Intracardiac Leiomyomatosis Research Articles

P75 MULTIDISCIPLINARY MANAGEMENT OF INTRACARDIAC LEIOMYOMATOSIS: A CASE REPORT

Abstract A 60–year–old woman with no cardiological history came to our attention for exertional dyspnea since few months. The electrocardiogram and chest radiograph were normal. Trans–thoracic echocardiogram revealed the presence of a floating parenchymatous formation measuring 12 x 2 cm starting from the inferior vena cava (IVC) and jutting into the right chambers, without obstruction to inflow. Chest CT and cardiac MRI described a nonvascularized inhomogeneous formation suggestive of thrombotic apposition in VCI, so the patient was treated with unfractionated heparin. Total body CT/PET scan excluded hypercaptant lesions. After two weeks of anticoagulant therapy, on echocardiogram the mass appeared unchanged in morphology and size. On reevaluation of the CT images, a probable origin of the formation at the level of the right ovarian vein was identified. In light of the positive history of bilateral hysteroannessiectomy for uterine myomas and the lack of response to anticoagulant treatment, the suspicion of intravascular and intracardiac leiomyomatosis was raised. After collegial discussion, given the high risk of an eventual thoraco–abdominal surgery, it was decided to remove only the portion of the mass adherent to the VCI jutting into the right heart chambers. The patient underwent cardiac surgery: a polypoid, capsular, hard–elastic mass, 12 x 2 cm in size, jutting into the right chambers and with the base of implantation located in the anterior wall of the VCI, was removed. The final histologic diagnosis was sclero–jalin leiomyoma. The surgery and postoperative course proceeded without complications. At follow–up at 20 months, the patient is asymptomatic and no mass is detectable on echocardiogram. Intracardiac leiomyomatosis is a rare condition characterized by benign smooth muscle tumors of extracardiac origin reaching the right heart by direct extension into the venous canal, described predominantly in women with a history of uterine myomas. Although histologically benign, intracardiac leiomyomatosis can result in sudden death if there is total obstruction to the outflow tract. In our case, the multimodality imaging approach and serial monitoring by echocardiography were essential to guide treatment choices and for the follow up.

One-stage resection of intravascular leiomyomatosis involving the right heart chamber through a single laparotomy.

This retrospective study aimed to summarize the feasibility and experience of utilizing a one-stage operation via single laparotomy to treat intracardiac leiomyomatosis (ICL). A retrospective study of 13 patients with ICL who underwent one-stage resections was conducted at Peking Union Medical College Hospital from June 2015 to December 2021. All patients had their tumors removed by single laparotomy and were divided into a short venotomy group (6 cases) and an extensive venotomy group (7 cases). We reviewed the patient characteristics, surgical procedures, postoperative pathology, and perioperative and follow-up outcomes of all patients. All patients underwent surgery for ICL resection using single laparotomy with a 100% success rate. Two patients had tumors distal to the right ventricle (RV), 2 patients had tumors that protruded into the RV in diastole and were confined to the right atrium (RA) in systole, and the other 9 patients had tumors confined to the RA that did not involve the tricuspid valve. The tumor was completely resected in 10 patients, yet 3 patients had a residual tumor. Six patients completed the surgery with short venotomy, 7 completed the surgery with extensive venotomy, and 9 underwent simultaneous total hysterectomy and bilateral adnexal resection. The mean operative time was 370.8 ± 111.0 min, and the mean blood loss was 992.3 ± 994.5 mL. Intraoperative blood loss was lower (483.3 ± 213.7 ml vs. 1429.2 ± 1208.0 ml; P = 0.020) and operative time was shorter (286.5 ± 71.9 min vs. 443.1 ± 84.4 min; P=0.004) in the short venotomy group than in the extensive venotomy group. At a mean follow-up of 26.3 ± 18.8 months, 1 patient had a local recurrence in the pelvis, and 1 patient died of pancreatic cancer, while the remaining patients had no recurrence during follow-up. One-stage resection of ICL patients by means of a single laparotomy is feasible and effective.

