OBJECTIVETo study the treatment strategy and survival of patients with primary leiomyosarcoma of inferior vena cava (PIVCLMS).

METHODSClinical data of 12 cases with PIVCLMS admitted in Peking University People's Hospital from January 2006 to September 2014 were reviewed retrospectively. All cases were confirmed by pathology examination. Among them, there were 4 male and 8 female patients with a mean age of (54 ± 9) years old. Tumors arose from the inferior vena cava (IVC) upper segment in 5 patients, from the middle in other 7 patients. Cardiac extension was observed in 4 cases. Tumor resection was undertaken in 8 patients, the other 4 patients were inoperable. The series was analyzed to identify clinical outcome of surgical strategy and protective factors for patient survival.

RESULTSIn tumor resection group, 6 patients had radical resection and 2 underwent palliative resection. As for IVC reconstruction, caval wall resection with a direct suture was carried out in 6 patients or with prosthetic patch in 1 patient. The other 1 patient underwent a segment caval resection and prosthetic graft replacement in situ. In 4 cases of suprahepatic PIVCLMS cardiopulmonary bypass or perfusion by right atrial intubation was performed to assist bleeding control and maintain circulation stabilization, among them 1 patient survived for more than 101 months with no tumor recurrence or metastasis. Among the patients submitted to tumor resection 2 early postoperative deaths occurred, and another 2 patients had complications. All 4 patients submitted to non-resective operation (only neoplasm biopsy) died of PIVCLMS within 8 months. Except for 2 cases of early death, mean survival after tumor resection was (54 ± 40) months. Two patients presented local recurrence and hepatic metastasis at follow-up of 16 months and 68 months.

CONCLUSIONSTumor resection is the only therapy for PIVCLMS with an expectation for long-term survival. The applicant of cardiopulmonary bypass makes some inoperable indicated to tumor resection.