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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
Retroperitoneal sarcomas grow silently at the back of the abdomen until they are the size of a melon. The treatment is one carefully planned operation in a sarcoma centre that removes the tumour with the neighbouring organs it touches. The STRASS trial showed radiotherapy before surgery does not help overall; a second trial is testing chemotherapy before surgery in the most aggressive types.
Retroperitoneal sarcomas are a site-defined group: well-differentiated and dedifferentiated liposarcoma make up about two thirds, leiomyosarcoma about a fifth, and solitary fibrous tumour, MPNST and undifferentiated pleomorphic sarcoma most of the rest. They present late as huge painless masses that displace the kidney, colon and pancreas, and the dominant pattern of failure differs by histology: liposarcoma recurs locally in the abdomen, often repeatedly and years later, while leiomyosarcoma metastasises to the liver and lungs. The Transatlantic Australasian Retroperitoneal Sarcoma Working Group consensus documents define the modern approach.
Complete en bloc resection at first presentation, removing adjacent organs such as kidney, colon and psoas to secure a margin, is the only curative treatment and has been associated with better local control in reference centres since the reports of Gronchi and Bonvalot in 2009. Adjuvant therapies were tested in the EORTC STRASS trial, which randomised 266 patients to preoperative radiotherapy plus surgery or surgery alone and found no improvement in abdominal recurrence-free survival overall, though an unplanned analysis suggested benefit in well-differentiated and low-grade dedifferentiated liposarcoma. Preoperative radiotherapy is therefore not routine and is discussed case by case for liposarcoma.
| Setting | Approach | Guideline |
|---|---|---|
| Primary, resectable | Single-stage complete en bloc resection with adjacent organs in a sarcoma reference centre, after core biopsy and multidisciplinary planning; preoperative radiotherapy not routine after STRASS, considered in well-differentiated and low-grade dedifferentiated liposarcoma. | not mapped |
| High-risk dedifferentiated liposarcoma or leiomyosarcoma | Neoadjuvant chemotherapy within STRASS2 (doxorubicin-ifosfamide or doxorubicin-dacarbazine) or up-front surgery; no proven adjuvant therapy. | not mapped |
| Recurrent or metastatic | Repeat resection for slowly growing, unifocal recurrence; histology-driven chemotherapy (doxorubicin plus trabectedin for leiomyosarcoma, LMS-04; eribulin or trabectedin for liposarcoma); MDM2 inhibitor trials. | not mapped |