Intravascular leiomyomatosis with intracardiac extension: a toraco-abdominal approach

Miró, Antonio; Bottazzi, Enrico Coppola; Vanella, Serafino; Palma, Tommaso; Noviello, Adele; Apicella, I.; Lombardi, G; Fiorani, Brenno; Crafa, Francesco

doi:10.1093/jscr/rjab249

Cited by 5 publications

(3 citation statements)

References 11 publications

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“…By contrast, there are low ER or PR expression levels in endothelial and subendothelial cells. This feature is consistent with the theory that IVL originates from uterine leiomyomas (13). In addition, several studies have reported the comparison of the gene expression between IVL and uterine leiomyomas (14-16).…”

Section: Etiologysupporting

confidence: 88%

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

Zhou,

Qi,

Zhao

et al. 2023

Oncol Lett

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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.

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Section: Etiologysupporting

confidence: 88%

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

Zhou,

Qi,

Zhao

et al. 2023

Oncol Lett

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show abstract

“…Common signs included pelvic pain, abdominal discomfort, and menorrhagia. In advanced-stage cases, when the inferior vena cava or the right heart and pulmonary artery were involved, some patients had dyspnea, chest pain, lower limb edema, and syncope, while some patients had no cardiopulmonary symptoms before the occurrence of cardiac insufficiency, pulmonary embolism, or sudden death ( 7 , 13 , 69 , 244 ). Therefore, IVL has an insidious onset and complicated clinical manifestations, and lacks specificity, with patients usually noticing IVL only after the tumor has invaded the inferior vena cava or above, causing serious clinical symptoms that are difficult to diagnose.…”

Section: Discussionmentioning

confidence: 99%

Clinical features and prognostic factors analysis of intravenous leiomyomatosis

Chen

Zhang

et al. 2023

Front. Surg.

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BackgroundThe treatment and prognostic factors of intravenous leiomyomatosis (IVL) remain lacking systematic evidence.MethodsA retrospective study was conducted on IVL patients from the Qilu Hospital of Shandong University, and IVL cases were published in PubMed, MEDLINE, Embase and Cochrane Library databases. Descriptive statistics were used for the basic characteristics of patients. The Cox proportional hazards regression analysis was used to assess the high-risk factors related to the progression-free survival (PFS). The comparison of survival curves was performed by Kaplan–Meier analysis.ResultsA total of 361 IVL patients were included in this study, 38 patients from Qilu Hospital of Shandong University, and 323 patients from the published literature. Age ≤45 years was observed in 173 (47.9%) patients. According to the clinical staging criteria, stage I/II was observed in 125 (34.6%) patients, and stage III/IV was observed in 221 (61.2%) patients. Dyspnea, orthopnea, and cough were observed in 108 (29.9%) patients. Completed tumor resection was observed in 216 (59.8%) patients, and uncompleted tumor resection was observed in 58 (16.1%) patients. Median follow-up period was 12 months (range 0–194 months), and 68 (18.8%) recurrences or deaths were identified. The adjusted multivariable Cox proportional hazard analysis showed age ≤45 years (vs. >45) (hazard ratio [HR] = 2.09, 95% confidence interval [CI] 1.15–3.80, p = 0.016), and uncompleted tumor resection (vs. completed tumor resection) (HR = 22.03, 95% CI 8.31–58.36, p < 0.001) were high-risk factors related to the PFS.ConclusionPatients with IVL have a high probability of recurrence after surgery and a poor prognosis. Patients younger than 45 years and with uncompleted tumor resection are at higher risk of postoperative recurrence or death.

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“…Given the rarity of IVL, there may be a delay in diagnosis or a chance of misdiagnosis, for example, attributing a right-sided cardiac mass to a right atrial myxoma. Other differentials worth considering include an extension of a tumor thrombus (TT) from a renal cell carcinoma, Budd-Chiari syndrome, propagation of a deep vein thrombus, or endometrial stromal sarcoma [6]. With these differentials, one can appreciate the difficulty in discerning between a thrombus and a tumor in transit.…”

Section: Discussionmentioning

confidence: 99%

Tumor in Transit: Intracardiac Leiomyomatosis

Abraham,

Marsic,

Das

et al. 2023

Cureus

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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.

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Intravascular leiomyomatosis with intracardiac extension: a toraco-abdominal approach

Cited by 5 publications

References 11 publications

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

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

Clinical features and prognostic factors analysis of intravenous leiomyomatosis

Tumor in Transit: Intracardiac Leiomyomatosis

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