Retroperitoneal sarcoma (RPS) is a rare disease, and until recently, its natural history and outcome were poorly understood. Recently, collaborations between individual centers have led to an unprecedented collection of retrospective and prospective data and successful recruitment to the first randomized trial as described here.
A debate about the beneficial role of extended surgery in RPS triggered an initial collaboration between Europe and North America, the TransAtlantic RetroPeritoneal Sarcoma Working Group (TARPSWG). This collaboration has been instrumental in harmonizing the surgical approach among expert centers, characterizing the pattern of postresection failure of the different histological subtypes, identifying new ways to stage RPS and testing the role of preoperative radiotherapy in a randomized fashion (STRASS-1 study). The collaboration has now expanded to include centers from Asia, Australia and South America. A prospective registry has been started and a new randomized trial, STRASS-2, is in preparation to analyze the role of neoadjuvant chemotherapy for high-grade liposarcoma and leiomyosarcoma of the retroperitoneum.
Collaboration is critical to study a rare disease like RPS. Both retrospective and prospective data are useful to improve knowledge, generate hypotheses and build evidence to test, whenever possible, in clinical trials.
aDepartment of Surgical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands
bDepartment of Surgery, Brigham and Women's Hospital, Dana-Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts, USA
cDepartment of Surgery, Institute Curie, Paris, France
dDepartment of Surgical Oncology, Princess Margaret Cancer Centre, and Department of Surgery, Mount Sinai Hospital, University of Toronto, Toronto, Canada
eDepartment of Radiotherapy, Netherlands Cancer Institute, Amsterdam, and the LUMC, Leiden, the Netherlands
fDepartment of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy
Correspondence to Alessandro Gronchi, MD, Chair Sarcoma Service, Department of Surgery, Fondazione IRCCS Istituto Nazionale dei Tumori, Via Venezian 1, 20133 Milan, Italy. Tel: +39 223903234; e-mail: email@example.com