Intracardiac leiomyomatosis: a comprehensive analysis of 194 cases

Abstract

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.

Inferior Vena Cava Syndrome of uncommon aetiology

“…There have been reports of recurrences 39 months after surgery [3], associated with the incomplete resection of the tumour [11].…”

Multiservice single-stage surgery for intracardiac leiomyomatosis

“…[7][8][9] The tumor travels from the uterine wall into the IVC via extension through the iliac vein (66.2%), ovarian vein (17.6%), or renal vein (14.2%). [4] With surgical treatment, survival is extremely high with reports suggesting more than 12 years without complications. [10] Recurrence from complete resection has never been documented while incomplete resections have a 33% recurrence rate.…”

“…However, it may also be found incidentally in an asymptomatic patient. [4,5] Intracardiac leiomyomatosis requires surgical intervention, with the first successful operative resection reported in 1980; all previous cases were fatal. [6] This condition can be treated via two-stage procedure with a sternotomy first for intracardiac tumor removal followed by a laparotomy for complete tumor excision, or via a single-stage operation with a sternotomy and laparotomy approach under cardiopulmonary bypass.…”

“…[6] This condition can be treated via two-stage procedure with a sternotomy first for intracardiac tumor removal followed by a laparotomy for complete tumor excision, or via a single-stage operation with a sternotomy and laparotomy approach under cardiopulmonary bypass. [2][3][4][5] The following case report employed a single-staged surgery with the involvement of Cardiothoracic, GynecologyOncology, Urological, and Vascular Surgery services.…”

Uterine Intravenous Leiomyomatosis with Right Ventricular Extension

“…1,2) As intracardiac leiomyomatosis may result in cardiac symptoms and may cause sudden death, prompt and adequate surgical intervention is mandatory. In the present report, we describe a case of uterine intravenous leiomyomatosis with cardiac extension that was successfully treated with a single-stage operation.…”