Retroperitoneal sarcomas present a therapeutic challenge based on their location, extent of invasion at diagnosis, and propensity for local recurrence. Surgical therapy remains the only potentially curative treatment option; however, even with aggressive surgical approaches, local recurrence remains a common type of failure.

A 75 year old Caucasian female was admitted to our Department in January 2006. Presenting symptoms were abdominal pain due to a bulky tumour located in the left hypochondrium and extending in the left flank. CT scan confirmed the presence of a huge retroperitoneal tumour infiltrating the left kidney, splenic flexure and descending colon, body and tail of the pancreas and the firs jejunal loop.

An exploratory laparotomy performed the 25/01/2006 confirmed the feasibility of a multiorgan resection. En bloc R0 resection involving left kidney, body and tail of the pancreas, spleen, first jeunal loop and fourth duodenum, left colon and intercavoaortic lymphectomy was accomplished. A latero lateral hand made duodenal jejunal anstomosis and a latero lateral colocolic mechanical anstomosis were realized. Intervention lasted 6 hours, blood loss was 600 cc, and diet was started on the 5th p.o. day. No complications were observed and the patient was discharges on the 20th p.o. day. Histology revealed a T4N0M0 (stage IV) left kidney sarcoma. Patients died of pulmonary metastases 18 months later.

Complete surgical excision offers patients with retroperitoneal sarcomas the best chance for long-term survival but recurrent disease and distal metastases remains a vexing problem.