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Soft Tissue Sarcoma Peri-operative Therapies for Localized Retroperitoneal Soft Tissue Sarcoma Stefano Radaelli, MD, 1 Anant Desai, MD, 2 Chiara Colombo, MD, 3 Marco Fiore, MD 3 and Alessandro Gronchi, MD 4 1. Fellow Surgeon, Department of Surgery, National Cancer Institute, Milan; 2. Attending Surgeon, Department of Surgery, National Cancer Institute, Milan and Queen Elizabeth Medical Centre, Birmingham; 3. Attending Surgeon, Department of Surgery, National Cancer Institute, Milan; 4. Chief, Sarcoma Service, Department of Surgery, National Cancer Institute, Milan Abstract Retroperitoneal soft tissue sarcomas are rare solid tumors that usually arise in the retroperitoneum or the pelvis. They are often large at presentation and push adjacent structures, displacing them, anatomically. The most common adult histotypes are liposarcoma and leiomyosarcoma. Five-year overall survival is less than 50 % and is mainly determined by the high incidence of local relapse and to a lesser extent by their capacity to metastasize. Radical surgery is still the most effective treatment. While an aggressive surgical policy consisting of multivisceral resections ‘en bloc’ with the mass has undoubtedly decreased, the rate of local recurrence, prognosis remains poor. Retrospective analyses and early prospective data suggest a benefit from using neoadjuvant radiotherapy in order to improve local control. Chemotherapy plays an important role in advanced disease, but very little evidence supports a real advantage in delivering it prior to surgery to make the resection more likely to achieve clear margins or postoperatively to decrease the risk of systemic recurrence. Further studies are required to explore the role of these neoadjuvant/adjuvant therapies, particularly in the context of new developments in molecular therapeutic agents. Keywords Sarcoma, retroperitoneal sarcoma, chemotherapy, surgery, radiotherapy, local control, survival Disclosure: The authors have no conflicts of interest to declare. Received: January 17, 2012 Accepted: January 31, 2012 Citation: Oncology & Hematology Review, 2012;8(1):65–70 Correspondence: Alessandro Gronchi, MD, Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale Tumori, Via Venezian 1, 20133 Milan, Italy. E: Retroperitoneal soft tissue sarcomas (RSTSs) are uncommon tumors composing less than 1 % of all adult cancers. They account for approximately 15  % of soft tissue sarcomas (STSs), i.e., the most frequent site of mesenchymal malignancy after the lower extremities. 1 Although many different sarcoma histotypes exist, the most common in the retroperitoneum are liposarcoma (41  %), leiomyosarcoma (28  %), pleomorphic sarcoma, solitary fibrous tumor, synovial sarcoma, and malignant peripheral nerve sheath tumor. Other subtypes, such as myofibroblastic inflammatory tumor, low-grade fibromyxoid sarcoma, and myxofibrosarcoma, have to be considered rarities. About 50  % of RSTSs are reported to be of high grade in terms of their biologic aggressiveness. 2 The retroperitoneum can also host a wide spectrum of other benign tumors, such as paragangliomas, angiomyolipomas, and schwannomas as well as primary epithelial malignancies from the kidney and the adrenal gland. It is also a common site for lymphomas and metastatic nodal lesions from germ cell neoplasms. The clinical presentation of an RSTS is that of a large mass in the right or left retroperitoneal space that is able to push and dislocate the surrounding viscera and vessels, without showing an infiltrative pattern of growth. Initially, these neoplasms are asymptomatic or give rise to few symptoms causing non-specific abdominal discomfort or pain, and diagnosis is often late. If found early, it is usually because the patient © TOUCH BRIEFINGS 2012 has had imaging for other indications and the mass is found incidentally. Over time, patients develop a palpable mass with a significant increase in volume and girth of the abdomen. The diagnostic approach generally starts with a contrast-enhanced abdominal and thoracic computed tomography scan. This is usually enough to achieve complete oncologic staging. Positron emission tomography, magnetic resonance imaging, and bone scans are seldom required. A pre-operative percutaneous core biopsy, where possible, should be performed. The histologic diagnosis, together with the radiologic findings and the patient’s performance status, should guide the multidisciplinary decision about the most appropriate therapeutic approach. An aggressive surgical policy, consisting of multiple visceral resection ‘en bloc’ with the mass, is still the most effective strategy to cure a primary retroperitoneal sarcoma. At the same time, a multidisciplinary approach, mostly based on pre-operative chemo-radiation therapy, is increasingly important in high-grade or unresectable RSTSs. 3 Surgery Surgery is the mainstay of treatment in localized disease. Local control is critical and largely depends on the quality of surgery. Anatomic constraints in the retroperitoneum limit the ability to achieve wide 65