Surgical strategy for intravenous leiomyomatosis spreading from uterine to the right atrium presenting with recurrent syncope.

Uterine leiomyoma invading internal iliac vein and consequently disseminating into the right atrium is an extremely rare condition, and surgical strategy is controversial. Here, we reported a specific case with successful surgical resection through one-stage total hysterectomy, bilateral oophorectomy, and the intracardiovascular lesion. This procedure would be an optimal choice for uterine leiomyoma invading inferior vena cava and spreading to right atrium.

Safety and efficacy of sirolimus in recurrent intravenous leiomyomatosis, pulmonary benign metastatic leiomyomatosis, and leiomyomatosis peritonealis disseminata: a pilot study.

BACKGROUND: Intravenous leiomyomatosis (IVL), pulmonary benign metastatic leiomyomatosis (PBML), and leiomyomatosis peritonealis disseminata (LPD) are leiomyomas with special growth patterns and high postoperative recurrence rates. We report the safety and efficacy of a pilot study of sirolimus in the treatment of recurrent IVL, PBML, and recurrent LPD. METHODS: This was a pilot study to evaluate the safety and efficacy of sirolimus in the treatment of leiomyomatosis (ClinicalTrials.gov identifier NCT03500367) conducted in China. Patients received oral sirolimus 2 mg once a day for a maximum of 60 months or until disease progression, intolerable toxicity, withdrawal of consent, or investigator decision to stop. The primary end point of this study was the objective response rate. Secondary end points included safety and tolerability, disease control rate, and progression-free survival. RESULTS: A total of 15 patients with leiomyomatosis were included in the study, including five with recurrent IVL, eight with PBML and two with recurrent LPD. The median follow-up time was 15 months (range 6-54 months), nine patients (60%) had treatment-related adverse events (including all levels), and two patients had treatment-related grade 3 or 4 adverse events. The objective response rate was 20.0% (95% CI, 7.1-45.2%), and the disease control rate was 86.7% (95% CI, 62.1-96.3%). Partial response was achieved in three patients. The median response time in the three partial response patients was 33 months (range 29-36 months), and the sustained remission time of these three patients reached 0, 18, and 25 months, respectively. CONCLUSIONS: Sirolimus was safe and effective in the treatment of recurrent IVL, PBML, and recurrent LPD. TRIAL REGISTRATION: ClinicalTrials.gov identifier NCT03500367. Registered on 18 April 2018.

MR imaging findings of stage I intravenous leiomyomatosis: a retrospective single-center study in 19 cases.

OBJECTIVES: The aim was to explore the magnetic resonance imaging (MRI) features of stage-I intravenous leiomyomatosis (IVL). MATERIALS AND METHODS: From January 2019 to January 2023, clinical, pathological, and MRI data were collected from 19 cases confirmed by surgical pathology. Two radiologists retrospectively measured the tumor sizes, T1WIs, T2WIs, and ADC values and evaluated contrast-enhanced T1WIs, DWIs, complications and parauterine infiltrations. The number of tumor cells and the total nuclear area were measured. The percentage of tumor cell area out of the total area was used as the tumor cell density. RESULTS: Nineteen patients with stage-I IVL aged 33 to 66 years (mean age: 46 ± 7.6 years) were included in this study. All 19 cases were located in the myometrium or parametrium, with a mean diameter of 11.2 ± 4.8 cm. Among these cases, 14 (73.6%) were associated with leiomyoma, and six (31.6%) involved the broad ligament. Isointensity was observed in the T1WIs of 12 cases (63.2%), while slight hypointensity was seen in five patients (26.3%). Isointensity was observed in the on T2WIs of four cases (21.1%), and iso- or slight hyperintensity was observed in 15 cases (78.9%). A significant difference was detected between the normalized T2WIs of IVL and myometrium (p < 0.001). A Pearson correlation test showed demonstrated a negative correlation between the ADC and tumor cell density values (r = - 0.946, p < 0.001). Tortuous vessels were present in 17 cases (89.5%) within or next to the lesions, and multiple winding cord-like filling defects were seen in 11 cases (57.9%) within the tortuous vessels on the T2WIs. CONCLUSION: Identifying the characteristic MRI features of stage-I IVL helped improve the diagnostic accuracy achieves for this rare tumor. Stage-I IVL often presents as a large mass accompanied by leiomyoma, and it easily invades the broad ligament. TIWI signals exhibited isointensity, and T2WI signals contained iso- or slight hyperintensity. Tortuous vessels were present within or next to the lesions, and multiple winding cord-like filling defects were observed within the tortuous vessels on the T2WIs.

