quasi-malignant intravascular growth. In this study, we investigated the molecular alterations in 17 IVL cases composed of concurrent uterine leiomyoma (n=12), uterine IVL (n=17) and extra-uterine IVL (n=12). We found that eight tumours had a somatic MED12 mutation (c.130G>A, p.G44S, n=7; c.131G>C, p.G44A, n=1). The frequency of MED12 mutations was significantly higher in concurrent uterine leiomyoma (6/12, 50%) than in uterine (0/17, 0%) and extra-uterine IVL (2/12, 16.7%). The frequency of HMGA2 over-expression or MED12 low-expression was not significantly different among uterine leiomyoma, IVL and extra-uterine IVL (p>0.05). Short tandem repeat (STR) analysis indicated that one uterine and two extra-uterine IVL tumours from three patients were microsatellite instability positive (MSI+) whereas loss of heterozygosity (LOH) was found in one uterine leiomyoma, three uterine and three extra-uterine IVL tumours from five patients. LOH was more frequently seen in uterine/extra-uterine IVL tumours (6/20, 30%) than in the concurrent leiomyomas (1/7, 14.3%) (p<0.05). MED12 mutation, MSI and LOH were discordant between uterine and extra-uterine IVL in all patients. These findings suggest that IVL harbours distinct molecular pathogenesis from common uterine leiomyomas. Uterine IVL and extra-uterine tumours may represent an independent origin rather than uniclonal dissemination from a single tumour. Further investigations are warranted to explore the underlying key molecular events in the pathogenesis of IVL.

39. Uterine intravenous leiomyomatosis with intracardiac extension: Report of two cases

Background: Intravenous leiomyomatosis (IVL) is a histomorphologically benign smooth muscle neoplasm characterised by spindle-cell proliferation primarily located within the uterine and pelvic venous system, inferior vena cava, and in rare instances, extending to the heart.

Intravenous leiomyomatosis of the uterus: A clinicopathologic analysis of 13 cases with an emphasis on histogenesis

The object of this study is to investigate the prognosis and potential histologic origin of uterine intravenous leiomyomatosis (IVL). We retrospectively analyzed the clinicopathologic and immunohistochemical features of 13 cases of IVL from a single institute. The patients underwent hysterectomy (3 with bilateral salpingo-oophorectomy, and 8 with excision of the broad ligaments and/or parametrium). They survived with no evidence of disease for 6–90 months. Intravascular worm-like plugs were macroscopically identified in the myometrium in 10 of 13 cases (76.9%). Six patients (46.1%) had extra-uterine involvement. Histopathologically, the intravascular tumor foci were covered with endothelium. We found the presence of congested vessels in the tumor and the surrounding myometrium in 9 of 13 cases (69.2%). The concurrent entities included seven uterine leiomyomas, one leiomyoma with adenomyosis, one adenomyosis, and one pulmonary benign metastasizing leiomyoma. Estrogen receptor and desmin were positive in IVL, and negative in the vascular wall. We conclude that IVL confined to the uterus may have a favorable prognosis, and we believe IVL is likely to originate from contiguous leiomyoma or myometrium.

Uterine Intravenous Leiomyomatosis with Cardiac Extension: Radiologic Assessment with Surgical and Pathologic Correlation.

We present the computed tomography (CT) and magnetic resonance (MR) imaging findings of a 71-year-old woman with a cardiac extension of intravenous leiomyoma (IVL) that arose from the uterus, extended to the inferior vena cava (IVC), and reached the right ventricle through the right ovarian vein. Radiologic-pathologic correlation showed that the intravascular cord-like mass originating from the IVC and extending to the right ventricle was composed of degenerated smooth muscle cells with a number of large vessels that were regarded as arteries; moreover, the arteries within the cord-like mass appeared to be looping internally. Given the disappearance of the right ovarian venous wall around the IVL pathologically, extracting the tumor from the ovarian vein during an operation is considered to be impossible retrospectively. Also it was difficult to identify even the intravenous extension of the uterine leiomyoma histopathologically. Therefore, contrast-enhanced CT, in particular arterial phase imaging, provided important information that revealed the mass, range, and path of the lesion, ensuring that an appropriate operative plan could be drawn up and the tumor completely excised.

