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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.
Systemic therapy is histology-driven: doxorubicin with ifosfamide for dedifferentiated liposarcoma, doxorubicin with dacarbazine or trabectedin for leiomyosarcoma (LMS-04), and eribulin and MDM2 inhibitors for liposarcoma. The STRASS2 trial is testing neoadjuvant chemotherapy against surgery alone in high-risk dedifferentiated liposarcoma and leiomyosarcoma, and repeat surgery for recurrence, selected by growth rate and multifocality, remains the main tool after relapse.
About 15 percent of soft tissue sarcomas, mostly well-differentiated and dedifferentiated liposarcoma and leiomyosarcoma, diagnosed as very large masses in the space behind the bowel; local recurrence, not metastasis, kills most patients with liposarcoma, and outcomes depend heavily on treatment in a specialist centre.
Bone sarcomas favour the fast-growing ends of long bones (osteosarcoma) or the shaft (Ewing), soft tissue sarcomas the deep muscle compartments; spread is through the blood to the lungs, rarely via lymph nodes.
Lymph node spread is rare (except epithelioid, synovial, clear cell and rhabdomyosarcoma); sarcomas go through the blood to the lungs.
Same organ: Adamantinoma of bone, Dedifferentiated chordoma, Poorly differentiated chordoma (SMARCB1-deficient), Desmoplastic small round cell tumour, Leiomyosarcoma, Liposarcoma, Synovial sarcoma, Sarcomas (soft tissue, bone, GIST), Osteosarcoma, Ewing sarcoma, Rhabdomyosarcoma, Chordoma, Desmoid tumour, Tenosynovial giant cell tumour (TGCT), Epithelioid sarcoma, Vascular tumours (angiosarcoma, epithelioid haemangioendothelioma, kaposiform haemangioendothelioma), Chondrosarcoma, Angiosarcoma, Undifferentiated pleomorphic sarcoma (UPS), Myxofibrosarcoma, Alveolar soft part sarcoma, Perivascular epithelioid cell tumour (PEComa), Epithelioid haemangioendothelioma, Malignant peripheral nerve sheath tumour (MPNST), Soft tissue sarcoma of the extremity (localised and advanced)
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.
Neoadjuvant chemotherapy within STRASS2 (doxorubicin-ifosfamide or doxorubicin-dacarbazine) or up-front surgery; no proven adjuvant therapy.
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.
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Doxorubicin-trabectedin is a first-line standard for fit patients with metastatic leiomyosarcoma, one of the few histology-specific first-line regimens in sarcoma.
The general principles on the sarcoma subtype pages (reference centre surgery, radiotherapy for high-grade deep tumours, doxorubicin first line, histology-directed later lines) follow this guideline.
Surgery alone in a reference centre is the standard for primary retroperitoneal sarcoma; preoperative radiotherapy is considered case by case in liposarcoma.
Histology-specific strategies, such as considering neoadjuvant chemotherapy for leiomyosarcoma and high-grade dedifferentiated liposarcoma in STRASS2, follow from these patterns.
Extended en bloc resection in a sarcoma reference centre is the standard operation for retroperitoneal sarcoma.
Query for this cancer: (TITLE:"Retroperitoneal sarcoma" OR ABSTRACT:"Retroperitoneal sarcoma" OR TITLE:"Retroperitoneal soft tissue sarcoma" OR ABSTRACT:"Retroperitoneal soft tissue sarcoma" OR TITLE:"RPS" OR ABSTRACT:"RPS" OR TITLE:"Abdominal sarcoma" OR ABSTRACT:"Abdominal sarcoma") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Retroperitoneal sarcoma, not a curated reading list.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
Cumulative dose: risk rises steeply above 400-550 mg/m² (see the anthracycline calculator).
Take on an empty stomach (1 hour before or 2 hours after food).
Alcohol: avoid (hepatotoxicity). Dexamethasone 20 mg before each dose protects the liver.
See all on the product pages:DacarbazineDoxorubicinEribulinIfosfamidePazopanibTrabectedin·Printable cards in the navigator
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