Received: 22 September 2017
Accepted: 21 October 2017
Current principles of surgery for retroperitoneal sarcomas
Ricardo J. Gonzalez
Chandrajit P. Raut
Department of Surgery, Brigham and
Women's Hospital, Boston, Massachusetts
Center for Sarcoma and Bone Oncology,
Dana-Farber Cancer Institute; Harvard
Medical School, Boston, Massachusetts
Sarcoma Department, Moffitt Cancer Center,
Department of Surgery, Royal Marsden
Hospital, NHS Foundation Trust, London, UK
Chandrajit P. Raut, MD, MSc, Department of
Surgery, Brigham and Women's Hospital, 75
Francis Street, Boston, MA 02115.
Surgery for primary retroperitoneal sarcomas (RPS) often requires a technically
challenging, en bloc multivisceral resection to optimize outcomes. Surgery may also be
appropriate for patients with localized recurrent RPS. Anatomic considerations and
tumor biology driven by histologic subtype may guide the extent of resection in
patients with RPS. This review provides an overview of the current surgical principles
for primary and recurrent RPS.
histology, leiomyosarcoma, liposarcoma, organ resection, surgery
Soft tissue sarcomas (STS) are rare malignant tumors that most
commonly arise from cells of mesenchymal origin and can arise in any
region of the body. With over 70 different major histologic subtypes and
an estimated 12 000 new diagnoses expected in 2017 in the United
States, the heterogeneity and rarity of these tumors has made
development of evidence-based guidelines difficult to establish.
STS of the extremity are most common, approximately 15-20% arise in
The primary location of STS is a significant
prognostic factor, with tumors of the retroperitoneum having a worse
prognosis than those of the trunk or extremity.
This is likely due to a
combination of factors, including the different histologic distribution of
retroperitoneal sarcomas (RPS), their typically largersize at presentation
with multi-organ involvement, and their close proximity to critical
structures. RPS may grow insidiously and reach a large size thus
presenting rather late as a palpable abdominal mass or alternatively may
be discovered during workup of non-specific abdominal symptoms.
Computed tomography (CT) imaging of the abdomen and pelvis is the
most useful initial imaging modality to evaluate the extent of adjacent
organ involvement and to rule out intra-abdominal metastatic disease.
Biopsy to confirm diagnosis is often warranted, providing the clinician
with critical information regarding the biologic behavior based on
histologic subtype and confirming a diagnosis in case preoperative
radiation therapy is considered.
Surgery involving en bloc resection of the tumor and involved
adjacent structures remains the standard of care for patients with
localized RPS. A macroscopically complete resection (R0/R1 resection)
improves survival, but local recurrence (LR) rates are high.
close proximity of vital structures in the retroperitoneum demands a
careful balance of achieving local control by oncologic resection and
preservation of critical structures.
Tumor biology varies significantly among the multiple RPS
histologic subtypes, not only with regard to recurrence patterns and
survival outcomes, but also in their ability to infiltrate adjacent
The optimal extent of resection is unclear and continues
to be a topic of debate. While resection has been historically limited to
adjacent organs with direct tumor involvement, recently retrospective
multicenter registries of a more aggressive approach involving a
compartmental resection of adjacent organs (with or without direct
involvement), has been advocated in order to minimize microscopically
positive margins and lower LR rates.
In the absence of a
randomized trial demonstrating a survival benefit, though, this
approach remains controversial. An alternative, perhaps more nuanced
approach is one driven by anatomical and histologic-specific
considerations. Histologic subtype influences not only patterns or
local/distant failure and survival outcomes but also a tumor's
propensity to invade adjacent structures. These critical elements of
tumor biology should guide the surgical approach to RPS. This review
focuses on the principles of surgery for primary and recurrent RPS with
J Surg Oncol. 2018;117:33–41. wileyonlinelibrary.com/journal/jso © 2018 Wiley Periodicals, Inc.