Anesthetic Management of a Rare Case of Uterine Leiomyoma with Intravenous and Intracardiac Leiomyomatosis

Anesthetic Management of a Rare Case of Uterine Leiomyoma with Intravenous and Intracardiac Leiomyomatosis

A Case of Syncope Due to Intracardiac Leiomyomatosis

We present a case of a 46-year-old female presenting with syncope. Echocardiography initially showed a right atrial mass. Further evaluation revealed a mass arising from the fundus of the uterus, with a tumor thrombus in the left gonadal vein, extending into the left renal vein and through the inferior vena cava (IVC) into the right heart across the tricuspid valve. She was managed with surgical resection, and postoperative pathology was consistent with intravenous leiomyomatosis (IVL). IVL is a rare uterine smooth muscle cell neoplasm which extends into the venous system. Gynecological tumors are often overlooked in differential diagnosis for atrial masses. A benign tumor like fibroid, in rare circumstances, can extend into the right side of the heart causing dynamic obstruction to outflow tract, thus increasing mortality. The objective of this article is to present such a case and highlight the broad differentials of atrial masses, including IVL.

Surgical treatment strategies for extra-pelvic intravenous leiomyomatosis

BackgroundExtra-pelvic intravenous leiomyomatosis (IVL) extending into inferior vena cava (IVC) or heart (i.e. intracardiac leiomyomatosis, ICL) is an extremely rare benign disease. No consensus has been reached on the optimal surgical strategy. The aim of this study is to introduce four types of one-stage surgical strategies including less invasive options and a guideline to select patient-specific strategy for this disease.MethodsTwenty-four patients of extra-pelvic IVLs receiving one-stage resections at the Zhongshan Hospital from July 2011 to November 2019 were reviewed retrospectively. Base on the initial experiences of the indiscriminate choices of tumor thrombectomies through sterno-laparotomy under cardiopulmonary bypass (CPB) in 6 ICLs, an anatomy-based guideline for four types of surgical strategies was developed and applied for the next 18 patients.ResultsUnder the direction of guideline, tumor thrombectomies through single laparotomy were applied without CPB in 2 ICLs and 4 IVLs confined in IVC, or with CPB in 7 ICLs. Guideline-directed double-incisions with CPB were applied in only 5 ICLs, including 1 receiving mini-thoracotomy and 4 receiving sternotomy because of tumor adherences with right atriums in 2 and with pulmonary arteries in 2. All 24 patients accomplished one-stage panhysterectomy, bilateral adnexectomy and complete resections of intracaval and intracardiac tumors. For residual pelvic intravenous tumors in 19 patients, 17 received macroscopically complete resections while the other 2 failed because of high risk of hemorrhage. Intraoperative blood losses, operation time and hospitalization expense in the single-laparotomy non-CPB group were significantly lesser than the other groups. In CPB groups, inpatient stay and hospitalization expense in the single-incision group were significantly lesser than the double-incisions group. All patients were alive and free of recurrences during a mean follow-up of 35.4 ± 27.2 months (range, 1–100 months). The pelvic tumor residues in 2 patients remained unchanged for 51 and 52 months since operation, respectively.ConclusionsFor various extra-pelvic IVLs, the 4 types of surgical strategies including less invasive options are feasible, providing these are selected by a guideline base on the tumor extension and morphology. The proposed guideline is believed to accommodate more patients receiving less invasive surgery without compromising the curative effect.

