Intracardiac leiomyomatosis: a comprehensive analysis of 194 cases

Bin Li 0 Xin Chen 0 Ya-Dong Chu 0 Ren-Yuan Li 0 Wei-Dong Li 0 Yi-Ming Ni 0 0 Department of Thoracic and Cardiovascular Surgery, First Affiliated Hospital, Zhejiang University School of Medicine , Hangzhou, China 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. Antioestrogen therapy is not imperative after incomplete removal owing to its inability to prevent recurrence. - Metastatic cardiac tumours are relatively common; however, intravenous tumour extension into the heart is very infrequent. Intracardiac leiomyomatosis (ICLM), a condition rarely seen, is characterized by histologically benign smooth muscle tumours of extracardiac origin reaching the right heart by direct extension into the venous channel. Owing to its rarity, the accumulated knowledge of ICLM has derived mainly from isolated case reports and case series. This tumour has been suggested to originate either from a uterine leiomyoma with vascular invasion or from the venous smooth muscle wall itself. When extending beyond the inferior vena cava (IVC), this intravenous tumour can reach as far as the right atrium (RA), the right ventricle (RV) or even the pulmonary arteries (PAs). Most patients are middle-aged women. Similar to other intracardiac tumours, the clinical presentation of ICLM is non-specific. The advent of echocardiography, computed tomography (CT) and magnetic resonance imaging (MRI) has allowed early and accurate evaluation of this lesion, while the final diagnosis must be made by postoperative pathological examination. Although histologically benign, ICLM is thought to be clinically aggressive owing to the risk of sudden death caused by total outflow tract obstruction. MATERIALS AND METHODS We performed an electronic literature search for ICLM in MEDLINE/PubMed, Web of Science and Cochrane Library. The search terms intravenous leiomyoma, intravenous leiomyomatosis, intracardiac leiomyoma, intracardiac leiomyomatosis, intravascular leiomyoma, intravascular leiomyomatosis cardiac extension, heart extension and pulmonary extension were used to identify case reports and case series published in English. Cases with intravascular mass extension into the right heart and confirmed by histopathological examination as leiomyoma were included. All the references cited in each publication were evaluated for additional eligible cases. We found 163 articles with cases that met the characterization of ICLM (see Supplementary material). Of these, any patient reported more than once in different articles was treated as one case, and the medical information was integrated into the analysis. Finally, we identified 194 cases of ICLM that were reported in English up to September 2012. Adhesion was defined as the attachment of tumour to the vascular wall, and it may lead to blood vessel rupture or even fatal haemorrhage when the tumour is removed. Complete removal was confirmed by the surgeon during the operation or by postoperative imaging examination. Unless otherwise specified, incomplete removal mentioned here includes partial excision of the tumour and non-surgical treatment (in patients who refused surgery), because both of these left residual tumour in the patient. Recurrence was defined as the postoperative regrowth of the tumour detected by imaging examination. The recurrent and survival data were analysed with the standard KaplanMeier actuarial technique. A two-tailed probability value of <0.01 was considered to indicate statistical significance. All the data in our The Author 2013. Published by Oxford University Press on behalf of the European Association for Cardio-Thoracic Surgery. All rights reserved. analysis, including demographics, clinical presentation, histopathological characterization, treatment and prognosis, were extracted from these 194 cases using Excel 2007 (Microsoft Corp., Redmond, WA, USA) and Prism 5.0 (Graphpad Software, La Jolla, CA, USA). According to our collection of 194 patients, ICLM is seen exclusively in women. Recently, this disorder has been reported with increasing frequency, but it is certain that cases still go unreported. Half of the cases were reported within the last 10 years. Age prevalence was accurately available in 187 patients (Fig. 1), giving an average 47.6 years (range: 2081 years). Reproductive history was not mentioned in 154 patients (79.4%), and of the remainder, 10 of 40 patients (25.0%) were nulliparous and 30 of 40 (75.0%) were multiparous. One hundred and four patients (53.6%) had undergone a previous hysterectomy/myomectomy, and 58 (29.9%) had a coexisting uterine leiomyoma on admission. Only four patients (2.1%) were reported with a normal uterus. The time between the hysterectomy/myomectomy and detection of ICLM was available for 84 patients, giving an average of 87.8 months (range: 3 months to 47 years). The clinical presentation of ICLM varied (n = 177 patients) and is shown in Table 1. On auscultation, a heart murmur was heard in 65 patients (33.5%; 54 systolic, six diastolic and five unidentified). Tumour plop was noted in 11 patients (5.7%) and pericardial rub in only two (1.0%). Chest roentgenogram was mentioned for 51 patients, and the results were normal in 30 (58.8%) and showed heart enlargement in 21 (41.2%). Electrocardiographic results, which were available in 54 patients, were normal in 22 (40.7%), and showed a low voltage in 13 (24.1%), non-specific STT change in 11 (20.4%), sinus tachycardia in seven (13.0%) and arrhythmia in four (7.4%). In 148 cases, the route of tumour extension was described and is shown in Table 1. Distant locations were recorded in 172 pa (...truncated)