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Left: Large exophytic mass arising from superior, lateral aspect of the uterus growing superiorly into the right gonadal vein and IVC. Right: Large enhancing exophytic mass arising from superior aspect of uterus.
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Highlights
► Intravenous leiomyomatosis is histologically benign, but biologically aggressive. ► No diagnostic tools reliably distinguish it from leiomyosarcoma prior to surgery. ► Complete resection is curative, but antiestrogens can stabilize disease.
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Key Clinical Message
Soft tissue sarcomas are rare tumors accounting for less than 1% of all malignancies. Multidisciplinary approach is used in the management of these patients.
Citations
... The common symptoms of these tumors are abnormal uterine bleeding (AUB), pelvic pain or pressure, abdominal mass and also reproductive effects (e.g., Stasis due to alternation in blood flow (as it happens in very large uterine leiomyoma) and vascular endothelial injury. Thrombosis and thromboembolism in combination with fibroma have been reported rarely [4][5][6][7][8][9][10][11][12]. Furthermore, portal vein thrombosis which is defined as a complete or partial occlusion of portal vein has been reported less frequent than thrombosis in other vessels. ...
... The most common manifestations of uterine fibroma are abnormal uterine bleeding, pelvic pain, abdominal mass and symptoms due to pressure on adjacent organs. Venous thrombosis is a very rare and life-threatening complication of uterine fibromas and is reported more frequent in Iliac veins and IVC and can causes pulmonary emboli [4][5][6][7][8][9][10][11][12]. Portal vein thrombosis with a large myoma is reported less frequent than any other veins [12]. ...
... Venous thrombosis is a very rare and life-threatening complication of uterine fibromas and is reported more frequent in Iliac veins and IVC and can causes pulmonary emboli [4][5][6][7][8][9][10][11][12]. Portal vein thrombosis with a large myoma is reported less frequent than any other veins [12]. Stasis, endothelial damage and hypercoagulable state are three causes of thrombosis (Virchow's triad). ...
This article presents a case of portal vein thrombosis accompanied by a large uterine fibroma. A 37-year-old virgin woman presented with vaginal bleeding, abdominal mass, fever, dyspnea and lower limbs edema. In past medical history, she did not have any systemic diseases. She had menometrorrhagia from four years ago. She was admitted with diagnosis of a large uterine fibroma and was suspected of COVID 19 pneumonia or thrombophlebitis. The final diagnosis was a large uterine fibroma with chronic portal vein thrombosis. Although uterine fibromas are benign tumors, they may make serious life-threatening complications like thrombosis. The cause of thrombosis is the pressure effect of fibroma and/ or transfusion to treat anemia. Although there may be other unknown etiologies for thrombosis in these patients. We suggest that existence of a large uterine myomas should be known as a risk factor for thrombosis ( like IBS, Covid 19 and etc.) and be given a score in Caprini Score system, to start anticoagulation before and after any surgical intervention.
... However, complete resection cannot be performed in many cases for extension of tumors [5,24,27,37], densely adherent to the luminal wall [4, 5, 7, 13-16, 19-22, 41, 42], and patients' will [27,28], or constrained medical resources. In such conditions, recurrence occurred postoperatively [4,9,10,12,15,21,22,24], lethal complications such as uncontrolled hemorrhage [20,42], heart failure, and sudden death happened [3,7]. The complete resection rate was significantly lower in patients with firm/extensive adhesion than that in those with no/slight adhesion of IVC or above (Table 2). ...
Background:
Intracardiac leiomyomatosis (ICLM) is a rare life-threatening form of intravenous leiomyomatosis (IVLM). The incomplete resection and recurrence are associated with high morbidity and mortality. The objective of this study is to identify that whether estrogen deprivation therapies, including bilateral salpingo-oophorectomy (BSO)-based surgery and gonadotrophin releasing hormone agonists (GnRHa) administration, could bring benefits to patients with primary unresectable ICLM.
Methods:
PubMed/MEDLINE (Ovid) was searched (up to May 2021) for studies reporting individual patient data on demographics, clinicopathological features, treatment, and follow-up information. Exclusion criteria were patients who may have been included in two or more publications. This study was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines.