P1704 An unusual mass in the right heart

Abstract A 39 year old patient after removal of the uterus, the left oviduct and the right appendages due to Tu (massive fibroids). The histopathological examination revealed: leiomyomatosis intravascularis masiva (multifocal, intravenous smooth muscle cell hyperplasia without necrosis, cellular atypia and mitotic activity. Vascular invasion within the uterus, parametrium, mesovarium, perinodule tissues). The patient was admitted to the Gynaecology Clinic on the 9th postoperative day due to pain in the right lumbar region. The angio-CT of the thoracic, abdominal and pelvic cavities revealed a tumour/thrombus in the right internal iliac vein, common iliac vein and inferior vena cava (VCI). Then, the patient underwent transesophageal echocardiography (TEE) and transthoracic echocardiography (TTE) revealing an additional mass in the VCI entering the right atrium (RA), periodically passing through the tricuspid valve to the right ventricle (RV). The mass was of irregular shape, free-floating and did not obstruct the right ventricle inflow. Due to a suspicion of thrombus, an attempt at pharmacological treatment was initiated: initially LMWH at a therapeutic dose, followed by UFH iv. (controlled by APTT). Follow-up echocardiography: the image has not changed, the mass is still in the same location. The patient then underwent a surgical treatment: the pathological mass with a length of 35 cm was removed from VCI and RA with extracorporeal circulation. The histopathological examination confirmed: numerous foci of intravenous leiomyomatosis in the form of smooth muscle hyperplasia penetrating into the light of numerous vessels, mainly venous. Discussion Uterine fibroids are the most common benign tumours of the reproductive tract in premenopausal women, they are also the most common cause of hysterectomy. They are formed as a result of benign proliferation of myometrial smooth muscle cells. A special, though very rare, form is intravenous leiomyomatosis (IVL) - caused by abnormal growth of benign tumours, arising from smooth muscle, into venous vessels. Although histopathologically they are benign tumours, due to the possible invasion of large vessels they can be highly "aggressive" and extend into the heart chambers and pulmonary arteries (intracardiac leiomyomatosis - ICLM). Possible symptoms of ICLM: dyspnoea (30-37%), peripheral oedema (20-28%), chest pain (12-30%), palpitation (10-17%), fainting (11%). Approximately 13% of patients have no symptoms at all. In the differential diagnosis of masses in the right heart cavities, the following should be taken into account : thrombus, myxoma, metastases of malignant neoplasms, in particular renal cancer, as well as: liver cancer, adrenal cancer, lymphoma, leiomyosarcoma. Conclusion IVL should be taken into account as a differential diagnosis in case of a free-floating mass in the right heart chambers, originating from the inferior vena cava, among premenopausal women with a history of hysterectomy. Abstract P1704 Figure. mass in the right atrium

P169 Uterine leiomyoma extension into right side cardiac

Abstract Funding Acknowledgements governmental grants Uterine leiomyoma is a commom disease in women, however, intravenous leiomyomatosis of uterine origin extending via inferior vena cava into the right side of the heart,known as intracardiac leiomyomatosis is a rare condition (3%). In 1907, Durk reported the first case of intracardiac extension. The patient was a Woman, 35 years old, admitted to our emergency department for an intracardiac mass. She had shortness of breath,fatigue and chest pain. The transthoracic two dimensional echocardiography showed an echogenic oval mass mobile in right atrium and projected through right ventricle in diastole. This mass was observed to extend from inferior vena cava to the right atrium. The echotransesophageal three dimensional showed a large mobile mass that extended from inferior vena cava to the right atrium. A Computer tomographic (CT) scan showed a hypodense multilobulated mass in the pelvis, which had invaded the inferior vena cava and right atrium. The patient underwent a two stages surgery. In first stage (transatrial tumor resection). The operation was performed normal temperature with establisment of cardiopulmonary bypass (CPB). Subsequently, the pathological report was confirmed uterin smooth muscle origin. The second stage surgery ( total histerectomy) was done four weeks later for removing lobulated mass uterin with dimensions 20x15x7.5cm with confirmed histopathological of leiomyoma. Because of it is nonspecific clinical presentation and rarity, an intracardiac Leiomyomatosis continues to be a misdiagnosed as either thrombus or myxoma. The cardiac imaging techniques like a transthoracic echocardiography 2d and transesophageal echocardiography 3d have been used to define the presence, extension of tumor as appearance of the mass and involvement of adjacent structures. Abstract P169 Figure. Echotransesophageal 3D (bicaval view)

Surgical treatment of intravenous leiomyomatosis involving the right heart: a case series.

ObjectiveThis study was performed to investigate the surgical treatment of intravenous leiomyomatosis involving the right heart.MethodsThe clinical data of five patients with intracardiac leiomyomatosis treated from April 2002 to October 2017 at a single center were retrospectively analyzed.ResultsAll five patients underwent successful intravenous and right atrial tumor removal via abdominal and inferior vena cava incisions. In three patients, these incisions were combined with thoracotomy and a right atrial incision, and in two patients, they were combined with uterine and bilateral fallopian tube and ovarian resection. One patient with advanced disease underwent a one-stage procedure and died thereafter. Of the remaining four patients who underwent follow-up for 1.5 to 12.0 years, one developed recurrence at 1 year postoperatively. The recurrent tumor, which was pathologically confirmed to be an intravenous leiomyoma, was removed via inferior vena cava and internal iliac vein incisions without subsequent recurrence.ConclusionsThe main treatment goal for inferior vena cava leiomyomas involving the right heart is to first address the severe obstruction of cardiac blood flow and then pursue second-stage surgery. Concurrent thoracotomy appears unnecessary because moderately sized right heart tumors can be gently removed via the inferior vena cava.

