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Updated: Feb 13, 2026

Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Surgical treatment for intravenous-cardiac leiomyomatosis
Jianzhou Liu1, Mei Liang1, Guotao Ma1
1Department of Cardiac Surgery, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences, Beijing, China.
Objectives:
There are few published studies on the rare disorder of intravenous-cardiac leiomyomatosis (IVCL). This study aimed to propose an individualized strategy for surgical treatment of IVCL.
Methods:
In this retrospective study, we reviewed 50 patients who had undergone IVCL removal from November 2002 to October 2017 in our hospital. IVCL was classified as Type A-E according to the extent and size, with Type E being the most severe. Clinical manifestations, surgical features and follow-up data were analysed.
Results:
Of the 50 patients in this series, 8 had Type A IVCL, 8 Type B, 29 Type C, 2 Type D and 3 Type E IVCL. One-stage removal of IVCL was performed via laparotomy without cardiopulmonary bypass (CPB) in the 8 patients with Type A, 1-stage tumour resection via sternolaparotomy under deep hypothermic arrest in 7 of the 8 patients with Type B and IVCL removal via sternolaparotomy under CPB, with 27 also under deep hypothermic arrest, in all 29 patients with Type C. Sixteen of the patients with Type C IVCL underwent staged procedures, 13 a 1-stage procedure and 21 required hepatic mobilization. All patients with Type C or E cases underwent 1-stage tumour removal via sternolaparotomy under deep hypothermic arrest. All 50 patients survived surgery. IVCL was confirmed postoperatively by histology. Ten patients had residual tumours; 9 of which did not progress. No deaths occurred during 47.8 ± 38.4 (range 1-177) months of follow-up.
Conclusions:
The only known curative treatment for IVCL is surgery. Herein, we present an individualized strategy for selecting surgical treatment.
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