Results:
A total of 114 patients from 70 papers were included. Several reports showed that the tumor in the right atrium and inferior vena cava shrank dramatically after BSO-based surgery, or GnRHa administrated preoperatively in premenopausal women. The rate of complete resection was 64.04% in patients with ICLM, which was 85.25% in no/slight adhesion and no pulmonary nodules group, while 22.22% in firm/extensive adhesion and/or pulmonary nodules group (p < 0.0001). Meanwhile, the recurrence rates in patients with complete resection and incomplete resection were 4.29% and 37.84% respectively (p < 0.0001). Furthermore, complete resection with BSO had the lowest recurrence rate of 3.13%, incomplete resection with BSO had a progression rate of 45.45%, while incomplete resection with ovarian preservation had the highest progression rate of 75.00%.
Conclusions:
The recurrence rate of ICLM was closely related to firm/extensive adhesion in IVC or above, and/or pulmonary nodules. BSO-based surgery might reduce the recurrence rate no matter ICLM could be completely resected or not. In addition, estrogen deprivation therapies could decrease tumor burden as a primary treatment, and further make a secondary complete resection feasible in premenopausal women with initially unresectable ICLM.
... Regardless of whether surgery is performed, long-term follow-up is essential. In previously published studies, some patients showed continued growth of incompletely resected intravenous tumors from 7 months to 15 years after surgery, resulting in a high recurrence rate of 30% (46). Du et al. reported that the recurrence rate of 18 patients with IVL their 2011 study was 16.6%, suggesting that the young age of onset and the initial size of the tumor may be predisposing factors for recurrence (47). ...
Intravenous leiomyomatosis (IVL) is a relatively novel disease and can be aggressive. Since the first case was reported, it has a history of more than one hundred years, but the clinical incidence is extremely low, which has profound clinical research significance. Early recognition and management can prevent fatal consequences. IVL should be considered in female patients with a history of leiomyomas and intravascular filling defects. Because the patient's symptoms are not obvious, it is often misdiagnosed. We report on a female patient with IVL who presented with dizziness, and discuss the unexplained cause of filling defects within the right cardiac chambers and pulmonary arterial system. We present a complicated and rare case of IVL in a woman with a right atrial mass. A long, cord-like mass with inhomogeneous echo was detected by echocardiography. Coronary computed tomography (CT) showed a large hypodense mass occupying most of the right atrial cavity, spreading in both pulmonary arteries. Vascular ultrasound revealed a mixed echogenic mass invading the inferior vena cava like a rope. We performed surgery on her and complete resection on the tumor. We search for documents by searching databases such as National Center for Biotechnology Information (NCBI), China National Knowledge Infrastructure (CNKI) and Web of Science (WoS), etc. the recurrence rate recorded in the 4 cases series was 47.83% (11/23), 43.48% (20/46), 12.50% (2/16), and 10.00% (2/20), respectively.
... Some patients have reportedly shown continued growth of an incompletely excised intravenous tumor during a time period ranging from 7 months to 15 years after surgery, leading to a high recurrence rate of 30%. 23 Du et al. 10 reported a recurrence rate of 16.6% in 18 patients with IVL in 2011, suggesting that a young age at onset and the initial size of the tumor may be predisposing factors for recurrence. Surgery is still the most effective treatment for recurrent IVL. ...
Background
We retrospectively reviewed the data of three patients with intravenous leiomyomatosis (IVL), one of whom had intracardiac leiomyomatosis, and analyzed their clinical symptoms, preoperative assessment findings, operative approaches, and recurrence.
Case presentation: The present study describes three cases of IVL extending into the inferior vena cava, even as far as the right atrium and ventricle, and discusses the imaging findings and differential diagnosis of this tumor entity. The three patients, two of whom were diagnosed during the first operation and one of whom had a giant pelvic mass, were surgically treated with complete tumor resection, hysterectomy, and bilateral salpingo-oophorectomy. The pathological examination findings were suggestive of IVL. The duration of time from the first myomectomy or hysterectomy to IVL occurrence ranged from 2 to 18 months. No signs of recurrence were observed during follow-up. Computed tomography and magnetic resonance imaging played a vital role in the diagnostic process and presurgical assessment.
Conclusion
In clinical practice, IVL should be considered before surgery for a broad ligament myoma or giant pelvic mass. Surgery should always aim for complete tumor excision and include hysterectomy and bilateral salpingo-oophorectomy. Vascular reconstruction computed tomography is a good choice for diagnosis and follow-up.
... There are two main theories; the first one is: a tumour arises from the walls of venous structures and the second one is: a primary tumour (e.g. myoma) directly spreads into adjacent small venouses (Moniaga and Randall 2012). The early studies have reported an incidence of 0.25-0.41% of IVL in patients with uterine fibroid tumours (Ma et al. 2016;Low et al. 2017). ...