Perioperative management of intracardiac leiomyomatosis: An observational cohort study.

Intracardiac leiomyomatosis (ICLM) is a rare condition in which the benign tumor extends into the right heart chambers through inferior vena cava. The best surgical approach still remains unclear.We present a retrospective cohort of 36 patients diagnosed with ICLM in Peking Union Medical College Hospital between 2002 and 2016.The mean patient age was 44.5 (range 25–55) years. The clinical manifestations were various, including shortness of breath, chest tightness, edema of the lower extremities, palpitations, syncope, etc. Cardiac function of 30 patients (80%) remained mildly influenced, classified as New York Heart Association (NYHA) I-II. After careful preoperative evaluation, 19 patients underwent 1-stage operation while the other 17 patients underwent 2-stage operations. The original surgical plans were changed in 5 patients (14%) due to intraoperative transesophageal echocardiography (TEE) monitoring, with the tumor directly extracted through abdominal approach or right atrium without cardiopulmonary bypass and/or deep hypothermic circulatory arrest. Complete resection was achieved in 32 patients (89%). Despite increased volume of blood loss (P < .05), patients undergoing 1-stage operation had significantly shorter operation time, anesthesia time as well as hospital length of stay (P < .05), compared with 2-stage operations. The postoperative complication rates were not different between the 2 groups (P = .684). During mean follow-up time of 36.1 months, recurrence occurred in 7 patients (23%) but all are survived.Precise and full-scale preoperative evaluation of both the tumor anatomy and the patient's tolerability to the surgery should be performed. TEE plays a crucial role in guidance of surgical decision making, and 1-stage extraction of tumor through either abdominal approach or right atrium may be possible.

Intravenous leiomyomatosis with right atrium extension in two patients: A case report.

Intravenous leiomyomatosis (IVL) is a rare benign tumor of the uterus mesoderm, which can spread via the vein, from the micro-vein to the inferior vena cava, and even to the right atrium. IVL was first described this disease in 1896 and the first presented case of IVL with right atrium extension in the autopsy was described in 1907. On rare occasions, the tumors may extend into the regional and systemic veins, thus reaching the heart. This may subsequently cause intracardiac leiomyomatosis (ICL), which may lead to congestive heart failure and on occasion, sudden fatalities. Due to its rarity and diffuse symptoms, the misdiagnosis of ICL is common and as a result, the condition may be under-reported. The present study reported two cases of IVL resected at the General Hospital of Shenyang Military Command. A hysteromyomectomy for uterine fibroids was performed on each patient (Case 1, 41 years ago; Case 2, 3 years ago). One patient presented with chest pain following a period of activity and the other presented with heart neoplasm during a routine health examination.

One-stage complete resection of giant intracardiac leiomyomatosis with moderate hypothermia extracorporeal circulation and beating heart technique with 36 months follow-up—a case report

BackgroundIntracardiac leiomyomatosis (ICL) is a rare benign neoplasm of the smooth muscle in the uterus extending into the heart. Complete resection is difficult because of the extensive range.Case PresentationWe report a case of one-stage complete resection of a giant ICL with moderate hypothermia extracorporeal circulation and beating heart technique.ConclusionsThe outcome of 36 months follow-up was very good.

Multiservice single-stage surgery for intracardiac leiomyomatosis

Intracardiac leiomyomatosis is a rare case that is often confused with more common diseases typically right atrial myxoma. It involves tumor extension of the uterine wall tumor through the inferior vena cava (IVC) and into the right atrium. Since its discovery in 1907, very few cases have been reported. The disease is treated surgically using one of two surgical approaches: the two-stage and the single-stage approach. The following case report involves an unusual presentation of a Caucasian woman with intracardiac leiomyomatosis who underwent a successful single-stage approach with coordinated involvement from Gynecological-Oncological, Urological, Vascular, and Cardiothoracic Surgery services.

