Surgical Management of Retroperitoneal Sarcoma

Abstract

Surgery is the cornerstone of treatment for retroperitoneal sarcoma (RPS). Surgery should be performed by a surgical oncologist with sub-specialization in this disease and in the context of a multidisciplinary team of sarcoma specialists. For primary RPS, the goal of surgery is to achieve the complete en bloc resection of the tumor along with involved organs and structures to maximize the clearance of the disease. The extent of resection also needs to consider the risk of complications. Unfortunately, the overarching challenge in primary RPS treatment is that even with optimal surgery, tumor recurrence occurs frequently. The pattern of recurrence after surgery (e.g., local versus distant) is strongly associated with the specific histologic type of RPS. Radiation and systemic therapy may improve outcomes in RPS and there is emerging data studying the benefit of non-surgical treatments in primary disease. Topics in need of further investigation include criteria for unresectability and management of locally recurrent disease. Moving forward, global collaboration among RPS specialists will be key for continuing to advance our understanding of this disease and find more effective treatments.

Keywords: collaboration; leiomyosarcoma; liposarcoma; multidisciplinary; retroperitoneal sarcoma.

Figure 1

Case example of retroperitoneal liposarcoma. (a,b) Axial and sagittal CT scan images showing likely high-grade or dedifferentiated (DD) and low-grade or well differentiated (WD) components of the tumor, abutting the right kidney (K); (c) intraoperative photograph after resection showing original area of tumor (blue dotted shape) with preserved right kidney (K), duodenum and head of pancreas (*). Organ-preservation is considered on an individual case basis; (d) gross resection specimen demonstrating one intact tumor with WD, DD components and some incorporated, normal-appearing adjacent fat; (e) sectioned gross tumor showing in this case, clear demarcation of WD and DD; (f) photomicrograph (40×) of tumor histology showing clear demarcation of WD and DD, inset showing MDM2 amplification by fluorescence in situ hybridization, confirming the diagnosis. This patient did not receive neoadjuvant or adjuvant therapy.

Figure 2

Case example of retroperitoneal leiomyosarcoma. (a) Coronal CT scan image showing large tumor extending into the pelvis with intraluminal tumor extension in the inferior vena cava; (b) intraoperative photograph prior to resection showing gross involvement of the right colon (Col) and intracaval tumor component (white circle). The right kidney was also involved; (c) intraoperative photograph after resection showing original area of tumor (blue dotted shape) with in-line IVC graft reconstruction performed by vascular surgery; (d) gross resection specimen; (e) photomicrograph (40×) of tumor histology, inset (200×). This patient received neoadjuvant systemic therapy with good response radiologically, clinically and pathologically.