THE RARE CASE OF A LEIOMYOSARCOMA ORIGINATING FROM THE RIGHT GONADAL VEIN

Objectives: The most common site for leiomyosarcomas (LMS) to originate is within the endometrium of the uterine wall. LMS may also originate from blood vessels and, the most common site is the inferior vena cava and secondly the renal veins. There had been only ten cases, as of 2015, reported in literature that documented LMS with a gonadal vein origin and even fewer reporting involvement with the right gonadal vein.

Our patient is a 61-year-old female who had previously undergone a right-sided nephrectomy for renal cell carcinoma. During routine surveillance, she was noted to have a new abdominal mass to the right of the inferior vena cava and inferior to the right renal vein. This was not seen on imaging 5 months prior. Computed tomography of the mass described it as circular in appearance with dimensions of 62mm x 48mm x 46mm. Biopsy of the mass confirmed the diagnosis of a leiomyosarcoma. The decision was then made to proceed with surgical excision. There were no intraoperative findings of sarcomatosis or involvement of the inferior vena cava, however significant involvement of the right gonadal vein was noted requiring en bloc resection with the mass. Final pathology confirmed negative margins had been achieved and the diagnosis of right gonadal vein leiomyosarcoma was made. The patient had an uneventful post-operative course and has since been seen in follow up with no evidence of recurrence .

Due to the aggressive nature and retroperitoneal location, venous leiomyosarcomas pose a unique challenge for both diagnosis and treatment. This case is unique with the origin of the LMS being the gonadal vein. This has rarely been reported in the literature, but one publication reported venous leiomyosarcomas not involving the vena cava, such as this case, only hold a 32% four-year survival rate. Although case reports are rare, it is crucial to consider venous leiomyosarcomas in the differential diagnosis when evaluating retroperitoneal tumors due to their significant lethality. More reports are needed in the future to add to the body of literature surrounding this rare diagnosis.

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