Intravenous Leiomyomatosis with Intracardiac Extension: Echocardiographic Study and Literature Review

Uterine leiomyomatosis is a common disease in women; however, intravenous leiomyomatosis with intracaval and intracardiac tumor extension is rare. We sought to analyze the clinical and echocardiographic features of intracardiac leiomyomatosis. From January 2003 through July 2012, 7 women (age range, 24-59 yr) underwent surgical resection of histopathologically diagnosed intracardiac leiomyomas at our hospital. Most of the patients had histories of hysterectomy or uterine leiomyoma. We retrospectively analyzed their preoperative echocardiograms. We found that the tumors had no stalks, did not adhere to the wall of the right side of the heart, were highly mobile, and moved back and forth in the right atrium near the tricuspid orifice. All tumors originated from the inferior vena cava and had borders well demarcated from that structure's wall. Most of the masses extended into the inferior vena cava and right atrium through the right internal and common iliac veins. Computed tomograms revealed pelvic tumors and contiguous filling defects in 6 patients. When echocardiograms reveal a right-sided cardiac mass that originates from the inferior vena cava, particularly in women who have a history of hysterectomy or uterine leiomyoma, intracardiac leiomyomatosis should be suspected. If the mass has no stalk and freely moves within the inferior vena cava and right-sided cardiac chambers without attachment to the endothelial surface or endocardium, intracardiac leiomyomatosis should be diagnosed. We discuss our findings and briefly review the relevant medical literature.

Intracardiac Leiomyomatosis: Clinical Findings and Detailed Echocardiographic Features—A Chinese Institutional Experience

Intravenous leiomyomatosis is a rare, benign, smooth muscle tumor originating in the uterus that may extend through the inferior vena cava into the heart. Intracardiac leiomyomatosis (ICL), present in 10% of patients with intravenous leiomyomatosis, may cause right heart failure, tricuspid valve obstruction, and pulmonary embolism. The imaging characteristics of ICL continue to be reported. The purposes of this study were to characterize the echocardiographic features of ICL and to correlate the clinical findings. Between 1999 and 2012, 12 female patients with suspected ICL underwent cardiac surgery and histologic confirmation of the tumor. The clinical data, echocardiographic findings, and histologic results were retrospectively reviewed. The ages of the patients with ICL ranged from 40 to 59 years. Ten patients (83%) had undergone myomectomy or hysterectomy, one patient had a uterine fibroid, and one patient had endometriosis. Seven patients (58%) reported dyspnea and/or palpitations, and one patient had syncope; four patients were asymptomatic. Echocardiographic findings included six patients with homogenous right atrial masses, four patients with myxoma-like right atrial masses, and two patients with serpentine, convoluted right atrial masses. In nine patients, the right atrial masses were noted to cross the tricuspid valve. All masses extended from the inferior vena cava. No masses appeared to adhere to the right atrium, right ventricular or pulmonary arterial walls, or tricuspid valve. Tricuspid regurgitation was noted in all patients. No pulmonary emboli were present. The echocardiographic features of the ICL tumors varied. Tricuspid regurgitation and tumors emerging from the inferior vena cava were seen in all patients. Cardiac symptoms, including dyspnea, palpitations, and syncope, occurred in 67% of patients; the remaining 33% were asymptomatic.

Successful one-stage extraction of an intracardiac and intravenous leiomyoma through the right atrium under transesophageal ultrasound monitoring

Intravenous leiomyomatosis is a rare disorder characterized by benign smooth-muscle tumours, termed leiomyomas, which originate from uterine leiomyomas or pelvic veins. Tumours may extend into the right-sided heart chambers, termed intracardiac leiomyomatosis (ICLM), and may be potentially life-threatening due to mechanical interference with cardiac structures or pulmonary arteries. While surgical excision is the optimal therapy, incomplete retrieval of a tumour or fatal retroperitoneal hemorrhage may occur. We present a case where intraoperative transesophageal ultrasound (TEU) guided complete removal of an intracardiac leiomyoma in a single-stage surgery solely through the right atrium without vein injury. A 46-yr-old female patient presented with a two-week history of exertional dyspnea, palpitations, and syncope. Preoperative imaging modalities revealed a continuous solid mass extending from the inferior vena cava (IVC) into the right atrium, and the patient subsequently underwent open heart surgery for tumour removal and definitive diagnosis. A systematic intraoperative TEU examination performed before resection showed that the serpentine tumour was free from any attachment to the IVC and the heart. Furthermore, the diameter of the intracardiac end of the tumour was wider than that of the IVC. Given these findings, the surgeons carefully drew the cord-like tumour out of the right atrium under close TEU monitoring without vein injury. Post-extraction TEU examination showed complete removal of the tumour. Microscopic examination of the specimen confirmed the diagnosis of intravenous leiomyomatosis. For cases with ICLM, intraoperative TEU plays a significant role in helping to plan the surgical approach, monitor the movement of the tumour and the IVC during the extraction, and assess the completeness of tumour resection.