Single-stage operation using hypothermic circulatory arrest to remove uterine intravenous leiomyomatosis extended to the vena cava and right atrium

Single-stage operation using hypothermic circulatory arrest to remove uterine intravenous leiomyomatosis extended to the vena cava and right atrium

“Uterus in My Lung?!” A Case of Pulmonary Leiomyomatosis as a Presentation of Uterine Intravenous Leiomyomatosis

“Uterus in My Lung?!” A Case of Pulmonary Leiomyomatosis as a Presentation of Uterine Intravenous Leiomyomatosis

Recurrent chromosomal aberrations in intravenous leiomyomatosis of the uterus: high-resolution array comparative genomic hybridization study.

Uterine intravenous leiomyomatosis (IVL) is a distinct smooth muscle neoplasm with a potential of clinical aggressiveness due to its ability to extend into intrauterine and extrauterine vasculature. In this study, chromosomal alterations analyzed by oligonucleotide array comparative genomic hybridization were performed in 9 cases of IVL. The analysis was informative in all cases with multiple copy number losses and/or gains observed in each tumor. The most frequent recurrent loss of 22q12.3-q13.1 was observed in 6 tumors (66.7%), followed by losses of 22q11.23-q13.31, 1p36.13-p33, 2p25.3-p23.3, and 2q24.2-q32.2 and gains of 6p22.2, 2q37.3 and 10q22.2-q22.3, in decreasing order of frequency. Copy number variants were identified at 14q11.2, 15q11.1-q11.2, and 15q26.2. Genes mapping to the regions of loss include CHEK2, EWS, NF2, PDGFB, and MAP3K7IP1 on chromosome 22q, HEI10 on chromosome 14q, and succinate dehydrogenase subunit B, E2F2, ARID1A KPNA6, EIF3S2 , PTCH2, and PIK3R3 on chromosome 1p. Regional losses on chromosomes 22q and 1p and gains on chromosomes 12q showed overlaps with those previously observed in uterine leiomyosarcomas. In addition, presence of multiple chromosomal aberrations implies a higher level of genetic instability. Follow-up polymerase chain reaction (PCR) sequencing analysis of MED12 gene revealed absence of G> A transition at nucleotides c.130 or c.131 in all 9 cases, a frequent mutation found in uterine leiomyoma and its variants. In conclusion, this is the first report of high-resolution, genome-wide investigation of IVL by oligonucleotide array comparative genomic hybridization. The presence of high frequencies of recurrent regional loss involving several chromosomes is an important finding and likely related to the pathogenesis of the disease.

Uterine intravenous leiomyomatosis with cardiac extension: Imaging characteristics and literature review

Intravenous leiomyomatosis (IVL), showing unusual growth patterns of uterine leiomyoma, is a rare neoplasm characterized by intravascular proliferation of a histologically benign-looking smooth muscle cell tumor mass, but not invading the tissue. To date, less than 300 cases have been reported and fewer than 100 cases with cardiac involvement. Imaging characteristics of IVL are still not clear so it is usually misdiagnosed before surgery. A 36-year-old woman, who had undergone hysterectomy due to hysteromyoma, presented with shortness of breath after activities. Imaging showed IVL with mass involvement of the left ovarian vein, left renal vein, left external and common iliac vein, as well as within the inferior vena cava (IVC), extending into the right atrium. The operation demonstrated that the mass had no stalk and had well-demarcated borders with the wall of the right atrium and IVC. The patient underwent a one-stage combined multidisciplinary thoraco-abdominal operation under general anesthetic. Subsequently, the pathology report confirmed IVL. IVL should be considered in a female patient presenting with an extensive mass in the right side of the heart. Imaging technology, such as echocardiogram, contrast-enhanced computed tomography and magnetic resonance imaging, can provide important information to reveal the mass, the range and path of the lesion, and relates to the surgical plan decision. Consequently, perfect and exact image examination is very necessary pre-operation.

