Received: 22 September 2017
Accepted: 31 October 2017
Multiply recurrent retroperitoneal liposarcoma
Sanjay P. Bagaria MD
Emmanuel Gabriel MD
Gary N. Mann MD
Department of Surgery, Mayo Clinic,
Arnot Health, Department of General and
Oncologic Surgery, Elmira, NY
Sanjay P. Bagaria, MD, Associate Professor of
Surgery, Mayo Clinic, Jacksonville, FL 32224.
Retroperitoneal liposarcomas (RPLPS) are rare tumors that represent at least 50% of all
retroperitoneal sarcomas. Surgical resection remains the standard of care. Unfortu-
nately, many RPLPS patients will develop a local recurrence and subsequently die in the
absence of distant metastasis. This review outlines the factors that predict local
recurrence and influence the management of first and subsequent multiply recurrent
liposarcoma, local recurrence, overall survival, retroperitoneal sarcoma
Retroperitoneal sarcoma accounts for 15% of all sarcomas and
liposarcoma represents approximately 50% of these rare malignan-
Complete surgical resection remains the most effective
therapeutic modality. Despite optimal extirpation of the tumor, local
recurrence of retroperitoneal sarcoma (RPLPS) is a common clinical
problem and is the most common cause of mortality, with up to 70% of
deaths occurring in the absence of distant metastasis.
An on-going problem following resection of the first local
recurrence is the management of multiple recurrences. Multiply
locally recurrent RPLPS can occur in up to 69–80% of first recurrent
cases and constitutes a challenging management problem for a
The extent and optimal timing of resection and role of
systemic and radiation therapy are not entirely clear. Therefore, there
is a critical need to understand the natural history and current
management of multiply recurrent RPLPS. This review article will
summarize predictors of recurrent (first and multiple) RPLPS and will
highlight the important concepts in the management of these difficult
to treat tumors.
FACTORS INFLUENCING RATES AND
PATTERN OF FAILURE
The factors that influence the rates and pattern of failure of all RPS
histologic subtypes can be attributed to patient characteristics, tumor
features, and surgical variables; however, few studies specifically
Even fewer studies address which of these
factors are associated with multiple recurrent disease.
section, we will review the key factors that impact first and subsequent
RPLPS local recurrences.
Histologic subtype and RPLPS recurrence
Biological features of the liposarcoma can determine long-term patient
A key biologic feature is histologic subtype. The
World Health Organization classification of liposarcoma includes four
recognized histologic types: well-differentiated, dedifferentiated,
myxoid, and pleomorphic.
Each type is associated with unique
genetic mutations and histologic morphologies, which ultimately
impact their behavior.
In a retrospective review of 177 patients
with primary RPLPS, 56% presented with well-differentiated, 37%
with dedifferentiated, and 7% with myxoid neoplasms.
will focus on well-differentiated and dedifferentiated liposarcoma.
Well-differentiated liposarcoma (WDLPS) is a locally aggressive
malignancy that rarely metastasizes. WDLPS is characterized by
repeated local recurrences where the reported local recurrence rate
ranges from 40% to 60%.
Since WDLPS do not metastasize,
disease-specific mortality is secondary to uncontrolled local disease.
The 5-year overall survival (OS) rates for WDLPS have been reported
to be approximately 80%.
DDLPS, on the other hand, is both locally
aggressive and has distant metastatic potential. The local recurrence
and distant metastasis rates range from 40% to 80% and 15% to 20%,
The most common site for distant metastasis is
© 2017 Wiley Periodicals, Inc. wileyonlinelibrary.com/journal/jso J Surg Oncol. 2018;117:62–68.