EComment. Misdiagnosis of intravenous leiomyomatosis with cardiac extension

We read with great interest the article by Li et al. [1]. They performed a comprehensive literature search including 194 cases of intracardiac leiomyomatosis. We also would like to add some comments on misdiagnosis of this complicated pathology. Intravenous leiomyomatosis (IVL) is a rare neoplastic disease, which is characterized by intravenous growth of histologically benign, smooth muscle tumours arising from either a uterine myoma or from the wall of a uterine vessel [2, 3]. The tumour usually enters through the lumen of the iliac vein and extends upward to varying locations, including the inferior vena cava, adrenal and renal veins, right heart (intracardiac leiomyomatosis) and pulmonary artery. It is an insidious process and may lead to heart failure, pulmonary embolization, or even sudden cardiac death. Intracardiac extension may be easily misdiagnosed as either a primary cardiac tumour or a venous thrombus-in-transit. Misdiagnosis subsequently may lead to improper treatment [2, 4, 5]. Differential diagnosis of right-sided cardiac masses includes thrombi-in-transit, right atrial myxomas, vegetations on the tricuspid valve, primary caval leiomyosarcoma, intracardiac electrodes, and tumour thrombi from malignancies such as hepatocellular carcinoma, renal cell carcinoma, Wilms' tumour (in children), and others [3, 4]. IVL may be diagnosed by echocardiography, abdominal ultrasound, magnetic resonance imaging, enhanced chest and abdominal computed tomography, and venography. The diagnosis is confirmed intraoperatively or by postoperative histopathological examination [3, 5]. A thrombus-in-transit appears as an elongated mobile mass of venous cast giving a popcorn appearance within the right-sided cardiac chamber, whereas there are multiple points of attachment in the inferior vena cava and right-sided cardiac chambers in patients with IVL [8]. A correct diagnosis depends on a high index of suspicion. This pathology should be considered in young women with a right-sided cardiac mass and a pelvic mass or those who have previously undergone hysterectomy for uterus leiomyoma [2, 3, 5]. Finally, we think that IVL is an underdiagnosed pathology and a huge index of clinical doubt with the modern diagnostic imaging tools will lead to an increased number of reported cases in the future. Conflict of interest: none declared.

EComment. Leiomyomatosis: intracardiac extension and pulmonary embolization

I read with great interest the article by Li et al. [1]. As rightly outlined in this remarkable review of the literature, extracting data solely from case reports is associated with an inherited bias. Only three intraoperative deaths were recorded, thus yielding a mortality rate of 1.5%. The one-stage approach in which a thoraco-laparotomy is performed under cardiopulmonary bypass with or without hypothermic circulatory arrest is challenging, is time-consuming, and carries a high incidence of perioperative complications. This one-stage operation is the procedure of choice for patients with cavo-atrial extension of renal cell carcinoma. Renal cell carcinoma, like uterine leiomyomatosis, characteristically grows into the inferior vena cava and extends to the right heart chambers. In spite of the notable extension of the tumour thrombus into the right atrium, surgical resection can result in prolonged survival albeit at an increased risk of postoperative mortality. The operative mortality of this procedure remains substantial, ranging from 2.7%-13% [2]. Accordingly, complete surgical resection of intracardiac leiomyomatosis carries a significant risk of postoperative mortality. However, surgical procedure remains the mainstay of therapy and can be curative. To date, a handful of reports, most of these isolated case reports and small clinical series, have described the management of intracardiac leiomyomatosis. However, it seems that Li et al. have overlooked two relevant papers. Thukkani et al. [3] report the case of a 36-year old woman with a history of recurrent pelvic leiomyomatosis and metastatic leiomyomatosis to the right heart chambers. The patient underwent a resection of the tumour with tricuspid valve replacement. A life-threatening bleed from the pelvic tumour was successfully managed at the end of the procedure. Zhang et al. [4] published their experience with intravenous leiomyomatosis with intracardiac extension in 5 women. Multiple approaches were described with excellent outcomes; the authors concluded that successful management requires timely recognition along with planed surgical intervention. Although there is a consensus about surgical resection of intracardiac leiomyomatosis because of the risk of sudden death, there are no guidelines for the treatment of pulmonary leiomyomatosis. Some advocated a surgical excision of the pulmonary nodule as a first-line strategy with or without hormone therapy. Others have shown that a wait-and-see strategy is an acceptable option in high-risk patients or in case of unrespectable tumour [5]. Conflict of interest: none declared.

