Received: 23 September 2017
Accepted: 14 October 2017
Prognostic models for RPS patients—Attempting to predict
Dario Callegaro MD
Rosalba Miceli PhD
Rebecca A. Gladdy MD, PhD
Department of Surgery, Fondazione IRCCS
Istituto Nazionale Tumori, Milan, Italy
Department of Clinical Epidemiology and
Trial Organisation, Fondazione IRCCS Istituto
Nazionale Tumori, Milan, Italy
Department of Surgery, Mount Sinai
Hospital, University of Toronto, Toronto,
Dario Callegaro, MD, Department of Surgery,
Fondazione IRCCS Istituto Nazionale Tumori,
via Venezian 1, 20133 Milan, Italy.
Retroperitoneal sarcoma (RPS) patients can have variable of outcomes after surgery.
The chance to recur locally or at distant sites varies according to tumor grade, histologic
subtype, and other patient- and tumor-related characteristics. The relative contribu-
tion of each prognostic variable on the oncological outcome of RPS patients can be
weighted by combining them in prognostic tools such as nomograms. With this review,
we critically appraise the available nomograms for RPS patients highlighting pros and
nomogram, prognosis, retroperitoneal sarcoma, sarcoma
Prognosis prediction in RPS patients
Retroperitoneal sarcomas (RPS) are a heterogeneous group of tumors
with a wide prognostic range.
Contrary to other sarcoma sites such as
the extremities where outcome is primarily limited by distant failure,
local control is the central challenge in RPS patients. The main tumor-
related prognostic factors in RPS have historically been pathologic
grade, size, histologic subtype, and multifocality. Also patient
characteristics such as age at presentation and treatment variables
such as completeness of resection, tumor rupture, and center expertise
exert a strong influence on patient prognosis.
contribution of each prognostic factor on the oncological outcome
is different and only considering the simultaneous effect of the
combination of each variable can the physician attempt to define the
clinical course of retroperitoneal sarcoma.
Although the 10-year overall survival (OS) of primary resected
RPS patients is about 46% the natural history of these tumors varies
widely according to the histological subtype.
favorable histologies include well-differentiated liposarcoma
(WDLPS) and solitary fibrous tumor (SFT), as the 7-year OS is
greater than 80% in these subtypes.
SFT are generally cured with
adequate surgical resection, although a minority (less than 10%),
have more aggressive behavior with metastatic potential. WDLPS in
contrast have limited early local recurrence but require long-term
follow up as they can recur locally decades after primary resection.
High-risk RPS include: dedifferentiated liposarcoma (DDLPS),
leiomyosarcoma (LMS), and malignant peripheral nerve sheath tumor
(MPNST). In DDLPS, the higher the grade of the dedifferentiated
component, the higher the risk to recur. For example, intermediate-
grade DDLPS have a higher tendency to recur locally while high-
grade DDLPS have an increased risk of metastasis, mainly to the
lung. The 7-year OS of high-grade DDLPS is as low as 30%.
the retroperitoneum are typically intermediate-to-high grade tumors
arising from major veins (ie, inferior vena cava, renal vein, gonadal
vein). Overall, LMS have a high metastatic incidence (50%), while the
risk of local recurrence is low, (crude cumulative incidence of local
recurrence about 10% at 5 years in recent series).
peripheral nerve sheath tumors (MPNST) tend to behave aggres-
sively with a high-risk of early local and distant spread of the
Other histologies in the retroperitoneum are rarely seen.
With such different biological behavior, the personalization of
patient prognosis is critical to aid in clinical decision-making and
patient counseling. Although surgery is the mainstay of treatment of
RPS, the extent of surgical resection, and the use of neoadjuvant/
adjuvant chemotherapy (CT) or radiotherapy (RT) need to be tailored to
the individual's risk. Therefore, tools such as nomograms to predict
prognosis of patients with RPS can guide the physician and the patient
to define a shared therapeutic strategy.
J Surg Oncol. 2018;117:69–78. wileyonlinelibrary.com/journal/jso © 2017 Wiley Periodicals, Inc.