Received: 26 September 2017
Accepted: 27 September 2017
Historical perspectives and future directions in the surgical
management of retroperitoneal sarcoma
Hyun Jae Seo BA
Raphael E. Pollock MD, PhD
Alessandro Gronchi MD
Department of Surgery, Section of Surgical
Oncology, Keck School of Medicine,
University of Southern California, Los Angeles,
Department of Surgery, The James
Comprehensive Cancer Center, Ohio State
University, Columbus, Ohio
Department of Surgery, Fondazione IRCCS
Istituto Nazionale dei Tumori, Milano, Italy
William W. Tseng, MD, Department of
Surgery, Section of Surgical Oncology, Keck
School of Medicine, University of Southern
California, 1510 San Pablo Street, Suite 514
Los Angeles, CA 90033.
Retroperitoneal sarcomas (RPS) have fascinated and intrigued physicians both past and
present. Operative mortality rates were historically very high and complete resection
was not possible for the majority of patients until only the last 2 decades. More
recently, changes to the surgical approach and clinical decision-making in RPS have
improved patient outcomes. With select integration of nonsurgical therapies,
continued RPS-specific research, and ongoing collaborative efforts among major
referral centers, the future appears promising.
history, liposarcoma, retroperitoneal sarcoma, sarcoma
of these tumors arise in the retroperitoneum. Retroperitoneal sarcomas
(RPS) are therefore extremely rare malignancies (0.15-0.2%).
multitude of histologic subtypes exist for sarcomas in other parts of
the body, for RPS the predominant subtypes are liposarcoma (well
differentiated and dedifferentiated), followed by leiomyosarcoma. Other,
subtypes (eg, solitary fibrous tumor, malignant peripheral nerve sheath
tumor) are also occasionally found in the retroperitoneum. Given the deep
body location, most RPS can reach large tumor sizes before detection and
in fact, RPS are among the largest tumors known in the human body. Due
to the rarity and dramatic presentation, RPS have fascinated physicians
both past and present. Surgery, although often challenging, has always
been recognized as the mainstay of treatment and remains so today. This
article will review the history and evolution of RPS surgery and conclude
with future directions in the management of this unique disease.
These are indeed large tumors—can we treat
them with surgery?
In Western medical literature, the first anatomic description of a large
tumor in the retroperitoneum is credited to the Italian anatomist,
Giovanni Battista Morgagni. In 1761, in his work entitled De Sedibus et
causis morborum per anatomem indagatis (“Of the seats and causes of
diseases investigated through anatomy”), Morgagni reported the
presence of a large lipomatous tumor in the back of the abdomen—
likely a retroperitoneal liposarcoma—found during an autopsy
performed on a 60 year old woman (Figure 1).
In 1829, Lobstein
gave a more comprehensive description of retroperitoneal “growths”
including probably the first use of the term “sarcoma” for tumors of this
In the ensuing decades, other individual cases and small case series
of RPS were sporadically presented at surgical meetings and reported
in the medical literature. In general, the focus of these early
descriptions was to discuss presenting symptoms and findings at the
time of autopsy or, for a minority of patients, surgery. By 1900, J
Dutton Steele published A Critical Summary of the Literature on
Retroperitoneal Sarcoma in which he found 61 reported cases in total.
Sarcomas were recognized as malignancies of the “flesh,” but
interestingly, Steele postulated that these retroperitoneal tumors
originated in the “lymph glands.”
In 1903, Howard Williams reported
his own experience with four RPS cases and performed his own review
of the literature to find 84 cases in total from across the world,
including reports from Paris, Pittsburgh, Buenos Ares, Australia, and
others. Among these cases, only 12 operations (14%) were described.
J Surg Oncol. 2018;117:7–11. wileyonlinelibrary.com/journal/jso © 2017 Wiley Periodicals, Inc.