Detection of intravenous leiomyomatosis with intracardiac extension by ultrasonography: A case report

Intravenous leiomyomatosis (IVL) is characterized by histologically benign tumors that exhibit aggressive clinical behavior. On rare occasions, the tumors may extend into the regional and systemic veins, thus reaching the heart. This may subsequently cause intracardiac leiomyomatosis (ICL), which may lead to congestive heart failure and occasionally, sudden fatalities. Due to its rarity and diffuse symptoms, the misdiagnosis of ICL is common and as a result, the condition may be under-reported. The present study reports a 33-year-old female who was admitted to Shandong Provincial Hospital Affiliated to Shandong University for myomectomy due to a rapidly growing myoma of the uterus. In routine pre-operative abdominal ultrasonography, a moderately sized echoic mass in the right internal iliac vein was observed, which extended to the common iliac vein, the inferior vena cava and the orifice of the right atrium. A presumptive diagnosis of ICL was made. The patient underwent a well-prepared one-stage thoraco-abdominal surgical procedure and the pathological report confirmed ICL. This case illustrates that the early detection of ICL may prevent a potential emergency situation and abdominal ultrasonography may be considered a useful tool in the diagnosis of ICL.

Intracardiac leiomyomatosis: a comprehensive analysis of 194 cases

Intracardiac leiomyomatosis is rare but has been increasingly reported in recent years. Owing to its rarity, intracardiac leiomyomatosis has been reported only as isolated case reports and case series. This disorder is thought to be underestimated and easily overlooked in the clinic, while it is dangerous owing to the risk of sudden death caused by total outflow tract obstruction. We performed an electronic literature search for intracardiac leiomyomatosis and identified 194 cases that were reported in English from 1974 (the first reported case) to September 2012. Our aim is to provide a detailed and comprehensive review of the clinical presentation, diagnosis, histopathological characterization, treatment and prognosis of this disorder. According to our analysis, intracardiac leiomyomatosis is most common in the fifth decade, and the mean age of detection is ~50 years. Most patients had undergone previous hysterectomy/myomectomy or had a coexisting uterine leiomyoma when admitted. The most common clinical presentations were dyspnoea, syncope, oedema of the lower extremities and palpitation. Transoesophageal echocardiography, computed tomography and magnetic resonance imaging are helpful in the preoperative diagnosis and to guide the surgical management. Complete removal guarantees an excellent outcome, with no recurrence or postoperative death, while incomplete removal leads to recurrence in one-third of patients. Anti-oestrogen therapy is not imperative after incomplete removal owing to its inability to prevent recurrence.

Multimodality treatment of two cases of intracardiac leiomyomatosis with enormous mass in the abdominopelvic cavity

Difficulties in the preoperative preparation and surgical management of patients with intravenous leiomyomatosis involving the heart and pulmonary artery resulting in intracardiac leiomyomatosis necessitate further investigation of this condition. In this report, we describe the interdisciplinary treatment of two cases of intracardiac leiomyomatosis with enormous mass in the abdominopelvic cavity. A single-stage surgical procedure with a combined thoracic and abdominal approach was performed in both cases and the tumors were successfully excised. The procedure also involved removal of the atrial, caval and other venular extensions, and total hysterectomy with bilateral salpingo-oophorectomy. Postoperative pathology findings confirmed the diagnosis of intravenous leiomyomatosis. Neither patient died perioperatively, and no complications were observed.