Léiomyomatose intravasculaire utérine avec atteinte cardiaque : à propos d’un cas

Uterine intravenous leiomyomatosis is a rare benign tumor, whose diagnosis of certainty is histological. It is characterized by its potential of intravascular extension with possibility of cardiac localisation imposing a complete surgical removal and a suitable imagery postoperatively. We report the case of a 63-year-old patient, presenting a cardiac extension complicating a LIV, by which we will discuss the diagnosis aspects, prognosis and therapeutic of this pathology.

Pulmonary Benign Metastasizing Leiomyoma Associated With Intravenous Leiomyomatosis of the Uterus: Clinical Behavior and Genomic Changes Supporting A Transportation Theory

Benign metastasizing leiomyoma is a rare lesion characterized by benign-appearing smooth muscle tumor most frequently involving the lung and usually associated with a benign leiomyoma or intravenous leiomyomatosis of the uterus. The pathogenetic mechanism of the tumor has not been clarified, but the possibilities including hormone-sensitive in situ proliferations of smooth muscle bundles, mechanical displacement or intravascular spread of preexisting benign uterine tumor tissue, and metastasized very low-grade uterine leiomyosarcoma have been proposed. We described a case of pulmonary benign metastasizing leiomyoma associated with a uterine intravenous leiomyomatosis in a 46-year-old woman with a result of comparative genomic hybridization study. The 2 lesions showed significantly overlapping, if not identical, complex genomic changes in the comparative genomic hybridization, suggesting that the 2 lesions are closely related to each other. Unresected pulmonary nodules were left untreated for 13 months after the hysterectomy and wedge biopsy of 3 pulmonary nodules to show no further growth, suggesting clinical behavior of nonmalignant tumor in our case. Benign metastasizing leiomyomas may comprise a heterogeneous group of tumors in terms of their malignant potential and pathogenetic mechanism.

One-Stage Surgery of Low-Grade Malignant Intravenous Uterine Leiomyosarcomatosis With Right Atrium Extension

Uterine leiomyosarcoma is a rare and aggressive malignant tumor arising from the smooth muscle cells of the myometrium, which rarely metastasizes to the heart. We report the case of a 49-year-old Chinese woman who underwent a successful one-stage surgical intervention after an initial diagnosis of intravenous uterine leiomyomatosis. However, her postoperative biopsy showed the presence of low-grade malignant uterine leiomyosarcoma with intravenous and right atrium extension. It is our conclusion that surgical resection is the mainstay treatment for this type of condition.

Léiomyomatose intravasculaire utérine. À propos d'un cas

Uterine intravenous leiomyomatosis is a rare benign tumor. We report the case of a 41-year-old woman, with no history, who presented an abdominopelvic mass arising from the uterus. Histological examination revealed uterine intravenous leiomyomatosis. The specificity of this tumor is hormonodependency and potential vascular extension.

Recurrence of Uterine Intravenous Leiomyomatosis with Intracardiac Extension: Diagnostic Considerations and Surgical Removal

A 28-year-old woman (gravida 2, para 2) was admitted 20 months after a hysterectomy because of fibromyoma. The hysterectomy specimen had shown intravenous leiomyomatosis. The patient presented with unspecific abdominal symptoms, serologic signs of hepatic and renal failure and clinical right-sided heart failure. Progression despite treatment with a gonadotropin-releasing hormone analogue promoted transferral to the present centre. Abdominal ultrasonography, phlebography and transoesophageal echocardiography showed a left pelvic mass and a seemingly free-floating tumour extending from the left main iliac vein via the inferior caval vein to the right ventricle. During a combined cardiac and distal caval approach using extracorporeal circulation, a 45 cm massive leiomyoma was removed successfully. Seven weeks later the left pelvic tumour was removed radically together with left oophorectomy. At control 12 months later the patient was well and without any remaining symptoms.