A wide midline abdominal incision was made extending from the xiphoid process down to the symphysis pubis. A self‐retaining retractor was put in place, exposing the retroperitoneal sarcoma. The medical student amazed, remarked: “Wow, that is a big tumor!” (Figure )Intraoperative photos of representative retroperitoneal sarcomasRetroperitoneal sarcomas (RPS) are among the largest tumors known in the human body. In addition to their large size, RPS are extremely rare, repressenting 0.2% of all solid tumors in adults. Importantly, RPS are very challenging to effectively treat. Surgery is the mainstay of treatment, however there is much more to the management of RPS than simply “getting it out.”The management of RPS begins with a fundamental understanding of the disease even before arriving in the operating room. The large size of these tumors, typically without evidence of distant metastatic disease at initial presentation, already speaks to their unique disease biology. RPS in fact, consist of several histologic subtypes (liposarcoma, leiomyosarcoma, and others) and recognition of this has critical implications to the approach to treatment and surveillance. Accurate pathologic diagnosis is important, however, even without a biopsy, hints of the subtype can already be made based preoperative imaging.Once in the operating room, “getting it
Journal of Surgical Oncology – Wiley
Published: Jan 1, 2018
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