... Первостепенность вмешательства на сердце в случаях опухолевого распространенного лейомиоматозного поражения подчеркивается многими авторами [1,2]. Однако имеются хирурги, предпочитающие начинать удаление опухолевых масс в подобных случаях с выполнения лапаротомии. ...
... В целом, по мнению большинства специалистов, для внутривенного лейомиоматоза является своего рода критичным полное удаление опухолевых масс. Рецидив заболевания отмечается в 15-30 % случаев, как правило, возникает спустя 6 месяцев после операции и может происходить в срок до 15 лет после оперативных вмешательств [1,2]. ...
... Нередкими источниками образования опухолевых тромбов в магистральных венах бассейна НПВ являются опухоли матки. Среди новообразований матки возможность внутривенной инвазии имеется, прежде всего, при лейомиоме матки и некоторых видах лейомиосарком матки [24][25][26][27][28][29][30][31]. Характерная клиническая картина -как правило, субклиническое течение заболевания, и обширное опухолевое поражение подвздошных вен, нижней полой вены и сердца, позволили многим исследователям [22,29,31] называть эти ситуации «внутривенный лейомиоматоз» и «внутривенный лейомиосаркоматоз». ...
... «Подвздошное» распространение лейомиоматоза отмечается примерно у 70 %, «гонадное» -у 30 % больных [28]. Это заболевание поражает женщин преимущественно в среднем возрасте 40-45 лет, у значительной части из которых в анамнезе имело место удаление матки и(или) придатков [26,27,30,31]. H. Zeng и соавт. ...
... При инструментальном обследовании (КТ, ядерная МРТ, эхокардиография) часто выявляется удручающая картина свободно флотирующих внутрисердечных масс. В постановке окончательного диагноза в последние годы активно используются методы эндоваскулярной биопсии или транскутанной биопсии под КТ-контролем [27,30]. ...
... Long-term follow-up is recommended for intravenous leiomyoma because of the high possibility of recurrence [21,30]. Multiple studies have reported recurrence rates of up to 30%, with a follow-up range of 7 months to 17 years [21,31,32]. It is suggested by Matos et al. that MR imaging is preferable for follow-up due to its superior soft tissue contrast resolution, being nonradiating, and with a higher safety profile of the intravenous contrast media [3]. ...
Leiomyoma of uterine origin is a common histologically benign neoplasm in women; however, growth intravenously with intracardiac extension is a rare phenomenon. This is a diagnostic challenge that can present with varied clinical manifestations and multiple differential diagnosis. This is a case of a 45-year-old female patient with chest heaviness and an intracardiac mass on 2-dimensional (2D) echocardiogram. Previous history of hysterectomy was likewise noted. Imaging workup, including 2D echocardiogram and contrast-enhanced chest and abdomen computed tomography scans, was performed which demonstrated a large, heterogeneous, elongated filling defect in the right atrium and right ventricle extending to the inferior vena cava, left renal vein, and left gonadal vein. The diagnosis was made after resection of the tumor in a single-stage operation. The histopathologic and immunoprofile of the resected tumor were consistent with leiomyoma. The use of multiple imaging modalities such as 2D echocardiogram and computed tomography are essential in the investigation of the intracaval masses with intracardiac extension. Although intravenous leiomyoma with intracardiac extension is a rare phenomenon, radiologists and clinicians alike should be mindful of this differential diagnosis.
... 8 IVL is most commonly misdiagnosed as venous thrombosis and atrial myxoma. 9,10 Growth of the tumor into the venous system follows the pattern of drainage from the uterus, starting in the ovarian vein and leading into the internal iliac and common iliac veins. The internal iliac vein drains the uterine plexus and vaginal plexus of veins. ...
Intravascular leiomyomatosis (IVL) is a benign smooth muscle tumor that evolves from the pelvic veins and can spread to the central veins and heart. Cardiac involvement is the most commonly reported presentation. Initial diagnosis is difficult, and IVL is commonly misdiagnosed as thrombus or atrial myxoma. Appropriate imaging and a high clinical suspicion are required for accurate diagnosis. We report a rare case of IVL in the external iliac vein that recurred 4 years after hysterectomy. Only four cases have been reported in the literature to involve the external iliac vein as it has no direct connection to pelvic venous drainage.
... Согласно другой теории, внутривенный лейомиоматоз первично развивается из мышечных клеток венозной стенки. Макроскопически внутривенный лейомиоматоз представляет собой спиральные массы в просвете яичниковой или подвздошной вен, фиксированные к интиме в нескольких местах, распространяющиеся в просвет нижней полой вены, вплоть до правых отделов сердца [8][9][10]. ...
