Objective

2

To investigate the diagnosis and treatment strategies for retroperitoneal vascular leiomyosarcoma which is a rare disease in clinical practice.

Methods

2

Related data were collected from 12 patients with retroperitoneal vascular leiomyosarcoma who were treated from July 2018 to April 2023，among whom there were 3 male patients and 9 female patients，with a mean age of 55.2 （32.0，69.0） years. The lesion was located in the right side for all patients，and clinical symptoms included abdominal discomfort，lower limb numbness，lower limb，and ascites. Among these patients，4 patients were misdiagnosed with adrenocortical carcinoma or paraganglioma，and 1 patient did not undergo surgery. Among the 11 patients undergoing surgery，4 underwent laparoscopy surgery，among whom 1 was converted to open surgery； 5 underwent open surgery； 2 underwent robot-assisted laparoscopy. All patients required the operation for the inferior vena cava（IVC） during surgery，including right renal vein repair by clamping the IVC in 1 patient，simple suture of the IVC in 3 patients，partial resection of the IVC wall and simple repair in 2 patients，biological patch repair of the IVC wall in 2 patients，transection and anastomosis of the IVC in 1 patient，and segmental resection of the IVC in 2 patients.

Results

2

The 12 patients had a mean tumor size of 10.6（4.2，22.0） cm，a mean time of operation of 268（151，467） minutes，and a mean blood loss of 715（20，1 550） mL. 8 patients received intraoperative blood transfusion，5 received right nephrectomy，1 had positive surgical margin，and 2 experienced delayed retroperitoneal hemorrhage after surgery. It was confirmed that 1 patient had leiomyosarcoma originating from the adrenal vein，2 had leiomyosarcoma originating from the renal vein，and 9 had leiomyosarcoma originating from the IVC. Tumor thrombus in the IVC was observed in 6 patients. Pathological examination showed that the tumor was composed of spindle cells，with mitotic figures observed. Immunohistochemistry showed positive SMA in all patients and positive Desmin in 8 patients，and the positive expression rate of Ki-67 was 20%-80%. The mean follow-up time was 21.6（1.0，54.0） months. Among these patients，3 died during follow-up，4 survived with postoperative recurrence，and 5 had no recurrence，among whom 3 were followed up for more than 2 years.

Conclusion

2

Retroperitoneal vascular leiomyosarcoma is easily misdiagnosed before surgery，and the operation for the IVC is required during surgery. The surgery is challenging，and surgical procedures should be used according to the actual situation. Open surgery is the preferred choice，and retroperitoneal laparoscopy should be selected with caution. The tumor is highly malignant with a high recurrence rate.