Unraveling the challenges of intravenous leiomyomatosis: a retrospective analysis of 11 cases.

OBJECTIVE: This study provides a concise overview of diagnostic and treatment strategies for intravenous leiomyomatosis (IVL), a rare disease with nonspecific clinical manifestations, based on cases from a tertiary referral hospital in China. METHODS: We retrospectively analyzed 11 premenopausal patients with confirmed IVL between 2018 and 2022. Clinical data from Ultrasound, Enhanced CT, and MRI were studied, along with surgical details, postoperative pathology, and follow-up information. RESULTS: Premenopausal patients showed no disease-specific symptoms, with 90.9% having a history of gynecological or obstetric surgery, and 72.7% having prior uterine fibroids. Cardiac involvement was evident in two cases, with echocardiography detecting abnormal floating masses from the inferior vena cava. Pelvic ultrasound indicated leiomyoma in 90.9% of cases, with ≥ 50 mm size. Surgery was the primary treatment, and lesions above the internal iliac vein resulted in significantly higher intraoperative blood loss (median 1300 ml vs. 50 ml, p = 0.005) and longer hospital stays (median 10 days vs. 4 days, p = 0.026). Three patients with lesions above the inferior vena cava required combined surgery with cardiac specialists. Recurrence occurred in 2 out of 11 patients with incomplete lesion resection. CONCLUSIONS: IVL mainly affects premenopausal women with uterine masses, primarily in the pelvic cavity (Stage I). Pelvic ultrasound aids early screening, while Enhanced CT or MR assists in diagnosing and assessing venous lesions. Complete resection is crucial to prevent recurrence. Lesions invading the internal iliac vein and above pose higher risks during surgery. A multidisciplinary team approach is essential for patients with lesions above the inferior vena cava, with simultaneous surgery as a potential treatment option.

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.

Identification of novel serum protein biomarkers in the context of 3P medicine for intravenous leiomyomatosis: a data-independent acquisition mass spectrometry-based proteomics study.

Background: Intravenous leiomyomatosis (IVL) is a rare endocrine-associated tumor with unique characteristics of intravascular invasion. This study aimed to identify reliable biomarkers to supervise the development or recurrence of IVL in the context of predictive, preventive, and personalized medicine (PPPM/3PM). Methods: A total of 60 cases were recruited to detect differentially expressed proteins (DEPs) in serum samples from IVL patients. These cases included those with recurrent IVL, non-recurrent IVL, uterine myoma, and healthy individuals without uterine myoma, with 15 cases in each category. Then, weighted gene co-expression network analysis (WGCNA), lasso-penalized Cox regression analysis (Lasso), trend clustering, and a generalized linear regression model (GLM) were utilized to screen the hub proteins involved in IVL progression. Results: First, 93 differentially expressed proteins (DEPs) were determined from 2582 recognizable proteins, with 54 proteins augmented in the IVL group, and the remaining proteins declined. These proteins were enriched in the modulation of the immune environment, mainly by activating the function of B cells. After the integrated analyses mentioned above, a model based on four proteins (A0A5C2FUE5, A0A5C2GPQ1, A0A5C2GNC7, and A0A5C2GBR3) was developed to efficiently determine the potential of IVL lesions to progress. Among these featured proteins, our results demonstrated that the risk factor A0A5C2FUE5 was associated with IVL progression (OR = 2.64). Conversely, A0A5C2GPQ1, A0A5C2GNC7, and A0A5C2GBR3 might act in a protective manner and prevent disease development (OR = 0.32, 0.60, 0.53, respectively), which was further supported by the multi-class receiver operator characteristic curve analysis. Conclusion: Four hub proteins were eventually identified based on the integrated bioinformatics analyses. This study potentiates the promising application of these novel biomarkers to predict the prognosis or progression of IVL by a 3PM approach. Supplementary Information: The online version contains supplementary material available at 10.1007/s13167-023-00338-0.

