Case Study-Rare Tumour

by Dr. Vishruth K Raj

A 32-year-old woman from Iran, a mother of five children, who presented with vague abdominal pain was evaluated in her country, found to have a large retroperitoneal mass suspected to be arising from the right kidney and was referred to our hospital for further management.

She was admitted at Aster RV Hospital in Bangalore, under the department of Urology and Uro-oncology and after review of the original imaging, an FDG PET CT Scan was done for metastatic work up. It revealed a huge, roughly 12cm tumor, in the space between the right kidney and the duodenum, infiltrating the infrahepatic and renal parts of the inferior vena cava, the whole of the right renal vein and the distal part of the left renal vein.

There was significant obstruction and hydronephrosis of the right kidney, with development of massive collateral vessels around both right and left kidney, as also severe engorgement of left gonadal and lumbar vessels. There were however no lymph nodal or other metastases. A DTPA scan was also done to evaluate the split renal function which revealed a moderately compromised right and normally functioning left kidney.

After discussion with intervention radiologist, a CT guided biopsy of the lesion was done. The histopathology and immunohistochemistry confirmed it to be a leiomyosarcoma and a probable diagnosis of Primary IVC Leiomyosarcoma was made.

An Iliac vessels doppler scan revealed no thrombus or stenosis. Keeping the rarity of the tumour (less than 400 cases such cases ever reported) in mind, a multi-disciplinary tumour board meeting was held in the presence of the patient’s attender, which included, It was eventually decided that radical excision surgery with multi-organ resection was the best therapeutic approach in this patient and she was optimized for the same.

Keeping the rarity of the tumour (less than 400 cases such cases ever reported) in mind, a multi-disciplinary tumour board meeting was held in the presence of the patient’s attender,It was eventually decided that radical excision surgery with multi-organ resection was the best therapeutic approach in this patient and she was optimised for the same.

Keeping a venous bypass ready, an exploratory laparotomy was performed via a midline incision. After mobilising the colon, the right kidney and the liver, supra-hepatic and distal IVC were looped. Tumour along with the involved parts of IVC, right kidney, second part of duodenum, right adrenal, the right and left renal veins and head of pancreas was resected en mass. The ends of the IVC and the stump of the left renal vein were sutured and a Whipple’s procedure done. The surgery took around 10 hours to complete and the blood loss was around 500 ml. Patient withstood the procedure and recovered well.

The post op Histopathology was reported as a high grade leiomyosarcoma originating from the IVC and infiltrating the right kidney and the duodenum. The other resected margins were all free of tumour. She received adjuvant chemotherapy, which she again tolerated well.

She flew back home more than a month after landing in India, and was reunited with her family and is now cancer free on follow up.