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.
References
- 1.Li B, Chen X, Chu Y-D, Li R-Y, Li W-D, Ni Y-M. Intracardiac leiomyomatosis: a comprehensive analysis of 194 cases. Interact CardioVasc Thorac Surg 2013;17:132–9 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Kalkat MS, Abedin A, Rooney S, Doherty A, Faroqui M, Wallace M, et al. Renal tumours with cavo-atrial extension: surgical management and outcome. Interact CardioVasc Thorac Surg 2008;7:981–5 [DOI] [PubMed] [Google Scholar]
- 3.Thukkani N, Ravichandran PS, Das A, Slater MS. Leiomyomatosis metastatic to the tricuspid valve complicated by pelvic hemorrhage. Ann Thorac Surg 2005;79:707–9 [DOI] [PubMed] [Google Scholar]
- 4.Zhang C, Miao Q, Liu X, Zhang H, Ma G, Chen G, et al. Intravenous leiomyomatosis with intracardiac extension. Ann Thorac Surg 2010;89:1641–3 [DOI] [PubMed] [Google Scholar]
- 5.Hoetzenecker K, Ankersmit HJ, Aigner C, Lichtenauer M, Kreuzer S, Hacker S, et al. Consequences of a wait-and-see strategy for benign metastasizing leiomyomatosis of the lung. Ann Thorac Surg 2009;87:613–4 [DOI] [PubMed] [Google Scholar]