Surgical management of intravenous leiomyomatosis with intracardiac extension.

Intravenous leiomyomatosis is a rare smooth muscle tumor that is associated with uterine leiomyomas. Intravenous leiomyomatosis often presents with nonspecific abdominal and cardiac symptoms, making the diagnosis difficult. We present a comprehensive review of a case of a 52-year-old woman with intravenous leiomyomatosis with intracardiac extension, who was successfully treated with complete surgical resection.

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

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.

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

Objective: 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. Methods: 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). Results: 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). Conclusion: 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.

A case of a recurrent low-grade endometrial stromal sarcoma extending to the inferior vena cava (IVC) after the primary fertility-sparing surgery.

INTRODUCTION AND IMPORTANCE: A case of Low-grade endometrial stromal sarcoma (LG-ESS) invading the great vessels is rare. CASE PRESENTATION: A 34-year-old female who had no past history presented to a previous hospital with abdominal distension. Magnetic resonance imaging revealed a 15 cm pelvic mass beside the uterus, and only the pelvic mass was removed at the surgery. The tumor was judged to be a LG-ESS. The patient chose to be observed to preserve her fertility, and no adjuvant treatment was undertaken. Two years later, she was referred to our hospital due to recurrence of the pelvic mass. Enhanced computed tomography revealed a large tumor in the vena cava which extended from the left internal iliac vein and which originated from the pelvic tumor. An operation was performed by a multidisciplinary team. Complete resection of the tumor was achieved with a radical hysterectomy, bilateral salpingo-oophorectomy, removal of recurrent pelvic masses and the intravascular tumor. We diagnosed a recurrence of LG-ESS. She received a postoperative adjuvant therapy of LG-ESS. CLINICAL DISCUSSION: Patients with fertility-sparing treatment had higher recurrence rates. In cases of tumor intravenous extension, we should make every effort to extract the tumor to avoid sudden death. CONCLUSION: This case highlights the importance of a multidisciplinary approach in treating this rare tumor with intravascular extension. In particular, patients with LG-ESS who receive fertility-sparing surgery should undertake postoperative chemotherapy or radiotherapy in order to reduce the risk of recurrence, as was in this case.

Tumor in Transit: Intracardiac Leiomyomatosis.

Intravenous leiomyoma is a rare condition that occurs when there is a vascular invasion of a pre-existing uterine leiomyoma. The tumor can metastasize to structures such as the heart and lungs. We discuss a case of metastasis to the heart resulting in severe tricuspid regurgitation. Surgical intervention is the primary modality; usually a staged approach involving cardiac surgery along with abdominal and/or pelvic surgery. We want to highlight the importance of fully investigating right-sided cardiac masses. While there are common etiologies for these masses, one must maintain a high degree of suspicion for an intravenous leiomyoma, especially if a female has certain risk factors such as a prior history of fibroids or a hysterectomy. We also stress the importance of a multi-disciplinary team approach when providing care to these patients, along with reviewing all modalities of imaging.

Case report: Cardiac metastatic uterine intravenous leiomyomatosis excision with extracorporeal venous shunt under the guidance of 3-dimensional printing.

