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

Su, Qingbo; Zhang, Xiquan; Zhang, Hui; Liu, Yan; Dong, Zhao‐Ru; Li, Guangzhen; Ding, Xiangjiu; Yang, Liu; Jiang, Jianjun

doi:10.1155/2020/9758302

Cited by 22 publications

(24 citation statements)

References 29 publications

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“…IVL is an extremely rare tumor that affects predominantly women of reproductive age, as it expresses both estrogen and progesterone hormone receptors. The etiology is unknown; however, the hypothesis is that it originates from the smooth muscles of the uterine vessels or by intraluminal invasion of the uterine fibroids [ 4 ]. Initial presentation is often vague, with a variety of clinical symptoms dependent on the origin, size, and growth of the tumor.…”

Section: Discussionmentioning

confidence: 99%

Intravenous Leiomyomatosis: An Uncommon Cause of Pulmonary Embolism

et al. 2021

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Patient: Female, 39-year-old Final Diagnosis: Intravenous leiomyomatosis Symptoms: Dyspnea • syncope Medication: — Clinical Procedure: — Specialty: Cardiology • Critical Care Medicine • General and Internal Medicine Objective: Rare disease Background: Intravenous leiomyomatosis (IVL) is a rare benign smooth muscle tumor originating in the uterus or in the uterine vessels. It is characterized by continuous intraluminal growth that may extend through iliac veins and inferior vena cava (IVC) to right chambers of the heart and pulmonary vasculature, leading to life-threatening complications. This case report describes an uncommon cause of non-thrombotic pulmonary embolism in young woman caused by extensive IVL. Case Report: A 39-year-old woman was admitted after multiple syncopal episodes. She was initially found to have a bilateral pulmonary embolism and large right atrial mass believed to be a thrombus. After an unsuccessful attempt to remove the thrombus with AngioVac (AngioDynamics, Latham, NY), subsequent sternotomy revealed a large pedunculated mass extending to the infra-hepatic IVC. Further abdominal imaging showed multiple uterine masses, with the largest about 17 cm, infiltrating the parauterine vessels and extending through the right iliac vein and inferior vena cava up to the right atrium. Pathology examination of the atrial mass revealed benign leiomyoma consistent with further pathology findings after hysterectomy. The pulmonary embolism was believed to be caused by tumor tissue, and anticoagulation was abandoned. Pulmonary nodule raised a suspicion of benign pulmonary metastases, but, fortunately, remained stable during follow-up and the patient had a successful recovery. Conclusions: Available information about IVL is scarce. This tumor, although benign and rare, should be included in the differential diagnosis of cardiac tumors and non-thrombotic pulmonary emboli in women with predisposing risk factors, as potential complications are life-threatening.

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Section: Discussionmentioning

confidence: 99%

Intravenous Leiomyomatosis: An Uncommon Cause of Pulmonary Embolism

et al. 2021

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“…Recent study shows that thoracotomy could be avoided by gently pulling the tumor from the abdominal level [28] . Nowadays one-stage approach are more frequently proposed because of increased knowledge of this disease [30] , [33] . Most of the one-stage procedures are performed with cardiopulmonary bypass and total circulatory arrest but it is possible to proceed with heart beating surgery because of the absence of adhesion to the IVC or the heart [34] , [35] .…”

Section: Ivl: Review Of Literaturementioning

confidence: 99%

Intravenous leiomyomatosis: Case series and review of the literature

Mathey

Huber

2021

International Journal of Surgery Case Reports

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Introduction Intravenous leiomyomatosis (ILV) is a rare pathology, part of leiomyoma beyond the uterus (LBU), characterized by benign smooth muscle cell tumor outside of the uterus and mainly affecting premenopausal woman with a medical history of leiomyoma or gynecologic surgical treatment. The treatment depends on the localization of the tumor, age of the patient, initial size, symptoms and the suitability for surgery but should always aims in toto surgical resection. Case presentation Retrospective case series and review of literature. Clinical discussion Symptoms presented by the patient were aspecific and only localized in the pelvic area. All cases were fortuitous histopathological diagnosis. No relapse was. Two out of 5 patients have pulmonary nodules, only one was biopsied and diagnosed with PBML (pulmonary benign metastasizing leiomyoma). Conclusion IVL and BML are rare disease that can co-exist. Because of tumoral hormonal receptors, hormonotherapy could be an optional treatment but to date no clear efficacy is demonstrated. In case of high recurrence risk such as voluminous initial mass, impairment of broad ligament, failure of total surgical resection, adjuvant hormonotherapy could be useful. Recurrence rate is about 16.6-30% and can occur even dozen years later and even after radical surgery, justifying a regular follow up.

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“…The tumor cells can reach the inferior vena cava, the right heart and the lungs. In a series of 14 cases of IVL [14],…”

Section: )mentioning

confidence: 99%