Intravenous leiomyomatosis (IVL) is relatively rare, and the incidence of cardiac IVL is even lower. The case report introduces a 48-year-old woman with two episodes of syncope in 2021. Echocardiography showed a cord-like mass in the inferior vena cava (IVC), right atrium (RA), right ventricle (RV) and pulmonary artery. Computed tomography venography and magnetic resonance imaging showed strips in RA, RV, IVC, right common iliac vein, and internal iliac vein, as well as a round-like mass in the right uterine adnexa. Combined with the patient's prior surgical history and rare anatomical structures, surgeons used cardiovascular 3-dimensional (3D) printing technology to create patient-specific preoperative 3D printed model. The model could help surgeons to visually and accurately understand the size of IVL and its relationship to adjacent tissues. Finally, surgeons successfully performed a concurrent transabdominal resection of cardiac metastatic IVL and adnexal hysterectomy with off-cardiopulmonary bypass. Preoperative evaluation and guidance of 3D printing may play a critical role to ensure this surgery for the patient with rare anatomical structures and high surgical risk. Clinical Trial Registration: [ClinicalTrials.gov], Protocol Registration System [NCT02917980].

Successful one-stage resection of intracardiac intravenous leiomyomatosis: A case report.

This case report is about a 47-year-old patient, who was diagnosed with intracardiac intravenous leiomyomatosis and received treatment at our institution. Intravenous leiomyomatosis is a rare, histologically benign, uterine neoplasm, which is characterized by non-invasive intravascular proliferation of smooth muscle cells. Intravenous leiomyomatosis arises from the myometrium and, in its most extensive form, can reach the heart via the pelvic veins and the inferior vena cava, causing hemodynamic complications. Treatment of choice is the complete resection of the tumor, even though there is no consensus on the optimal surgical approach. In this case, complete resection of the tumor was accomplished in a one-stage procedure. The patient recovered well and CT scan did not show any signs of recurrence after five months.

Diagnostic experience of intravenous leiomyomatosis with emphasis on conventional ultrasonography imaging: a single-center study.

Objective: Intravenous leiomyomatosis (IVL) is a rare and aggressive tumor type that has the potential to extend into the inferior vena cava (IVC) and is susceptible to be misdiagnosed and neglected. Despite its clinical significance, there is a paucity of research that has focused on the specific manifestations of IVL on ultrasonography. Therefore, this study aims to systematically analyze the specific ultrasound features of IVL and augment its diagnostic accuracy. Materials and method: Prospective inclusion was granted to patients admitted to our hospital between December 2016 and March 2021 for an IVC-occupying lesion. Multi-modal ultrasonography, encompassing gray-scale and color Doppler, was conducted. Lesions were categorized as IVL or non-IVL based on pathological or follow-up data. Two ultrasound sonographers with over 5 years of experience read and recorded ultrasound data for all lesions, which were subsequently comparatively analyzed to identify specific signs of IVL. Results: A total of 284 patients diagnosed with IVC-occupying lesions were included in the study. The lesion types comprised of IVL (n=67, 23.6%), IVC thrombus (n=135, 47.5%), tumor thrombus of renal carcinoma involving the IVC (n=35, 12.4%), tumor thrombus of liver carcinoma involving the IVC (n=24, 8.5%), leiomyosarcoma of the IVC (n=14, 4.9%), and tumor thrombus of adrenocortical adenocarcinoma (n=9, 4.1%). The presence of "sieve hole" and "multi-track" signs was observed in 20 IVL lesions under the grey-scale modality, while both signs were absent in the non-IVL group (P<0.01). The study found no statistically significant differences in the presentation of "sieve hole" and "multi-track" signs under the grey-scale and color Doppler modalities in cases of intravascular lithotripsy (IVL) (P>0.05). Using these two signs as diagnostic criteria for IVL, the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), miss rate, misdiagnosis rate, and accuracy were determined to be 29.9%, 100%, 100%, 82.2%, 70.1%, 0, and 83.5%, respectively (AUC ROC=0.649; 95%CI: 0.537-0.761). Conclusion: IVL exhibits distinct ultrasound presentations, including "sieve hole" and "multi-track" signs, which demonstrate high specificity and accuracy as diagnostic indicators. Furthermore, these signs are corroborated by pathological evidence and effectively distinguish IVL from other lesions occupying the IVC.

Intravenous leiomyomatosis of the uterus: Preoperative and intraoperative assessment.

OBJECTIVE: To assess factors influencing preoperative diagnosis and hemorrhage during surgery with uterine intravenous leiomyomatosis. METHODS: 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. RESULTS: 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. CONCLUSIONS: Treatment emphasis should focus on lesions extending to the broad ligament. Intraoperative bleeding associated with parauterine involvement should be stopped as effectively as possible.

Intravenous leiomyomatosis involving the right heart: Comprehensive evaluation using echocardiography combined with abdominal ultrasonography.

Intravenous leiomyomatosis (IVL) is a rare condition characterized by a tumor that originates within a pelvic vein wall or the uterine smooth muscle, with extension into the right heart (referred to as intracardiac leiomyomatosis) in approximately 10% of all IVL cases. Usually, computed tomography (CT) or magnetic resonance imaging (MRI) is performed for diagnostic imaging of IVL. Notably, this neoplasm presents with characteristic ultrasonographic findings. In this report, we present the case of a 49-year-old woman with IVL, which extended into the right heart. Echocardiography combined with abdominal ultrasonography was useful to outline the course of the tumor from the right heart to the uterus. Our findings suggest that in addition to CT or MRI, ultrasonography shows high diagnostic value in cases of IVL, and ultrasonography combined with CT or MRI can further improve the preoperative diagnosis rate of IVL.

Intravenous leiomyomatosis: Case report and review of the literature / Leiomiomatosis intravenosa: a propósito de un caso y revisión de la literatura

Introduction: We retrospectively reviewed a case of a 42-year-old woman with intravenous leiomyomatosis. Clinical findings: The present study describes a case of IVL extending into the right internal and common iliac veins. Diagnosis: The patient was diagnosed by definitive pathological examination after a hysterectomy and double salpingectomy. Hypovolaemic shock due to delayed intraperitoneal bleeding from the ovarian vessels pedicle was observed. An emergent laparotomy for haemostasis was performed. A follow-up MRI, 2 months later, showed a polylobulated mass extending inside the right internal and common iliac veins. Intervention and result: The patient underwent a third laparotomic procedure with the removal of the right pelvic mass together with the involved veins. The postoperative course was uneventful, and the patient remains well at 3-month follow-up, with no signs of lower limb edema or venous disorders. Conclusion: Intracardiac leiomyomatosis is mostly diagnosed in premenopausal women. The most severe manifestation could be a vascular thrombosis or a right atrial tumor in the case of intracardiac involvement. Early and appropriate diagnosis is essential for optimal treatment. Surgery is the best treatment.(AU)

Introducción: Presentamos el caso de una paciente de 42 años con leiomiomatosis intravenosa. Hallazgos clínicos: El estudio presente describe un caso de IVL con afectación de las venas ilíaca común e interna derecha. Diagnóstico: La paciente fue diagnosticada mediante el examen histológico definitivo tras una histerectomía total con doble salpingectomía. Se objetivó un shock hipovolémico por sangrado intraperitoneal tardío del pedículo de vasos ováricos. Se realizó una laparotomía urgente para hemostasia. Una RMN durante el seguimiento, 2 meses después, mostró una masa polilobulada con afectación de la vena ilíaca común y la vena ilíaca interna derecha. Intervención y resultado: A la paciente se le realizó una tercera laparotomía con la extirpación de la masa pélvica derecha. El curso postoperatorio fue satisfactorio y la paciente permaneció asintomática a los 3 meses de seguimiento, sin signos de edema en extremidades inferiores ni otras afectaciones vasculares. Conclusión. La leiomiomatosis intracardíaca se diagnostica más frecuentemente en mujeres premenopáusicas. La forma de manifestación más severa sería una trombosis vascular o un tumor intracardíaco en la aurícula derecha, en el caso de afectación intracardíaca. Un diagnóstico precoz es esencial para un tratamiento óptimo. La cirugía es el mejor tratamiento.(AU)